Phyllodes Tumor
Phyllodes Tumor
Phyllodes Tumor
• Phyllodes tumors are rare fibroepithelial lesions (0.3 to 0.5% of female breast tumors), incidence of about 2.1 per million
• Peak of which occurs in women aged 45 to 49 years, rarely found in adolescents and the elderly.
• The majority of phyllodes tumors have been described as benign (35% to 64%), with the remainder divided between the
borderline and malignant subtypes.
• The term phyllodes tumor represents a broad range of fibroepithelial diseases and presence of an epithelial component with
stromal components differentiates the phyllodes tumor from other stromal sarcomas.
• Accurate preoperative pathological diagnosis allows correct surgical planning and avoidance of reoperation, either to achieve
wider excision or for subsequent tumor recurrence.
• Malignant phyllodes tumors, if inadequately treated, have a propensity for rapid growth and metastatic spread.
• Benign phyllodes tumors on clinical, radiological, and cytological examination are often indistinguishable from fibroadenomas
and can be cured by local surgery.
• With the nonoperative management of fibroadenomas widely adopted, the importance of phyllodes tumors today lies in the need
to differentiate them from other benign breast lesions.
• Treatment can be either wide local excision or mastectomy provided histologically clear specimen margins are ensured.
ETIOLOGY
• At present time, the exact etiology of phyllodes tumor and its relationship with
fibroadenoma are unclear.
• Most fibroadenomas have polyclonal elements and should be regarded as hyperplasic
rather than neoplastic lesions
• It has also been postulated that stromal induction of phyllodes tumors can occur as a
result of growth factors produced by the breast epithelium.
• Trauma, lactation, pregnancy, and increased estrogen activity occasionally have been
implicated as factors stimulating tumor growth.
• The nature of these factors is unclear but endothelin-1, a stimulator of breast
fibroblast growth, may be important.
PATHOGENESIS
• Unlike carcinoma breast, phyllodes tumors start outside of the ducts and
lobules, in the breast’s connective (stroma) which includes the fatty tissue and
ligaments that surround the ducts, lobules, and blood and lymph vessels in the
breast.
• In addition to stromal cells, phyllodes tumors can also contain cells from the
ducts and lobules.
CLASSIFICATION
DIAGNOSIS
Clinical Manifestations:
• Most of the tumor arises in women aged between 35 - 55 years (approximately 20
years later than fibroadenoma),more prevalent in the Latin American white and Asian
populations.
• Few cases have been reported in men.
• It usually presents as a rapidly growing but clinically benign breast lump.
• In some patients a lesion may have been apparent for several years, with clinical
presentation precipitated by a sudden increase in size.
• The skin over large tumors may have dilated veins and a blue discoloration but nipple
retraction is rare.
• Fixation to skin and pectoralis muscles has been reported, but ulceration is uncommon.
• More commonly found in upper outer quadrant with an equal propensity to occur in
either breast.
• Rarely presentation may be bilateral.
• The median size of phyllodes tumors is around 4 cm. 20% of tumors grow larger than
10 cm (giant phylodes tumor). These tumors can reach sizes up to 40 cm in diameter
• A significant proportion of patients have history of fibroadenoma and in a minority
these have been multiple.
• Palpable axillary lymphadenopathy can be identified in up to 10–15% of patients but
<1% had pathological positive nodes.
DIAGNOSIS
• Radiological Investigations
-Mammography and ultrasonography are mainstay of routine imaging of breast
lumps.
-Round or lobulated shape, well-defined margins, heterogeneous internal
structure, and nonenhancing internal septations are more common findings in
phyllodes tumors than in fibroadenomas.
DIAGNOSIS
• Radiological Investigations
b. Mammography
(i) It shows well circumscribed oval or lobulated mass with rounded borders.
(ii) A radiolucent halo may be seen around the lesion due to compression of the
surroundings.
(iii) Coarse calcification (but malignant microcalcifica- tion is rare) may be
present.
