Pleomorphic Adenoma Ameloblastoma
Pleomorphic Adenoma Ameloblastoma
Pleomorphic Adenoma Ameloblastoma
reveal a tumor consisting of nests of tumor cells that appear dark and crowded at the
periphery of the nests and loose in the center (similar to the stellate reticulum of a
developing tooth). as shown in the image below. Grossly, the lesions consist of
multiple cysts filled with a thick, “motor oil”–like fluid . Which of the following is the
most likely diagnosis? *
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Pleomorphic adenoma
Ameloblastoma
Mucoepidermoid carcinoma
Correct answer
Ameloblastoma
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A rare tumor of the oral cavity (found most commonly in the mandible) that is similar to
the enamel organ of the tooth is the ameloblastoma. This locally aggressive tumor
consists of nests of cells that at their periphery are similar to ameloblasts and centrally
are similar to the stellate reticulum of the developing tooth. A similar lesion occurs in
the sella turcica and is called a craniopharyngioma. In contrast, pleomorphic
adenomas, mucoepidermoid carcinomas, adenoid cystic carcinomas, and acinic cell
carcinomas are all tumors that originate in salivary glands.
A 2-month-old male infant, who was born at term without any prenatal abnormalities,
is being evaluated for possible visual problems. He is noted to have an abnormal
white light reflex involving his right eye, and examination finds a large mass that has
almost completely filled the posterior chamber of this eye. Which of the following cells
are most likely to be seen proliferating in histologic sections from this mass as show
in the image below? *
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Correct answer
Small cells forming occasional rosette structures
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Retinoblastoma is the most common malignant tumor of the eye in children. Clinically,
retinoblastoma may produce a white pupil (leucoria). This is seen most often in young
children in the familial form of retinoblastoma, which is due to a deletion involving
chromosome 13. These familial cases of retinoblastoma are frequently multiple and
bilateral, although like all the sporadic, nonheritable tumors they can also be unifocal
and unilateral. Histologically, rosettes of various types are frequent (similar to
neuroblastoma and medulloblastoma). There is a good prognosis with early detection
and treatment; spontaneous regression can occur but is rare. Retinoblastoma belongs
to a group of cancers (osteosarcoma, Wilms tumor, meningioma, rhabdomyosarcoma,
uveal melanoma) in which the normal cancer suppressor gene (antioncogene) is
inactivated or lost, with resultant malignant change. Retinoblastoma and
osteosarcoma arise after loss of the same genetic locus—hereditary mutation in the
q14 band of chromosome 13.
. A 35-year-old woman presents with slowly progressive weakness involving the left
side of her face. She says she cannot completely close her left eye. Physical
examination finds facial asymmetry characterized by flattening of the entire left side
of her face, but no abnormalities are seen on the right side. A 1.5-cm mass is found
involving the deep portion of the left parotid gland. At the time of surgery the mass is
found to be infiltrating along the facial nerve. Which of the following histologic
changes is most likely to be seen in a biopsy specimen taken from this mass? *
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Acquired achalasia
Barrett esophagus
Hamartomatous polyp
Metastatic adenocarcinoma
Reflux esophagitis
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The presence of columnar epithelium lining part or all of the distal esophagus is
known as Barrett esophagus. It is considered an acquired change resulting from reflux
of acidic gastric contents with ulceration of the esophageal squamous epithelium and
replacement by metaplastic, acid-resistant, columnar epithelium. Endoscopically it
has a velvety-red appearance. Microscopically, intestinal-type epithelium is most
common, but gastric- type epithelium is also seen. Varying degrees of dysplasia may
be present. The risk of carcinoma is increased 30- to 40-fold. Virtually all of these
tumors are of the adenocarcinoma type and they account for up to 10% of all
esophageal cancers.
. A biopsy of the antrum of the stomach of an adult who presents with epigastric pain
reveals numerous lymphocytes and plasma cells within the lamina propria, which is
of normal thickness. There are also scattered neutrophils within the glandular
epithelial cells. A Steiner silver stain from this specimen is positive for a small, curved
organism. These histologic changes are most consistent with infection by which one
of the following organisms? *
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Enterotoxigenic E coli
Helicobacter pylori
Salmonella typhi
Shigella species
Correct answer
Helicobacter pylori
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A 56-year-old woman presents with a small mass overlying her left clavicle. She
states that she has lost about 15 pounds over the past several months and has had
trouble falling asleep at night because of “heartburn.” She states that her last
menstrual period was 10 years ago, and she denies any vaginal bleeding. Physical
examination finds a solitary enlarged lymph node over her left clavicle. The lymph
node measures 1.5 cm in greatest dimension, and a biopsy from this enlarged node
reveals numerous malignant cells that are similar in appearance to those seen in the
picture below. These cells stain positively for mucin. Which one of the listed sites is
the most likely source for these malignant cells? *
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Breast
Kidney
Parotid gland
Ovary
Stomach
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A 53-year-old man presents with increasing gastric pain and is found to have a 3-cm
mass located in the anterior wall of his stomach. This mass is resected and histologic
examination reveals a tumor composed of cells having elongated, spindle-shaped
nuclei. The tumor does not connect to the overlying gastric epithelium and is instead
found only in the wall of the stomach. This tumor is usually defined histologically by
the expression of which one of the listed markers? *
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CD10 (CALLA)
CD15 (leuM1)
CD30 (Ki-1)
CD95 (Fas)
CD117 (c-KIT)
Correct answer
CD117 (c-KIT)
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Gastrointestinal stromal tumors (GIST) are mesenchymal tumors of the GI tract that
are lumped together because of a common histologic finding of spindle-shaped tumor
cells and are associated with mutations in the Kit gene or PDGFRA gene. GIST stain
positively with CD117 (the KIT protein) and negatively with desmin and S-100.
Spindle cell tumors that are negative for CD117 and positive for desmin are
leiomyomas, which are also found in the wall of the stomach. If the CD117 stain is
negative and suspicion remains that the tumor is a GIST, the newer antibody DOG-1
(discovered on GIST-1), which is expressed ubiquitously in gastrointestinal stromal
tumors irrespective of KIT or PDGFRA mutation status, can be used.
GISTs are thought to arise from interstitial cells of Cajal, which are thought to serve as
pacemaker cells that triggers gut contraction. Seventy percent of GIST occur in the
stomach, most of these behaving in a benign fashion, and 30% occur in the small
intestines, most of these behaving in a malignant fashion. Therapy for this type of
tumor is with the tyrosine kinase inhibitor imatinib (Glivec/Gleevec), which is also used
to treat chronic myelocytic leukemia (CML).
CD10 (CALLA, common acute lymphocytic leukemia antigen) is a cell surface marker
for some type of acut lymphoblastic lymphoma. CD15 (leuM1 or Lewis X) is
expressed in the malignant Reed-Sternberg cells in most patients with Hodgkin
disease. CD30 (Ki-1) is also expressed in Reed-Sternberg cells, but it is also
associated with anaplastic large cell lymphoma (Ki-1 lymphoma) and some other rare
types of malignancies.
A 39-year-old man presents with bloody diarrhea. Multiple stool examinations fail to
reveal any ova or parasites. A barium examination of the patient’s colon reveals a
characteristic “string sign.” A colonoscopy reveals the rectum and sigmoid portions of
the colon to be unremarkable. A biopsy from the terminal ileum reveals numerous
acute and chronic inflammatory cells within the lamina propria. Worsening of the
patient’s symptoms results in emergency resection of the distal small intestines.
Gross examination of this resected bowel reveals deep, long mucosal fissures
extending deep into the muscle wall. Several transmural fistulas are also found.
Which of the following is the most likely diagnosis? *
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Ulcerative colitis
Lymphocytic colitis
Infectious colitis
Eosinophilic colitis
. Crohn disease
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The two inflammatory bowel diseases (IBDs), Crohn disease (CD) and ulcerative
colitis (UC), are both chronic, relapsing inflammatory disorders of unknown etiology.
They both may show very similar morphologic features and associations, such as
mucosal inflammation, malignant transformation, and extragastrointestinal
manifestations that include erythema nodosum (especially ulcerative colitis), arthritis,
uveitis, pericholangitis (especially with ulcerative colitis, in which sclerosing
pericholangitis may produce obstructive jaundice), and ankylosing spondylitis. CD is
classically described as being a granulomatous disease, but granulomas are present
in only 25% to 75% of cases. Therefore, the absence of granulomas does not rule out
the diagnosis of CD. CD may involve any portion of the gastrointestinal tract and is
characterized by focal (segmental) involvement with “skip lesions.” Involvement of the
intestines by CD is typically trans-mural inflammation, which leads to the formation of
fistulas and sinuses. The deep inflammation produces deep longitudinal, serpiginous
ulcers, which impart a “cobblestone” appearance to the mucosal surface of the colon.
