Cystic Hygroma
Cystic Hygroma
Cystic Hygroma
structures)
CLINICAL FEATURES
Sex and Age Distribution
■ No significant sex difference
Approximately two-thirds of lymphangiomas are noted
■ Most present shortly after birth and 95% by the second year
shortly after birth, and 95% are present by the end
of the second year of life. Cystic hygroma may also be Clinical Features
detected in utero with ultrasonography. Cystic hygroma ■ Slowly enlarging painless mass
may be associated with fetal hydrops and several genetic ■ May produce pressure symptoms owing to its size
abnormalities, most notably Turner syndrome (Fig. ■ Associated with fetal hydrops and Turner syndrome
A B
C
FIGURE 21.1
(A) Bilateral cystic masses are noted in
the posterior neck of this fetus, affected
with Turner syndrome. (B) Turbid-milky
fluid removed during a fine needle
aspiration of a large posterior neck cyst
in a child (lymphangioma). (C) A
translucent mass, showing delicate
vessels in the wall of a lymphangioma,
filled with clear-watery fluid.
A B
FIGURE 21.2
(A) Cystic lymphangioma showing dilated lymphatic spaces. (B) Lymphocytes are noted within the proteinaceous fluid.
A B
C D
FIGURE 21.3
(A) Lymphatic spaces subtended by smooth muscle. (B) Lymphoid cells and proteinaceous fluid. (C) Lymphoid elements with fibrosis surrounding fluid-filled
spaces. (D) Flat, attenuated endothelial cells line the cavity.
Microscopic Findings
DIFFERENTIAL DIAGNOSIS
■ Dilated, thin-walled spaces filled with proteinaceous fluid
the spaces, but TTF1 and/or thyroglobulin will be positive. CD9 and LYVE-1, although less common)
Furthermore, a lymph node architecture should be rec-
Pathologic Differential Diagnosis
ognizable, often at the periphery.
■ Cavernous hemangioma, metastatic papillary thyroid carcinoma
Mortality rates are between 3% and 7%, specifically treatment or injected sclerosing agents (such as bleomycin)
related to pressure destruction of vital structures of the are alternative therapies. Recurrence rates range from
neck. Lymphangiomas may occasionally become infected 15% to 50% and are highest when the lymphangioma
and may cause difficulty swallowing or respiratory dis- is incompletely removed. Malignant transformation is
tress. Surgery is the treatment of choice, while laser not documented.
512 HEAD AND NECK PATHOLOGY
as benign or malignant depending on the degree of tissue ectoderm, endoderm, and mesoderm
maturation. In general, teratomas of the neck in neonates
or infants are clinically benign, although they may be Incidence and Location
■ Cervical teratomas represent 3% of all teratomas
histologically mature or immature. By comparison, tera-
■ Less than 1% of pediatric neck masses
tomas in adults are more likely to be clinically malignant
and histologically immature.
Morbidity and Mortality
■ High morbidity but low mortality in neonates and infants
CLINICAL FEATURES
Sex and Age Distribution
■ Equal sex distribution
promise and requiring immediate surgery. Polyhydramnios ■ Polyhydramnios seen in 20% of neonatal lesions
A B
FIGURE 21.4
Magnetic resonance imaging demon-
strates a large mass involving the
oropharynx, nasopharynx, and neck. (A)
Axial T2-weighted image. (B) Coronal
T1-weighted image.
CHAPTER 21 Benign Neoplasms of the Neck (Soft Tissue, Bone, and Lymph Node) 513
FIGURE 21.5
This benign, mature teratoma contains a
primitive esophagus adjacent to a primitive
trachea. Elements from other germ cell
layers were noted elsewhere.
A B
FIGURE 21.6
(A) Teratoma containing neuroglial tissue with focal fibrosis. (B) Adjacent focus showing ependymal and choroid plexus elements.
514 HEAD AND NECK PATHOLOGY
A B
C D
FIGURE 21.7
(A) Retinal anlage epithelium. (B) Mature cartilage adjacent to pancreatic tissue. (C) Thyroid parenchyma with immature fat. (D) A rosette of immature glial
tissue.
Microscopic Findings
■ Mature or immature tissues from all germ cell layers
ANCILLARY STUDIES
■ Squamous, respiratory, glandular, and cuboidal epithelium
MICROSCOPIC FINDINGS or clear cell differentiation (Figs. 21.10 and 21.11; see
also Fig. 21.9). Occasionally, focal smooth muscle and
EHTs are composed of a mix of spindle and epithelial psammoma bodies have been reported. There is no
cells, with islands of mature adipose tissue (Fig. 21.9). The cytologic atypia, mitotic activity is very low, and necrosis
spindle cells are an admixture of plump and delicate cells; is absent. Rare cases with malignant transformation are
the epithelial component may show squamous, glandular, documented.
FIGURE 21.9
Tumor is composed of spindle cells
entrapping epithelial and mature adipose
tissue components.
FIGURE 21.10
Spindle cell component with fascicular
arrangement; epithelial component with
glands and cystic structures.
CHAPTER 21 Benign Neoplasms of the Neck (Soft Tissue, Bone, and Lymph Node) 517
FIGURE 21.11
Epithelial component showing interanas-
tomotic strands of squamous cells.