DIAGNOSIS
• Radiological Investigations
c. MRI
(i) round or lobulated shape and well-defined margins,
(ii) heterogeneous internal structure/nonenhancing sep- tations,
(iii) exhibits hypointense signals on T1-weighted images,
(iv) exhibits hyper/isointense signals on T2-weighted images,
(v) contrast enhancement pattern:
(a) benign lesion:
-slow initial enhancement with persistent delayed phase;
(b) malignant lesion:
-fast initial enhancement with plateau phage,
-fast initial enhancement with wash-out phenomenon.
DIAGNOSIS
Pathological/Histological Assessment
• As both phyllodes tumors and fibroadenomas belong to a spectrum of fibroepithelial lesions, accurate cytological diagnosis
of phyllodes tumors by fine needle aspiration can be difficult.
• Cytologically, it is often easier to differentiate benign from malignant phyllodes tumors than to separate benign phyllodes
tumors from fibroadenomas.
• In the correct clinical setting, the presence of both epithelial and stromal elements within the cytological smear supports the
diagnosis.
• Epithelial cells may, however, be absent from specimens taken from malignant lesions.
• The presence of cohesive stromal cells (phyllodes fragments), isolated mesenchymal cells, clusters of hyperplastic duct
cells, foreign body giant cells, blood vessels crossing the stromal fragments, and bipolar naked nuclei and the absence of
apocrine metaplasia are highly suggestive of a phyllodes tumor.
• Value of FNAC in the diagnosis of phyllodes tumor, overall accuracy of about 63%
• Core tissue biopsy is an attractive alternative to FNAC because of the extra architectural information provided by histology
compared with cytology (sensitivity 99%, negative predictive value 93 % and positive 83%)
DIAGNOSIS
Macroscopic Appearance
• Macroscopically most small tumors have a uniform white consistency with a lobulated surface, similar to that
of a fibroadenoma.
• Large tumors on cut section often have a red or grey “meaty” consistency with fibrogelatinous, hemorrhagic,
and necrotic areas with leaf like protrusions into the cystic spaces.
DIAGNOSIS
Microscopic Appearance
• Microscopic Appearance Fine Needle Aspiration Cytology.
• The cytological diagnosis of phyllodes tumors is mainly suggested by the presence of hypercellular stroma
and the stromal elements on the smears being more numerous than the epithelial ones. The cells on the
smears by comparison with small lymphocytes, in :
(1) short, round/oval cells, two-size smaller than the size of a lymphocyte: considered to be epithelial cells;
(2) long, spindle cells, three-size larger than the size of a lymphocyte: considered to be stromal cells.
DIAGNOSIS
Many authors considered that the following aspects should also be taken into consideration in the case of
cytological diagnosis of phyllodes tumors:
(a) the presence of hypercellular stromal fragments;
(b) the cellular composition of the stromal fragments;
(c) the amount of naked nuclei on the background of the smears;
DIAGNOSIS
RECURRENCE
• Recurrence. To date, local recurrence rates ranging from 10% to 40% have
been reported with most series averaging about 15%.
• Local recurrence appears to be related to the extent of the initial surgery and
should be regarded as a failure of primary surgical treatment.
• Whether malignant tumors have an increased risk of recurrence is unclear but
when it does occur it is invariably seen earlier than with benign tumors.
METASTASIS
• Overall, 10% of patients with phyllodes tumors develop distant metastases and
these eventually occur in approximately 25% of patients with histologically
malignan
• The commonest sites for distant metastases are the lungs (66%), bones (28%),
and brain (9%) and in rare instances, the liver and heart
FOLLOWUP.
• Since phyllodes tumors are locally recurrent tumor especially when not excised
with a clear margins and very unpredictable in growth and metastatic activity
• it is very necessary to follow up the patient regularly at 6-month interval for the
first two years (chances of recurrence are maximum in the first two years) and
then on yearly basis.
• Patients must be instructed to self examine her breast regularly and consult her
doctor, if any abnormality detected. In followup, patient should be examined
and, if any abnormality detected, it should be investigated with USG,
mammogram, MRI, or tissue biopsy
CONCLUSION