Additionally in Crohn disease, the mesenteric fat wraps around the bowel surface,
producing what is called “creeping fat,” and the thickened wall narrows the lumen,
producing a characteristic “string sign” on x-ray. This narrowing of the colon, which
may produce intestinal obstruction, is grossly described as a “lead pipe” or “garden
hose” colon. In contrast to CD, UC affects only the colon, and the disease involvement
is continuous. The rectum is involved in all cases, and the inflammation extends
proximally. Because UC involves the mucosa and submucosa, but not the wall, fistula
formation and wall thickening are absent (but toxic megacolon may occur). Grossly,
the mucosa displays diffuse hyperemia with numerous superficial ulcerations. The
regenerating, nonulcerated mucosa appears as “pseudopolyps.”
A 39-year-old woman presents with chronic abdominal cramps, watery diarrhea, and
periodic facial flushing. Physical examination reveals wheezing and a slightly
enlarged liver. Workup reveals several masses within the liver and a large mass in
the small intestine. Which of the following substances is likely to be elevated in her
urine? *
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N-formiminoglutamate (FIGlu)
Normetanephrine
Correct answer
5-hydroxyindoleacetic acid (5-HIAA)
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The patient shows signs of the carcinoid syndrome, which include flushing, diarrhea,
and bronchoconstriction. The syndrome results from elaboration of serotonin
(5-hydroxytryptamine) by a primary carcinoid tumor in the lungs or ovary or from
hepatic metastases from a primary carcinoid tumor in the gastrointestinal tract.
However, primary appendiceal carcinoid tumors, the most common gastrointestinal
carcinoid tumors, very rarely metastasize and are virtually always asymptomatic.
Carcinoid tumors arise from cells of the neuroendocrine system, which, as part of the
amine precursor uptake and decarboxylation (APUD) system, are capable of
secreting many products. Grossly, carcinoid tumors, which tend to be multiple when
they occur in the stomach or intestines, are characteristically solid and firm and have
a yellow-tan appearance on sectioning. Histologically, they are composed of nests of
relatively bland-appearing monotonous cells. Diagnosis is based on finding increased
urinary 5-hydroxyindoleacetic acid (5-HIAA) excretion from metabolism of excess
serotonin. In contrast, increased urinary levels of aminolevulinic acid (ALA) are seen
with lead toxicity, increased N-formiminoglutamate (FIGlu) with folate deficiency, and
increased normetanephrine or vanillylmandelic acid (VMA) with tumors of the adrenal
medulla (pheochromocytoma in adults and neuroblastoma in children).
An 18-year-old woman presents with abdominal pain localized to the right lower
quadrant, nausea and vomiting, mild fever, and an elevation of the peripheral
leukocyte count to 17,000/μL. An appendectomy is performed. Which of the following
statements best describes the expected microscopic appearance of her appendix? *
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Lymphoid hyperplasia and multinucleated giant cells within the muscular wall
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A 2-year-old girl is being evaluated for strikingly yellow skin and is found to have
elevated serum levels of indirect bilirubin. After appropriate workup the diagnosis of
type II Crigler-Najjar syndrome is made. She is then treated with phenobarbital,
which causes hyperplasia of the smooth endoplasmic reticulum in hepatocytes and
decreases her serum indirect bilirubin levels. What is the basic defect that caused
this child’s illness? *
2/2
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. A 4-year-old boy presents with mild fatigue and malaise. Several other children in
the day-care center he attends 5 days a week have developed similar illnesses.
Physical examination finds mild liver tenderness, but no lymphadenopathy is noted.
Laboratory examination finds mildly elevated serum levels of liver enzymes and
bilirubin. The boy recovers from his mild illness without incident. Which of the
following organisms is the most likely cause of this child’s illness? *
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Cytomegalovirus (CMV)
Hepatitis A virus
Hepatitis B virus
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Several types of viruses are implicated as being causative agents of viral hepatitis.
Each of these has unique characteristics. Hepatitis A virus, an RNA picornavirus, is
transmitted through the fecal-oral route (including shellfish) and is called infectious
hepatitis. It is associated with small outbreaks of hepatitis in the United States,
especially among young children at day care centers. Hepatitis B virus, which causes
“serum hepatitis,” is associated with the development of a serum sickness-like
syndrome in about 10% of patients. Immune complexes of antibody and HBsAg are
present in patients with vasculitis. Hepatitis C virus is characterized by episodic
elevations in serum transaminases and also by fatty change in liver biopsy specimens.
Hepatitis D virus is distinct in that it is a defective virus and needs HBsAg to be
infective. Hepatitis E virus is characterized by waterborne transmission. It is found in
underdeveloped countries and has an unusually high mortality in pregnant females. It
is important to remember that the liver may be infected by other viruses, such as
yellow fever virus, Epstein-Barr virus (EBV, the causative agent of infectious
mononucleosis), CMV, and/or herpes virus. The latter is characterized histologically
by intranuclear eosinophilic inclusions (Cowdry bodies) and nuclei that have a
ground-glass appearance.
A 48-year-old man with fatigue is being evaluated for a 1-year history of elevated
serum liver enzymes. A liver biopsy is taken and the pathology report of this
specimen states there is grade 2 inflammatory activity with piecemeal necrosis and
stage 1 fibrosis. The term “piecemeal necrosis” refers to which one of the following
pathologic abnormalities? *
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Destruction of the limiting plate with necrosis of hepatocytes surrounding the portal
triad
Fibrosis around the central hepatic veins with apoptosis of adjacent hepatocytes
Necrosis of hepatocytes extending from the portal area of one hepatic lobule to the
central vein of an adjacent lobule
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Chronic hepatitis has been defined clinically as an inflammatory process of the liver
that lasts longer than 6 months. The diagnosis and classification of chronic hepatitis
has changed somewhat over the past several years. Previously chronic hepatitis was
classified histologically into chronic active hepatitis and chronic persistent hepatitis. In
chronic active hepatitis, an intense inflammatory reaction with numerous plasma cells
spreads from portal tracts into periportal areas. The reaction destroys the limiting
plate and causes necrosis of the hepatocytes surrounding the portal triad. This
histologic change is called “piecemeal necrosis” or “interface hepatitis.” Chronic
persistent hepatitis was differentiated from chronic active hepatitis by the fact that the
portal inflammation did not extend into the periportal areas; that is, there was no
piecemeal necrosis.
Today chronic hepatitis is given a histologic grade, which is based on the
inflammation activity present, and a histologic stage, which is based on the amount of
fibrosis present. A histologic grade of 0 is characterized by minimal portal
inflammation and no changes in the hepatic lobule, while a grade of 4 is characterized
by severe limiting plate destruction with bridging necrosis. Mild to moderate
piecemeal necrosis is seen with inflammatory activity grades 2 and 3. Stage 0 fibrosis
is characterized by minimal to no fibrosis, while stage 4 fibrosis is characterized by
cirrhosis.
In contrast to destruction of the limiting plate, Congo red–positive extracellular
deposits surrounding hepatocytes are diagnostic for amyloidosis; fibrosis around
central hepatic veins suggests alcoholic liver disease; and apoptosis of hepatocytes
suggests viral hepatitis.
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Autoimmune hepatitis
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Cholangitis
Cholestasis
Cirrhosis
Hepatitis
Steatosis
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Cirrhosis refers to fibrosis of the liver that involves both central veins and portal triads.
This fibrosis is the result of liver cell necrosis and regenerative hepatic nodules.
These nodules consist of hyperplastic hepatocytes with enlarged, atypical nuclei,
irregular hepatic plates, and distorted vasculature. There is distortion of the normal
lobular architecture. These changes diffusely involve the entire liver; they are not focal.
It is thought that the fibrosis is the result of fibril-forming collagens that are released by
Ito cells, which are fat-containing lipocytes found within the space of Disse of the liver.
They normally participate in the metabolism and storage of vitamin A, but they can
secrete collagen in the fibrotic (cirrhotic) liver. Normally types I and III collagens
(interstitial types) are found in the portal areas and occasionally in the space of Disse
or around central veins. In cirrhosis, types I and III collagens are deposited throughout
the hepatic lobule. These Ito cells are initiated by unknown factors and then are
further stimulated by such factors as platelet-derived growth factor and transforming
growth factor-beta to secrete collagen.