DIFFERENTIAL DIAGNOSIS
Microscopic Findings
■ Triphasic histology: spindle cells (predominant, bland, plump to
thin); epithelioid nests and cords haphazardously arranged The differential diagnosis includes mixed tumors of skin
(squamous, glandular, clear cells); mature adipocytes adnexal or salivary gland origin (EHT lacks chondroid/
■ No immature T cells
cartilaginous foci), spindle cell/pleomorphic lipoma
■ No evidence of thymic differentiation
(CD34-positive), sarcomatoid carcinoma (cytologically
Ancillary Studies malignant), synovial sarcoma, cystic teratoma (the origin
■ Smooth muscle actin-, keratin-, p63-positive is deep-seated), and glandular malignant peripheral nerve
■ Occasional CD34 positivity sheath tumor (patchy S100 protein and strong SOX10
positivity).
Pathologic Differential Diagnosis
■ Mixed tumors of skin adnexal or salivary gland origin, spindle
ANCILLARY STUDIES
■ SPINDLE CELL LIPOMA/
PLEOMORPHIC LIPOMA
IMMUNOHISTOCHEMICAL FINDINGS
The spindled areas display strong and diffuse positivity Spindle cell and pleomorphic lipomas are distinctive types
for cytokeratin and epithelial membrane antigen. Myo- of lipoma histologically on a continuum and characterized
epithelial phenotype with the coexpression of keratins by replacement of mature fat cells by bland spindle cells,
and smooth muscle actin has been also reported. The hyperchromatic round cells, and multinucleated giant
518 HEAD AND NECK PATHOLOGY
CLINICAL FEATURES
SPINDLE CELL LIPOMA/PLEOMORPHIC LIPOMA—DISEASE
FACT SHEET
More than 90% of spindle cell/pleomorphic lipomas occur
in men (men outnumbering women 10 : 1), with a peak Definition
■ A distinct group of lipomas composed of spindle cells,
incidence in the 6th decade. Almost all of the tumors
adipocytes, and multinucleated giant cells associated with ropy
are located in the subcutaneous tissue of the posterior
collagen
neck, upper back, and shoulder girdle. Rarely, the salivary
gland, lip, tongue, and maxillofacial region will be affected. Incidence and Location
Patients are usually asymptomatic but may present with ■ About 1.5% of all adipose tissue tumors
a painless, mobile, subcutaneous mass that has often ■ Subcutaneous tissues of posterior neck and upper back most
Clinical Features
PATHOLOGIC FEATURES ■ Painless, subcutaneous, mobile mass
■ Seldom multiple
GROSS FINDINGS
Prognosis and Treatment
Spindle cell/pleomorphic lipomas range in size from
■ Excellent, although there are isolated reports of recurrence
1 to 13 cm (mean, 5 cm). Grossly, they resemble an ■ Surgery
ordinary lipoma (Fig. 21.12), although some are gray-white
or pale-pink, and deeper tumors may be myxoid.
FIGURE 21.12
Well-circumscribed and encapsulated,
intramuscular spindle cell lipoma. Note
the myxoid appearance from low power
as well as areas of increased cellularity.
CHAPTER 21 Benign Neoplasms of the Neck (Soft Tissue, Bone, and Lymph Node) 519
FIGURE 21.13
Spindle cell lipoma with bland spindle
cells in a background of adipocytes. Note
the areas of thick, ropy collagen fibers.
A B
FIGURE 21.14
(A) Marked myxoid stroma with delicate collagen fibers and fibroblasts in a spindle cell lipoma. (B) Pleomorphic lipoma: Hyperchromatic nuclei are seen in
cells separated by dense collagen. There are a few floret-like (“petal”) multinucleated giant cells associated with mast cells and collagenized stroma.
spindle cells admixed with wire- or rope-like collagen At the opposite end of the spectrum lies pleomorphic
fibers (“ropy collagen”) and myxoid stroma (Figs. 21.13 lipoma, which is characterized by small, round hyper-
and 21.14). The fibroblast-like cells may be arranged chromatic cells and multinucleated giant cells with radially
in a parallel fashion. Mast cells are frequently numer- arranged “floret-like” nuclei—so named for their resem-
ous. It is important to note that fat may be sparse blance to the petals of a flower (Fig. 21.14). Cases with
and difficult to find in selected tumors. The myxoid mixed features of spindle cell and pleomorphic lipoma
stroma may be a dominant finding. Profound but focal occur quite often, making any distinction impossible and
nuclear pleomorphism can be seen and is considered arbitrary. Secondary changes of fat necrosis or hyaliniza-
“degenerative” or “ancient change.” Mitoses are usually tion can be seen and may also be associated with “aging”
absent. or “ancient” change. By definition, lipoblasts are absent.
520 HEAD AND NECK PATHOLOGY
Microscopic Findings
■ Mixture of bland spindle cells arranged in parallel, adipocytes,
Smears will contain large, atypical, floret-type cells Complete local excision is curative for both types of
within a background of mature adipocytes. Needless to lipomas.
say the cells are frequently interpreted to be malignant.