In contrast cholangitis refers to inflammation of the bile duct, while cholestasis refers
to decreased flow of bile, usually due to obstruction. Hepatitis is characterized by
inflammation within the liver, and steatosis by fatty change within hepatocytes.
A 45-year-old obese woman presents with increasing fatigue, malaise, and fullness
in the right upper quadrant of her abdomen. Pertinent clinical history includes type II
diabetes mellitus and hyperlipidemia. Laboratory test finds elevated liver enzymes
along with increased serum cholesterol. Which one of the following clinical
procedures or tests should be used to confirm a diagnosis of nonalcoholic
steatohepatitis? *
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Liver biopsy
Liver ultrasonography
Oral cholecystogram
Correct answer
Liver biopsy
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The correct answer is liver biopsy
A 51-year-old male alcoholic with a history of chronic liver disease presents with
increasing weight loss and ascites. Physical examination reveals a slightly enlarged,
soft, nontender prostate. Examination of the scrotum is unremarkable, and fecal
occult blood tests are negative. A chest x-ray is unremarkable, but a CT scan of the
abdomen reveals a single mass in the left lobe of the liver. Workup reveals elevated
levels of α-fetoprotein in this patient’s blood. Which of the following is the most likely
diagnosis? *
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Angiosarcoma
Cholangiocarcinoma
Hepatoblastoma
Hepatocellular carcinoma
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The most common primary malignancy of the liver is the hepatocellular carcinoma
(hepatoma). These tumors are associated with certain viral infections (hepatitis B and
hepatitis C viruses), aflatoxin (produced by Aspergillus flavus), and cirrhosis.
Microscopic sections of these tumors reveal pleomorphic tumor cells that form
trabecular patterns, which are similar to the normal architecture of the liver.
Hepatomas may secrete α-fetoprotein (AFP), but this tumor marker may also be seen
in yolk sac tumors or fetal neural tube defects. Clinically, hepatocellular carcinomas
have a tendency to grow into the portal vein or the inferior vena cava and may be
associated with several types of paraneoplastic syndromes, such as polycythemia,
hypoglycemia, and hypercalcemia. There is a microscopic fibrolamellar variant of
hepatocellular carcinoma that is seen more often in females, is not associated with
AFP, is grossly encapsulated, and has a better prognosis. It is important to compare
the characteristics of hepatocellular carcinomas with those of another type of primary
tumor of the liver, namely cholangiocarcinoma, which is a malignancy of bile ducts.
This tumor is associated with Thorotrast and infection with the liver fluke (C sinensis),
but it is not associated with cirrhosis. Histologically, the tumor cells contain
cytoplasmic mucin, which is not found in hepatomas. Instead, these malignant cells
may contain cytoplasmic bile. Malignant metastatic tumors are the most common
tumors found in the liver. Grossly there may be multiple or single nodules, which
microscopically usually resemble the primary tumor. For example, metastatic colon
cancer to the liver histologically reveals adenocarcinoma. Metastatic disease to the
liver usually does not cause functional abnormalities of the liver itself, and the liver
enzymes and bilirubin levels in the blood are usually normal. Angiosarcomas are
highly aggressive malignant tumors that arise from the endothelial cells of the
sinusoids of the liver. Their development is associated with certain chemicals, such as
vinyl chloride, arsenic, and Thorotrast. A malignant tumor of the liver that is found in
children is the hepatoblastoma. Microscopically, these tumors consist of ribbons and
rosettes of fetal embryonal cells.
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Acute cholecystitis
Acute colitis
Acute pancreatitis
Chronic gastritis
Chronic hepatitis
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Patients with acute pancreatitis typically present with abdominal pain that is localized
to the epigastrium and radiates to the back. It is usually associated with increased
serum levels of pancreatic enzymes. Laboratory confirmation of pancreatic disease
involves the finding of elevated serum amylase levels in the first 24 h and rising lipase
levels over the next several days. Other pancreatic enzymes, such as trypsin,
chymotrypsin, and carboxypeptidases, have not been as useful for diagnosis as have
amylase and lipase. Other signs of acute pancreatitis include vomiting, and shock, the
latter being the result of hemorrhage and kinins released into the blood. In severe
pancreatitis, there may be hemorrhage in the subcutaneous tissue around the
umbilicus (Cullen sign) and in the flanks (Turner sign). Acute pancreatitis usually
presents as a medical emergency. Activation of the plasma coagulation cascade may
lead to disseminated intravascular coagulopathy (DIC). Complications seen in
patients who survive the acute attack include pancreatic abscess formation,
pseudocyst formation, or duodenal obstruction.
Most cases of acute pancreatitis are associated with either alcohol ingestion or biliary
tract disease (gallstones). Alcohol ingestion is the most common cause, and
pancreatitis usually follows an episode of heavy drinking. Other, less frequent causes
include hypercalcemia, hyperlipidemias, shock, infections (CMV and mumps), trauma,
and drugs.
A 48-year-old male alcoholic presents with malaise, fever, and midabdominal pain
that radiates to his back. Pertinent medical history includes repeated bouts of
pancreatitis that mainly occur after times of binge drinking. Physical examination
finds a low-grade fever, and a mass is palpated in the epigastric area. An abdominal
CT scan finds a fluid-filled mass in the pancreas. This mass is removed at celiotomy
and has a similar appearance to the cystic mass shown in the photograph. It is filled
with clear fluid, and histologic sections reveal a large cystic structure that lacks an
epithelial lining. Which of the following is the most likely diagnosis? *
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Cylindroma
Hydrocystoma
Pseudocyst
Pseudomyxoma
Syringoma
Correct answer
Pseudocyst
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Pseudocysts of the pancreas are so named because the cystic structure is essentially
unlined by any type of epithelium. True cysts, wherever they are found in the body,
are always lined by some type of epithelium, whether columnar cell, glandular,
squamous, or flattened cuboidal cell. The pancreatic pseudocyst is most commonly
found against a background of repeated episodes of pancreatitis. Eventual
mechanical large duct obstruction by an inflammatory process per se, periductal
fibrosis, or an abscess along with inspissated duct fluid from secretions and enzymes
leads to the expanding mass. The mass lesion may be located between the stomach
and liver, between the stomach and the colon or transverse mesocolon, or in the
lesser sac. Drainage or excision is necessary for adequate treatment. Acute bacterial
infection may complicate the clinical course.
In contrast, a hydrocystoma is a benign cyst filled with clear fluid. They are found on
the skin, especially the eyelids. Cylindromas and syringomas are two types of benign
skin tumors, while pseudomyxoma is a condition where mucinous tumors are found in
the abdomen (pseudomyxoma peritonnei). This condition is most commonly
associated with a mucocele of the appendix, but is also found with mucus-secreting
tumors of the ovaries.
A 69 year-old man presents with slowly progressive jaundice, weight loss, abdominal
pain, and migratory thrombophlebitis. Physical examination reveals a palpable gall
bladder in the right upper quadrant of the abdomen. Laboratory examination reveals
the presence of obstruction of the biliary tract, while a CT scan of the abdomen
reveals a mass in the head of the pancreas. What histologic sections from this mass
are most likely to reveal? *
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Adenocarcinoma
Mucinous cystadenocarcinoma
Papillary-cystic tumor
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Most carcinomas of the pancreas arise from the ductal epithelium of the pancreas and
are adenocarcinomas. Pancreatic cancers are highly malignant tumors that account
for about 5% of cancer deaths in the United States. Their occurrence has increased
threefold in the past 40 years, mainly as a result of smoking and exposure to chemical
carcinogens. They are more frequent in diabetics than nondiabetics. Most cases are
found in the head of the pancreas (70%) and produce symptoms such as obstructive
jaundice and migratory thrombophlebitis, usually in the superficial veins of the leg
(Trousseau sign).
Courvoisier law states that obstructive jaundice in the presence of a dilated
gallbladder is most suggestive of cancer of the head of the pancreas. About 20% of
pancreatic adenocarcinomas are found in the body and 10% are found in the tail.
Tumors located in the tail of the pancreas present late, when therapy is no longer
possible. The major symptoms of pancreatic carcinomas in general include weight
loss, abdominal pain (usually the first symptom), back pain, and malaise. Surgery for
a tumor of the head of the pancreas may involve pancreatoduodenectomy, which is
called a Whipple procedure.
Cystic neoplasms of the pancreas, which comprise only about 5% of pancreatic
neoplasms, include mucinous cystadenomas, mucinous cystadenocarcinomas,
microcystic adenomas (benign serous tumors), and solid-cystic tumors. Serous
tumors (also called microcystic adenomas) are almost always benign, while mucinous
tumors may be either benign (mucinous cystadenomas) or malignant
(cystadenocarcinoma). An unusual cystic tumor is the solid-cystic (papillary-cystic)
tumor which is seen in women younger than 35 years of age and is usually cured by
resection.
A 12-year-old nonobese boy presents for evaluation after becoming sick at school.
Pertinent recent medical history includes weight loss with polyphagia, polydipsia, and
polyuria. Laboratory examination finds hyper-glycemia, while urinary examination
reveals increased glucose and trace ketones. Which of the following abnormalities is
most likely to be present in this boy? *
0/2
Correct answer
Lymphocytic infiltration in the pancreatic islets
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There are many clinical and pathophysiologic differences between type 1 and type 2
diabetes mellitus (DM). Type 1 DM occurs most often in children, while type 2 DM is
found in adults. Type 2 DM is often associated with obesity (and also decreased
numbers of insulin receptors on adipocytes), but children with IDDM are of normal
weight, even though they have an increased appetite (polyphagia). The basic defect
in type 1 DM is decreased blood insulin levels due to a decrease in the number of
insulin-producing beta cells. The mechanisms involved in this beta-cell destruction
include genetic susceptibility, autoimmunity, and environmental factors. Type 1 DM is
more common in genetically susceptible individuals, that is, type 1 DM is more
common in individuals of Northern European descent, and it is also linked to HLA
types DR3, DR4, and DR3/4. In contrast, type 2 DM is not linked to any HLA type. The
destruction of the beta cells is thought to be autoimmune mediated, as the majority of
patients with type 1 DM have circulating islet cell antibodies (including anti–glutamic
acid decarboxylase [anti-GAD]). Additionally, histologic examination of the islets of
patients with type 1 DM reveals a lymphocytic infiltrate (insulitis). In contrast, the islets
of patients with type 2 DM lack the inflammation, but may show focal atrophy with
amyloid deposition. This amyloid is composed of amylin, a normal product of the beta
cells. Finally, it is thought that environmental factors may trigger the autoimmunity that
produces type 1 DM in genetically susceptible individuals. Possible causes for this are
being investigated and include viruses (especially group B coxsackievirus), chemical
toxins, and even cow’s milk ingested early in life.
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Which of the following is the most likely cause of the clinical combination of increased
blood pressure, edema, slight proteinuria, hematuria, and red blood cell casts in the
urine? *
1/1
Nephritic syndrome
Nephrotic syndrome
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YOU GOT IT RIGHT!
A 28-year-old man with a history of malaise and hemoptysis presents with the acute
onset of renal failure. Laboratory examination reveals increased serum creatinine
and BUN, but neither antineutrophil cytoplasmic antibodies (ANCA) nor antinuclear
(ANA) antibodies are present. Urinalysis reveals the microscopic presence of red
blood cells and red blood cell casts, while a renal biopsy reveals crescents within
Bowman space of many glomeruli. Immunofluorescence reveals linear deposits of
IgG and C3 along the glomerular basement membrane. Which of the following is the
most likely diagnosis? *
1/1
Alport syndrome
Diabetic glomerulopathy
Goodpasture syndrome
Henoch-Schönlein purpura
Wegener granulomatosis
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A 26-year-old woman presents with increasing fatigue and malaise. She states that
recently she develops a red facial rash whenever she goes outside on a sunny day.
Physical examination finds that she is afebrile, but her blood pressure is slightly
increased and slight peripheral edema is found. Laboratory evaluation finds slightly
elevated BUN and creatinine, while dip-stick examination of her urine reveals slight
proteinuria with microscopic hematuria. Very rare granular and red cell casts are
seen. Laboratory examination is also positive for serum antinuclear antibodies, one
of which is anti–double-stranded DNA. A renal biopsy reveals changes of diffuse
proliferative glomerulonephritis, and the diagnosis of class IV lupus nephritis is made.
Which of the following histologic changes is most characteristic of class IV lupus
nephritis? *
1/1
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The glomerular diseases of patients with systemic lupus erythematosus (SLE) are
many and include mesangial lupus glomerulonephritis (GN), focal or diffuse
proliferative GN, and membranous GN. The World Health Organization (WHO)
classifies SLE renal disease into five classes as follows: class I = no changes; class II
= mesangial GN; class III = focal proliferative GN; class IV = diffuse proliferative GN
(the most common class); class V = diffuse membranous GN. All of these glomerular
diseases are the result of the deposition of immune complexes (DNA-anti-DNA
complexes) that may be in a mesangial, intramembranous, subepithelial, or
subendothelial location. In membranous lupus GN, the deposits are in a subepithelial
location, while in diffuse proliferative lupus GN (WHO class IV) the deposits are
mainly in a subendothelial location and produce a characteristic “wire-loop”
appearance due to thickening of the capillary wall. None of these changes are specific
for lupus.
In contrast to the “wire-loop” appearance of the glomerular capillaries with lupus
nephritis, “holly leaf” mesangial deposits are seen with focal segmental GN (IgA
deposits suggests Berger disease), a “string of popcorn” immunofluorescence pattern
is also seen with membranous glomerulonephropathy, “tram-track” splitting of the
basement membrane is seen with both types of membranoproliferative GN, and a
“spike and dome” appearance of the basement membrane is seen with membranous
glomerulonephropathy
A 54-year-old man presents with left-sided costovertebral pain and gross hematuria.
A large mass is found in the upper pole of one of his kidneys, as seen in the picture.
A biopsy from this mass reveals uniform cells with clear cytoplasm containing
glycogen and lipid. What is the best diagnosis? *
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Neuroblastoma
Wilms tumor
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The major histologic types of malignant kidney tumors (renal cell carcinomas) are
clear cell carcinoma, papillary carcinoma, chromophobe renal cell carcinoma, and
collecting duct carcinoma. Renal cell carcinomas are predominantly of the clear cell
type (clear cell carcinoma) with intracytoplasmic glycogen and lipid. These tumors are
associated with loss deletions involving the short arm of chromosome 3 in the region
of the VHL gene. Papillary renal cell carcinomas are characterized by their papillary
structures and in some cases are associated with abnormalities of the MET gene.
Chromophobe renal carcinomas are composed of cells with pale eosinophilic
cytoplasm, while collecting duct (Bellini duct) renal cell carcinomas are characterized
by nests of malignant cells within a prominent fibrotic stroma.
Renal cell carcinoma accounts for the majority of primary renal tumors and usually
occurs in the sixth decade, although sometimes at a much younger age. The
combination of costovertebral pain, a palpable mass, and hematuria is the classic
triad of symptoms seen in about 10% of patients with renal cell carcinoma. Hematuria
is often the first symptom, but it often occurs late, after invasion of the renal vein or
widespread metastases, frequently to lung, bone, or brain. Grossly, renal cell
carcinoma are greater than 3 cm in diameter and are yellow in color.
In contrast, carcinomas originating from the renal pelvis (not the cortex) arise from
transitional epithelial cells and microscopically are similar to tumors arising in the
urinary bladder, that is, transitional cell carcinomas. A Wilms tumor is a malignant
tumor of the kidney in children. Histologically, they are composed of a combination of
metanephric blastema, undifferentiated mesenchymal cells, and immature tubule or
abortive glomerular formation. They result from a deletion of the WT1 gene, which is
on chromosome 11. Neuroblastomas are another malignant tumor of children. They
are the most common tumor of adrenal medulla in children. Histologically, theses
tumors are composed of small cells forming Homer-Wright rosettes, which are groups
of cells arranged in a ring around a central mass of pink neural filaments.
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MET gene
PRCC gene
p16INK4a gene
VHL gene
WT1 gene
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Malignant tumors of the kidney in children are called nephroblastomas (Wilms tumor)
and histologically reveal a combination of metanephric blastema, undifferentiated
mesenchymal cells, and immature tubule or glomerular formation. Children present
with an enlarging abdominal mass that, in contrast to adrenal neuroblastoma, is
associated with normal urinary vanillylmandelic acid (VMA) levels. Deletions involving
WT1, located on chromosome 11, are associated with the development of Wilms
tumor (nephroblastoma). Several syndromes are associated with genetic deletions of
WT1, that lead to an increased incidence of Wilms tumor. These include WAGR
syndrome (characterized by aniridia, genital abnormalities, and mental retardation)
and Denys-Drash syndrome (characterized by gonadal dysgenesis and renal failure).
Deletions involving a second Wilms tumor gene (WT2) are associated with
Beckwith-Wiedemann syndrome (characterized by hemihypertrophy, renal medullary
cysts, and adrenal cytomegaly).
In contrast, the development of renal cell carcinoma (adenocarcinoma of the kidney)
is associated with defects involving several different genes, including the VHL gene,
the MET protooncogene, and the PRCC (papillary renal cell carcinoma) gene. Defects
of the p16INK4a tumor-suppressor gene are associated with the development of
urothelial (transitional cell) tumors.
The photomicrograph is of a section from a testis removed from the inguinal region of
a man aged 25. Which of the following statements about best describes this
condition? *
0/2
Correct answer
There is increased risk of malignancy in the contralateral testis
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The correct answer is there is increased risk of malignancy in the contralateral testis
The condition illustrated is cryptorchidism (failure of the testis to descend into the
scrotum). It is present in up to 1% of males after puberty and is unilateral in the
majority of cases. The testis is small, brown, and atrophic grossly. Microscopically, the
tubules are atrophic with thickened basement membranes. The interstitial cells are
usually prominent and occasional focal proliferations of Sertoli cells may be seen. The
incidence of malignancy is increased 7- to 11-fold, and this risk is greater for
abdominal than for inguinal locations. Seminoma is the most common malignancy.
The risk of malignancy is not reduced by orchiopexy. There is a smaller but definite
risk of malignancy in the contralateral, correctly placed testis.
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Choriocarcinoma
Embryonal carcinoma
Seminoma
Teratoma
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The picture shows a tumor composed of large cells with distinct cell membranes and
clear cytoplasm. There are lymphocytes in the fibrous stroma between the groups of
tumor cells. This is the classic histologic appearance of a seminoma.
Germ cell tumors of the testis are clinically divided into two categories—seminomas
and nonseminomatous germ cell tumors (NSGCTs)—because of their differences in
presentation, metastasis, prognosis, and therapy. The NSGCTs include embryonal
carcinomas, yolk sac tumors (infantile embryonal carcinomas or endodermal sinus
tumors), choriocarcinomas, and immature teratomas. When compared with NSGCTs,
seminomas are extremely radiosensitive, and they are more commonly present with
stage I disease. NSGCTs are relatively radioresistant, are more aggressive, and have
a worse prognosis. Seminomas typically spread via lymphatics after having remained
localized for a long time. Embryonal carcinoma, chorio-carcinoma, and mixed tumors
with an element of choriocarcinoma tend to metastasize early via the blood.
Choriocarcinomas are the most aggressive variant.
An important, distinct variant of seminoma is the spermatocytic seminoma. It is
characterized by being found in older individuals and by the fact that it does not
metastasize. Histologically, a spermatocytic seminoma is characterized by maturation
of the tumor cells, some of which histologically resemble secondary spermatocytes. In
contrast to the histologic appearance of seminomas and spermatocytic seminomas,
choriocarcinomas have a mixture of malignant cytotrophoblasts and
syncytiotrophoblasts, malignant teratomas have tissue derived from all three germ
levels with scattered immature neural elements, Leydig cell tumors have large tumor
cells with abundant eosinophilic, granular cytoplasm, and rare intracytoplasmic
rhomboid crystals, and finally, embryonal carcinomas have sheets of undifferentiated
tumor cells. Focal glandular differentiation may be present.
A 69-year-old man presents with urinary frequency, nocturia, dribbling, and difficulty
in starting and stopping urination. Rectal examination reveals the prostate to be
enlarged, firm, and rubbery. A needle biopsy reveals increased numbers of glandular
elements and stromal tissue. The glands are found to have a double layer of
epithelial cells. Prominent nuclei or back-to-back glands are not seen. Which of the
following is the most likely diagnosis? *
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Atrophic prostatitis
Prostatic adenocarcinoma
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Condyloma acuminatum
Verrucous carcinoma
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Adenocarcinomas of the vagina and cervix have always existed, but rates are
increased in young women whose mothers received diethylstilbestrol (DES) while
pregnant. DES, which has estrogenic activity, was used in the past to terminate an
attack of threatened abortion and thereby stabilize the pregnancy. However, a side
effect of this therapy proved to be a particular form of adenocarcinoma, clear cell
carcinoma. The tumor, which carries a poor prognosis, has at least three histologic
patterns. One is a tubulopapillary configuration, followed by sheets of clear cells and
glands lined by clear cells, and solid areas of relatively undifferentiated cells. Many of
the cells have cytoplasm that protrudes into the lumen and produces a “hobnail”
(nodular) appearance. Prior to the development of adenocarcinoma, a form of
adenosis consisting of glands with clear cytoplasm that resembles that of the
endocervix can be seen. This has been termed vaginal adenosis and may be a
precursor of clear cell carcinoma. Clinically, adenosis of the vagina is manifested by
red, moist granules superimposed on the pink-white vaginal mucosa.
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Adenomyosis
Endometriosis
Hydatid cyst
Hydatidiform mole
Luteal cyst
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2/2
Option 1
Option 2
Option 3
Option 4
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Cancers that originate from the endometrium grossly may present as a polypoid mass
within the uterine cavity or a diffuse tumor involving the endometrium with possible
spread into the myometrium. Histologically, they are adenocarcinomas that are
composed of malignant, infiltrating glandular structures. If there are areas of
squamous differentiation within these tumors, they are called adenoacanthomas. If
there are areas of malignant squamous differentiation, they are called
adenosquamous carcinomas. Endometrial carcinoma affects menopausal and
postmenopausal women, with the peak incidence at 55 to 65 years of age. Although it
was much less common than squamous cervical cancer several decades ago, it has
not been controlled as effectively as cervical cancer by the Papanicolaou smear
technique and therapy, so that it is now more common than invasive cervical cancer.
However, the major symptom of endometrial carcinoma—postmenopausal
bleeding—results in diagnosis while the tumor is still confined to the uterus (stage I or
II), which permits cure by surgery or radiotherapy. Risk factors for endometrial cancer
include obesity and glucose intolerance or diabetes. Option 1 is clear cell carcinoma ,
option 2 is small cell carcinoma and option 4 is squamous cell carcinoma of the
endometrium
A 25-year-old woman presents with lower abdominal pain, fever, and a vaginal
discharge. Pelvic examination reveals bilateral adnexal (ovarian) tenderness and
pain when the cervix is manipulated. Cultures taken from the vaginal discharge grow
Neisseria gonorrhoeae. Which of the following is the most likely cause of this
patient’s adnexal pain? *
1/1
Adenomatoid tumor
Ectopic pregnancy
Endometriosis
Luteoma of pregnancy
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0/1
Brenner tumor
Mucinous cystadenoma
Serous cystadenocarcinoma
Correct answer
Mucinous cystadenoma
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0/2
Acid phosphatase
α-Fetoprotein
Estrogen
Testosterone
Correct answer
Estrogen
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The most common type of ovarian tumor that is composed of cells that stain positively
with inhibin is a granulosa cell tumor. Histologically, the cells may form Call-Exner
bodies, which are gland-like structures formed by the tumor cells aligning themselves
around a central space that is filled with acidophilic material. The tumor cells may
secrete estrogens and cause precocious sexual development in girls or increase the
risk for endometrial hyperplasia and carcinoma in women. Less commonly granulosa
cell tumors can secrete androgens and produce masculinization. Sertoli-Leydig
tumors (androblastomas) also may secrete androgens and produce virilization in
women. The tumor cells may stain positively with inhibin, but Call-Exner bodies are
not present. Granulosa cell tumors vary in their clinical behavior, but they are
considered to be potentially malignant.
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Brenner tumor
Chronic salpingitis
Mucinous cystadenoma
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Benign cystic teratomas constitute about 10% of cystic ovarian tumors. The cysts
contain greasy sebaceous material mixed with a variable amount of hair. The cysts’
walls contain skin and skin appendages, including sebaceous glands and hair follicles.
A variety of other tissues—such as cartilage, bone, tooth, thyroid, respiratory tract
epithelium, and intestinal tissue—may be found. The presence of skin and skin
appendages gives the tumor its other name, dermoid cyst.Mature cystic teratomas
are benign, but in less than 2%, one element may become malignant, most frequently
the squamous epithelium.
In contrast to the characteristic histologic findings with a dermoid cyst, an ovarian
Brenner tumor histologically is similar to the transitional lining of the renal pelvis or
bladder. Chronic salpingitis would reveal the presence of chronic inflammatory cells,
such as lymphocytes and macrophages, within the fallopian tubes, while histologic
sections from an ectopic pregnancy would reveal chorionic villi, and possibly, fetal
tissue.
A 26-year-old woman develops acute lower abdominal pain and vaginal bleeding.
While in the bathroom she passes a cast of tissue composed of clot material and
then collapses. She is brought to the hospital, where a physical examination reveals
a soft, tender mass in right adnexa and pouch of Douglas. Histologic examination of
the tissue passed in the bathroom reveals blood clots and decidualized tissue. No
chorionic villi or trophoblastic tissue are present. Which of the following conditions is
most likely present in this individual? *
2/2
Ectopic pregnancy
Endometrial hyperplasia
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A 25-year-old woman in her 15th week of pregnancy presents with uterine bleeding
and passage of a small amount of watery fluid and tissue. She is found to have a
uterus that is much larger than estimated by her gestational dates. Her uterus is
found to be filled with cystic, avascular, grapelike structures that do not penetrate the
uterine wall. No fetal parts are found. Immunostaining for p57 was negative in the
cytotrophoblasts and villi mesenchyme. Which of the following is the best
diagnosis? *
0/1
Invasive mole
Choriocarcinoma
Correct answer
Complete hydatidiform mole
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. A 23-year-old woman presents with a rubbery, freely movable 2-cm mass in the
upper outer quadrant of the left breast. Which of the following histologic
characteristics best describes the mass as shown in the image below ? *
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Large numbers of neutrophils
Correct answer
A mixture of fibrous tissue and ducts
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The most common benign neoplasm of the breast is fibroadenoma, which typically
occurs in the upper outer quadrant of the breast in women between the ages of 20
and 35. These lesions originate from the terminal duct lobular unit and histologically
reveal a mixture of fibrous connective tissue and ducts. Clinically, fibroadenomas are
rubbery, freely movable, oval nodules that usually measure 2 to 4 cm in diameter.
Numerous neutrophils are seen in acute bacterial infection of the breast (acute
mastitis), which is usually seen in the postpartum lactating or involuting breast.
Dilation of the breast ducts (ectasia) with inspissation of breast secretions is
characteristic of mammary duct ectasia, which is common in elderly women. If large
numbers of plasma cells are also present, the lesion is called plasma cell mastitis. Fat
necrosis of the breast, associated with traumatic injury, is characterized by necrotic fat
surrounded by lipid-laden macrophages and a neutrophilic infiltration.
392. A 39-year-old woman presents with new onset of a bloody discharge from her
right nipple. Physical examination reveals a 1-cm freely movable mass that is located
directly beneath the nipple. Sections from this mass reveal multiple fibrovascular
cores lined by several layers of epithelial cells. Atypia is minimal. The lesion is
completely contained within the duct and no invasion into underlying tissue is seen.
Which of the following is the most likely diagnosis? *
2/2
Benign phyllodes tumor
Ductal papilloma
Intraductal carcinoma
Paget disease
Papillary carcinoma
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Ductal papillomas are usually found near the nipple and present with a bloody nipple
discharge. The histologic distinction between benign cystic intraductal papillomas of
the breast and papillary adenocarcinomas is based on multiple criteria. The age of the
patient is not of immense importance, since papillomas occur in both younger and
older women. Benign papillomas are structured with a complex arrangement of
papillary fronds of fibrovascular stalks, covered by one or (usually) two types of cells
(epithelial and myoepithelial). Papillary carcinomas are usually of one monotonous
cell type and have either no or only a few fibrovascular stalks. Papillary carcinomas
show a uniform growth of cells with similar appearance with enclosed tubular spaces;
the whole arrangement bridges across the entire lumen at times or simply lines the
outer rim of the duct (cribriforming). Peripheral invasion of the stroma, if present at all,
makes the diagnosis of carcinoma rather certain. There are lesions in which the
differentiation is exceedingly difficult, even in the hands of renowned surgical
pathologists. Many competent pathologists understandably prefer to defer the
diagnosis on all papillary lesions of the breast on frozen section until well-fixed and
optimally prepared permanent sections are available.
. A 35-year-old woman presents with a 2.2-cm mass in her left breast. The mass is
excised, and histologic sections reveal a tumor composed of a mixture of ducts and
cells, as seen in the photomicrograph. The epithelial cells within the ducts are not
atypical in appearance. There is a marked increase in the stromal cellularity, but the
stromal cells are not atypical in appearance and mitoses are not found. Which of the
following is the most likely diagnosis? *
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Fibroadenoma
Medullary carcinoma
Correct answer
Benign phyllodes tumor
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Neoplastic proliferations of the stroma of the breast may lead to the formation of either
fibroadenomas or phyllodes tumors. Fibroadenomas are characterized histologically
by a mixture of fibrous tissue and ducts, with no increase in cellularity or mitoses. Only
the stromal cells, not the glandular cells, are clonal proliferations. Another neo-plastic
tumor that arises from the stromal cells is the phyllodes tumor. It is distinguished from
fibroadenomas by a more cellular stroma and the presence of stromal mitoses. The
phyllodes tumor, which has been called a cystosarcoma phyllodes, may either be
benign or malignant. A benign phyllodes tumor is characterized by increased stromal
cells with few mitoses, while a malignant phyllodes tumor has increased numbers of
stromal cells that are atypical along with numerous mitoses.
A 48-year-old woman presents with a painless mass located in her left breast.
Physical examination finds a firm, nontender, 3-cm mass in the upper outer quadrant
of her left breast. There was retraction of the skin overlying this mass, and several
enlarged lymph nodes were found in her left axilla. The mass was resected and
histologic sections revealed an invasive ductal carcinoma. Biopsies from her axillary
lymph nodes revealed the presence of metastatic disease to 4 of 18 examined
axillary lymph nodes. Response to therapy with Trastuzumab is most closely
associated with expression of which of the following? *
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BRCA1
Estrogen receptors
HER2/neu
Progesterone receptors
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Prognostic factors for women with breast cancer have been divided into major
prognostic factors and minor prognostic factors. Systemic therapy may be beneficial
for most women with nodal metastases or carcinomas larger than 1 cm. Three of the
minor prognostic factors are used to help determine the type of chemotherapy to be
used. These three factors are the presence of estrogen receptors, the presence of
progesterone receptors, and the overexpression of HER2/neu. The evaluation of the
hormone receptor status of breast cancer cells is useful to predict the response to
hormonal manipulation. Most tumors that are positive for estrogen and progesterone
receptors respond to hormone manipulation, such as with tamoxifen, a selective
estrogen receptor modulator. Amplification of HER2/neu, which is a membrane
glycoprotein that is involved in control of cell growth, is associated with a poor
prognosis. Trastuzumab (Herceptin) is an antibody to HER2/neu. The use of
Trastuzumab improved the response to chemotherapy of patients whose tumors
overexpressed HER2/neu.
Major prognostic factors include tumor size, the presence of invasion, lymph node
metastases, and distant metastases. The presence of metastases to axillary lymph
nodes is the single most important prognostic indicator for invasive breast cancers in
the absence of distant metastases. The absolute number of involved lymph nodes is
directly related to the survival rate. There is a significant decrease in 5-year survival if
one to three nodes are positive, but with four or more positive nodes at the time of
diagnosis the survival rate is much less. The histologic type and grade of tumor and
its size are also important, but minor factors for predicting prognosis. The histologic
grade is based on the degree of tubule formation, the number of mitoses present, and
the degree of nuclear pleomorphism. Tubule formation is associated with a lower
grade and a better prognosis, while nuclear pleomorphism and the number of mitoses
are associated with a higher grade and a worse prognosis. Finally, tumors with a high
proliferative rate are associated with a worse prognosis, while high levels of urokinase
plasminogen activator are associated with a better prognosis.
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Correct answer
Proliferation of ducts in hyalinized fibrous tissue with periductal edema
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The correct answer is proliferation of ducts in hyalinized fibrous tissue with periductal
edema
A 25-year-old woman who has never been pregnant presents with amenorrhea for 3
months and a milky discharge from her nipple. She states that her menstrual cycles
have been irregular for the past year. Laboratory tests show that her serum LH and
estradiol levels are below normal, and a pregnancy test is negative. Which of the
following is the most likely cause of these signs and symptoms? *
1/1
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Pituitary adenomas are the most common neoplasms of the pituitary gland. These
benign neoplasms are classified according to the hormone or hormones that are
produced by the neoplastic cells. The cell types, in order of decreasing frequency, are
the following: lactotrope adenomas (which secrete prolactin), null cell adenomas
(which do not secrete hormones), somatotrope adenomas (which secrete growth
hormone), corticotrophic adenomas (which secrete ACTH), gonadotrope adenomas
(which secrete FSH and LH), and thyrotrope cell adenomas (which secrete TSH).
Prolactin-secreting tumors (lactotrope adenomas or prolactinomas) produce
symptoms of hypogonadism and galactorrhea (milk secretion not associated with
pregnancy). In females this hypogonadism produces amenorrhea and infertility, while
in males it produces impotence and decreased libido. The same symptoms that are
seen with a prolactin-secreting pituitary adenoma can also be produced by certain
drugs, such as methyldopa and reserpine.
A 5-year-old boy presents with the acute onset of fever, chills, and severe, throbbing
pain over the metaphysis of his left femur. His peripheral leukocyte count is
increased, and an x-ray of his left femur reveals a lytic focus of bone surrounded by a
zone of sclerosis. Which of the following is the most likely diagnosis? *
1/1
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A 49-year-old man who smokes 2 packs of cigarettes a day presents with a lung
mass on x-ray and recent weight gain. Laboratory examination shows hyponatremia
with hyperosmolar urine. Which of the following is the most likely cause of these
clinical findings? *
1/1
. Renal failure
Pituitary failure
Conn syndrome
Cardiac failure
. Excess ADH
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Diffuse fibrous deposition between atrophic follicles
Correct answer
Lymphoid infiltrate with scattered Hurthle cells
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Hashimoto thyroiditis, one of the autoimmune thyroid diseases, is associated with the
HLA-B8 haplotype and high titers of circulating autoantibodies, including
antimicrosomal, antithyroglobulin, and anti-TSH receptor antibodies. This abnormality,
which is not uncommon in the United States, is characterized histologically by an
intense lymphoplasmacytic infiltrate, with the formation of lymphoid follicles and
germinal centers. This produces destruction and atrophy of the follicles and
transforms the thyroid follicular cells into acidophilic cells. There are many different
names for these cells, including oxyphilic cells, oncocytes, and Hürthle cells. Not
uncommonly, patients develop hypothyroidism as a result of follicle disruption, and
the manifestations consist of fatigue, myxedema, cold intolerance, hair coarsening,
and constipation. Rarely, cases of Hashimoto thyroiditis may develop hyperthyroidism
(Hashitoxicosis), while the combination of Hashimoto disease, pernicious anemia,
and type I diabetes mellitus is called Schmidt syndrome. This is one type of
multiglandular syndrome.
Although subacute thyroiditis and Riedel thyroiditis may have similar symptoms to
Hashimoto thyroiditis, biopsy findings in these disorders are distinctly different.
Subacute (de Quervain’s, granulomatous, or giant cell) thyroiditis is a self-limited viral
infection of the thyroid. It typically follows an upper respiratory tract infection. Patients
develop the acute onset of fever and painful thyroid enlargement and may develop a
transient hypothyroidism. Histologically, there is destruction of the follicles with a
granulomatous reaction and multinucleated giant cells that surround fragments of
colloid. One-half of patients with Riedel thyroiditis are hypothyroid, but, in contrast to
the other types of thyroiditis, microscopic examination reveals dense fibrosis of the
thyroid gland, often extending into extrathyroidal soft tissue. This fibrosis produces a
rock-hard enlarged thyroid gland that may produce the feeling of suffocation. This
combination of signs and symptoms may be mistaken clinically for a malignant
process. Additionally, these patients may develop similar fibrosis in the mediastinum
or retroperitoneum. Subacute lymphocytic thyroiditis is also a self-limited, painless
enlargement of the thyroid that is associated with hypothyroidism, but that lacks
antithyroid antibodies or lymphoid germinal centers within the thyroid. Finally, follicular
cell hyperplasia with scalloping of colloid is characteristic of hyperthyroidism due to
Graves disease, while the extracellular deposition of amyloid in the thyroid gland is
characteristic of medullary thyroid carcinoma
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BCL2
EWS
RAS
UBE3A
WT1
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0/2
Correct answer
Optically clear nuclei with longitudinal nuclear grooves
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THe correct answer is optically clear nuclei with longitudinal nuclear grooves
The four major histologic subtypes of thyroid carcinoma are papillary carcinoma,
follicular carcinoma, medullary carcinoma, and undifferentiated (anaplastic)
carcinoma. Papillary carcinomas of the thyroid are composed of papillary structures
with fibrovascular cores, while follicular carcinomas typically show a microfollicular
pattern. It is important prognostically to differentiate papillary carcinomas from
follicular carcinomas, as papillary carcinomas tend to be indolent (up to 80% survival
at 10 years), while follicular carcinomas are much more aggressive (5-year mortality
of up to 70%). Follicular areas may be present within a papillary carcinoma and in fact
may be quite extensive. If present, these changes can make diagnosis difficult. It is
important to recognize this follicular variant of papillary carcinoma because its
behavior remains similar to that of indolent papillary carcinoma. Features consistent
with papillary carcinoma, even in predominantly follicular areas, include optically clear
nuclei (“ground glass,” “Orphan Annie eyes”), nuclear grooves, calcospherites
(psammoma bodies), and intranuclear cytoplasmic pseudoinclusions.
In contrast to the histologic features of papillary carcinoma, follicular carcinoma of the
thyroid has a histology that is similar to a follicular adenoma, but capsular and blood
vessel invasion is present. Medullary carcinoma is characterized by its amyloid
stroma, its genetic (familial) associations, and its elaboration of calcitonin and other
substances. It is a malignancy that originates from the parafollicular C cells.
Undifferentiated (anaplastic) carcinoma, seen in individuals over the age of 50, is
characterized by anaplastic spindle or giant cells with frequent mitoses. This tumor is
characterized by rapid growth and a poor prognosis.
A 65-year-old man presents with bone pain and is found to have hypocalcemia and
increased parathyroid hormone. Surgical exploration of his neck finds all four of his
parathyroid glands to be enlarged. Which of the following disorders is the most likely
cause of this patient’s enlarged parathyroid glands? *
0/1
Primary hyperplasia
Parathyroid adenoma
Parathyroid carcinoma
Lung carcinoma
Correct answer
Chronic renal failure
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The correct answer is chronic renal failure
0/1
3-β-dehydrogenase
11-hydroxylase
17-hydroxylase
21-hydroxylase
1-α-hydroxylase
Correct answer
11-hydroxylase
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Ambiguous external genitalia can be seen in some female newborn infants with
congenital adrenal hyperplasia (CAH), a syndrome that results from a defect in the
synthesis of cortisol. This leads to excess ACTH secretion by the anterior pituitary and
resultant adrenal hyperplasia. The defect in the synthesis of cortisol is the result of a
deficiency in one of the enzymes in the normal pathway of cortisol synthesis, such as
21-hydroxylase or 11-hydroxylase.
A deficiency of 11-hydroxylase, which is rare, leads to decreased cortisol production
and increased ACTH secretion. This in turn leads to the accumulation of
deoxycorticosterone (DOC) and 11-deoxycortisol, both of which are strong
mineralocorticoids. This results in increased sodium retention by the kidneys and
hypertension. Patients also lose potassium and hydrogen ions in the urine, which
leads to a hypokalemia metabolic alkalosis. Because of the enzyme block there is
increased formation of 17-hydroxyprogesterone, which is then shunted into the
production of testosterone. This may cause virilism (pseudohermaphroditism) in
female infants. That is, XX females with CAH develop ovaries, female ductal
structures, and external male genitalia.
Most cases of CAH, however, result from a deficiency of 21-hydroxylase. Two forms
of this deficiency include salt-wasting adrenogenitalism and simple virilizing
adrenogenitalism. The salt-wasting syndrome results from a complete lack of the
hydroxylase. There is no synthesis of mineralocorticoids or glucocorticoids in the
adrenal cortex. Decreased mineralocorticoids cause marked sodium loss in the urine,
hyponatremia, hyperkalemia, acidosis, and hypotension.
Much more often there is only a partial deficiency of 21-hydroxylase, which leads to
decreased production of both aldosterone and cortisol. The decreased cortisol levels
cause increased production of ACTH by the pituitary, which results in adrenal
hyperplasia, enough to maintain adequate serum levels of aldosterone and cortisol. In
contrast to a complete deficiency of 21-hydroxylase, there is no sodium loss with a
partial deficiency of 21-hydroxylase. The excess stimulation by ACTH, however, leads
to increased production of androgens, which may cause virilism in female infants.
Patients with a deficiency of 17-hydroxylase also exhibit impaired cortisol production,
increased ACTH, and secondary increased DOC. These patients, however, cannot
synthesize normal amounts of androgens and estrogens. This is because the gene
that codes for 17-hydroxylase is the same for the enzyme in the adrenal cortex and
the gonads, and the deficiency is the same in both organs. Because of decreased sex
hormones, genotypic females develop primary amenorrhea and fail to develop
secondary sex characteristics, while genotypic males present as
pseudohermaphrodites. Additionally, the plasma LH levels are increased due to
decreased feedback inhibition.
A 35-year-old man who presents with a neck mass is found to have a serum calcium
level of 11.8 mg/dL and periodic elevation of his blood pressure. Extensive workup
reveals the presence of a medullary carcinoma of the thyroid, a pheochromocytoma,
and hyperplasia of the parathyroid glands. Which of the following is the most likely
diagnosis? *
1/1
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A 51-year-old woman presents with problems seeing and drooping eyelids. She had
been diagnosed 5 years prior as having lupus. A chest x-ray reveals a mass in the
anterior mediastinum, which is confirmed by a CT scan. Surgical exploration finds a
well-encapsulated tumor. No evidence of invasion is seen. The mass is resected,
and histologic sections reveal scattered lymphocytes within a diffuse proliferation of
spindle-shaped cells as shown in this image. Which of the following is the cell of
origin of this tumor? *
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Thymomas are tumors arising from thymic epithelial cells and are among the most
common mediastinal neoplasms, especially in the anterosuperior mediastinum.
Histologic sections reveal a proliferation of spindle-shaped cells. There is a scanty or
rich lymphocytic infiltrate of T cells, which are not neoplastic, although their size and
prominent nucleoli may cause histologic confusion with lymphoma. About 90% of
thymomas are benign and occur at a mean age of 50 years. They are very rare in
children. They may be asymptomatic or may cause pressure effects of dysphagia,
dyspnea, or vena cava compression. Associated systemic disorders include
myasthenia gravis, hematologic cytopenias, collagen vascular disease (lupus), and
hypogammaglobulinemia. Malignant thymomas show infiltration and capsular
invasion plus pleural implants or distant metastasis.
In contrast, the other type of cell found in the thymus, T lymphocytes, give rise to
T-cell lymphoblastic lymphomas. Finally, recall that fibroblasts give rise to fibromas or
fibrosarcomas, and mesothelial cells give rise to benign or malignant mesotheliomas.
A recent 59-year-old immigrant to the United States presents with back pain along
with fever and weight loss. Workup finds a lesion of the lower spinal cord lesion that
destroys both the bone and the disk space (cartilage). What is the most likely cause
of this abnormality? *
0/1
Metastatic carcinoma
Multiple myeloma
Non-Hodgkin lymphoma
Syringomyelia
Tuberculosis
Correct answer
Tuberculosis
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A 59-year-old woman presents with difficulty swallowing, ptosis, and diplopia. Which
of the following is the most likely cause of this woman’s disease? *
1/1
Rhabdomyolysis
Corticosteroid therapy
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A 73-year-old woman presents with progressive memory loss. Her family relates
several recent episodes where she left home by herself, got lost in her own
neighborhood, and could not find her way back. Neurologic examination was
unremarkable except for severe cognitive deficits. Within 2 years she had to be
admitted to a nursing home, where she died 3 years later. Autopsy revealed bilateral,
symmetrical atrophy of the frontal lobes with wide sulci and narrow gyri. Microscopic
examination of tissue taken from this area revealed bundles of filaments in the
cytoplasm of neurons and focal collections of neuritic processes surrounding central
amyloid cores. What is the main composition of these amyloid cores? *
1/1
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Alzheimer disease (AD) is the most common cause of dementia in elderly (followed by
vascular multi-infarct dementia and diffuse Lewy body disease). AD often begins
insidiously with impairment of memory and progresses to dementia. Histologically, AD
is characterized by numerous neurofibrillary tangles and senile plaques with a central
core of β-amyloid. The gene for β-amyloid (A-beta) is located on chromosome 21
(note the high incidence of Alzheimer disease in individuals with trisomy 21).
Cleavage of the β-amyloid precursor protein (β-APP) by alpha-secretase precludes
beta-A formation; but cleavage of β-APP at a site N-terminal to the start of the
transmembrane domain by beta-secretase (BACE-1) or within the transmembrane by
gamma-secretase produces fragments that tend to aggregate into the pathogenic
amyloid fibrils. β-Amyloid deposition is necessary but not sufficient for the
development of Alzheimer disease.
Both tangles and plaques are found to a lesser extent in other conditions, for example,
neurofibrillary tangles in Down syndrome. Silver stains demonstrate tangles and
plaques and Congo red shows amyloid deposition in plaques and also vascular walls
(amyloid angiopathy). In AD there are also numerous Hirano bodies, and
granulovacuolar degeneration is found in more than 10% of the neurons of the
hippocampus. Grossly, brain atrophy (narrowed gyri and widened sulci) is
predominant in the frontal and superior temporal lobes.
38-year-old woman presents with increasing frequency of severe headaches. The
previous day she had a seizure that lasted several minutes. Her past medical history
is otherwise unremarkable, and she has no previous history of seizure activity. She is
admitted to the hospital and a CT scan of her head finds a 2-cm mass attached to the
dura in her right frontal area. Which of the following histologic changes is most likely
to be seen in a biopsy specimen taken from this tumor? *
1/1
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A tumor that is attached to the dura is most likely to be a meningioma. This type of
tumor arises from the arachnoid villi of the brain or spinal cord. Although they usually
occur during middle or later life, a small number occur in persons 20 to 40 years of
age. They commonly arise along the venous sinuses (parasagittal, sphenoid wings,
and olfactory groove). Although meningiomas are benign and usually slow growing,
some have progesterone receptors and rapid growth in pregnancy occurs
occasionally. The rare malignant meningioma may invade or even metastasize. The
typical case, however, does not invade the brain, but displaces it, causing headaches
and seizures. Histologically, many different patterns can be seen, but psammoma
bodies and a whorled pattern of tumor cells are somewhat characteristic. In contrast
to this histologic appearance, Antoni A areas with Verocay bodies are seen in
schwannomas, endothelial proliferation and serpentine areas of necrosis are seen in
glioblastoma multiformes, a “fried-egg” appearance of tumor cells is characteristic of
oligodendrogliomas, and true rosettes and pseudorosettes can be seen in
medulloblastomas.
After recovering from a viral respiratory tract infection, a 23-year-old woman presents
with weakness in her distal extremities that rapidly ascends to involve proximal
muscles. Physical examination reveals absent deep tendon reflexes, and a lumbar
puncture reveals the CSF protein to be increased, but very few cells are present. A
biopsy of a peripheral nerve reveals inflammation and demyelination
(radiculoneuropathy). Which of the following is the most likely diagnosis? *
1/1
Brown-Séquard syndrome
Charcot-Marie-Tooth disease
Diabetes mellitus
. Guillain-Barré syndrome
Syringomyelia
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An 18-year-old male high school baseball player gets hit in the head with a fastball in
the temporal area. He does not lose consciousness, but afterward develops a slight
headache. He is not taken to the emergency room. By evening he develops severe
headache with vomiting and confusion. At that time he is taken to the emergency
room, where, after being examined by a neurosurgeon, he is taken to the operating
room for immediate surgery for an epidural hematoma. Which of the following is most
likely present in this individual? *
0/1
Transection of a branch of the middle meningeal artery
Correct answer
Transection of a branch of the middle meningeal artery
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Epidural hemorrhages result from hemorrhages into the potential space between the
dura and the bone of the skull. These hemorrhages result from severe trauma that
typically causes a skull fracture. The hemorrhage results from rupture of one of the
meningeal arteries, as these arteries supply the dura and run between the dura and
the skull. The artery involved is usually the middle meningeal artery, which is a branch
of the maxillary artery, as the skull fracture is usually in the temporal area. Since the
bleeding is of arterial origin (high pressure), it is rapid and the symptoms are rapid in
onset, although the patient may be normal for several hours (lucid interval). Bleeding
causes increased intracranial pressure and can lead to tentorial herniation and death.