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Cystosarcoma Phyllodes

Cystosarcoma phyllodes is a rare, predominantly benign tumor that occurs almost exclusively in the
female breast.[1] Its name is derived from the Greek wordssarcoma, meaning fleshy tumor, and phyllo,
meaning leaf. Grossly, the tumor displays characteristics of a large, malignant sarcoma, takes on a
leaflike appearance when sectioned, and displays epithelial, cystlike spaces when viewed histologically.
Because most tumors are benign, the name may be misleading. Thus, the favored terminology is now
phyllodes tumor. Phyllodes tumor is the most commonly occurring nonepithelial neoplasm of the breast,
although it represents only about 1% of tumors in the breast.[2] It has a smooth, sharply demarcated
texture and typically is freely movable. It is a relatively large tumor, with an average size of 5cm.
However, lesions of more than 30cm have been reported. The etiology of phyllodes tumors is unknown.
While most phyllodes tumors are benign, the possibility exists for underestimating their potential for
malignancy. Moreover, some juvenile fibroadenomas in teenagers can look histologically like phyllodes
tumors; however, they behave in a benign fashion similar to that of other fibroadenomas. The difficulty in
distinguishing between fibroadenoma, benign phyllodes tumors, and malignant cystosarcoma phyllodes
may be vexing for even the most experienced pathologist.
Unlike breast cancers called carcinomas, which develop insidethe ducts (milk-carrying tubes) or lobules
(milk-producing glands) of the breast, phyllodes tumors start outside of the ducts and lobules. Phyllodes
tumors develop in the breast's connective tissue, called the stroma. The stroma includes the fatty tissue
and ligaments that surround the ducts, lobules, and blood and lymph vessels in the breast. It may be
helpful to think of the stroma as the tissue that "holds everything together" inside the breast. In addition to
stromal cells, phyllodes tumors can also contain cells from the ducts and lobules .

Sex- and age-related demographics

Phyllodes tumors occur almost exclusively in females, although rare case reports have been described in
males. The tumors can develop in people of any age; however, the median age is the fifth decade of life.

Patient education
As with all breast neoplasms, self-examination remains the most important initial mechanism for the
detection of phyllodes tumors. Appropriate teaching of this procedure is vital for early detection of all
breast neoplasms.

Benign Versus Malignant Phyllodes Tumors

Because of limited data, the percentage of benign versus malignant phyllodes tumors is not well defined.
Reports suggest, however, that about 85-90% of phyllodes tumors are benign and that approximately 1015% are malignant.[4]
Although the benign tumors do not metastasize, they have a tendency to grow aggressively and can recur
locally.[2] Similar to other sarcomas, the malignant tumors metastasize hematogenously. Unfortunately, the
pathologic appearance of a phyllodes tumor does not always predict the neoplasm's clinical behavior; in
some cases, therefore, there is a degree of uncertainty about the lesion's classification.
The characteristics of a malignant phyllodes tumor include the following[5] :

Recurrent malignant tumors seem to be more aggressive than the original tumor
The lungs are the most common metastatic site, followed by the skeleton, heart, and liver
Symptoms of metastatic involvement can arise from as early as a few months to as late as 12 years
after the initial therapy
Most patients with metastases die within 3 years of the initial treatment[6]
No cures for systemic metastases exist
Roughly 30% of patients with malignant phyllodes tumors die from the disease

History and Physical Examination

Phyllodes tumors generally manifest as larger masses and display rapid growth. A small mass may
rapidly increase in size in the few weeks before the patient seeks medical attention. Tumors rarely involve
the nipple-areola complex or ulcerate to the skin. Patients with metastases may present with such
symptoms as dyspnea, fatigue, and bone pain.

Physical examination
A firm, mobile, well-circumscribed, nontender breast mass is appreciated. Curiously, cystosarcoma
phyllodes tends to involve the left breast more commonly than the right one. Overlying skin may display a
shiny appearance and be translucent enough to reveal underlying breast veins. Physical findings (ie, the
occurrence of mobile masses with distinct borders) are similar to those of fibroadenoma. [3]

Differential Diagnosis
Conditions to consider in the differential diagnosis of phyllodes tumors include the following:

Giant fibroadenoma
Radial scar
Breast abscess

*Breast cancer
*Inflammatory carcinoma
*Fat necrosis

*Juvenile fibroadenoma
*Sclerosing adenosis
*Fibrocystic change

Biopsy and Histology

Open excisional breast biopsy for smaller lesions and incisional biopsy for large lesions are the definitive
methods for diagnosing phyllodes tumors.
Fine-needle aspiration for cytologic examination usually is inadequate for the diagnosis of phyllodes
tumors. Core biopsy is more reliable, but there still can be sampling errors and difficulty in distinguishing
the lesion from a fibroadenoma.
All phyllodes tumors contain a stromal component that can vary significantly in histologic appearance
from one lesion to another.[7] In general, benign phyllodes tumors demonstrate a markedly increased
number of regular fusiform fibroblasts in the stroma. Occasionally, highly anaplastic cells with myxoid
changes are observed. A high degree of cellular atypia, with increased stromal cellularity and an
increased mitotic count, is almost always observed in the malignant form of cystosarcoma phyllodes.
Ultrastructurally, in the benign and malignant forms of phyllodes tumors, nucleoli may reveal a coarsely
meshed nucleolonema and abundant cisternae in the endoplasmic reticulum.

ab and Imaging Studies

Lab studies
No specific hematologic tumor markers or other blood tests can be used to diagnose cystosarcoma

Imaging studies
Although mammography and ultrasonography generally are important in the diagnosis of breast lesions,
they are notoriously unreliable in differentiating benign cystosarcoma phyllodes from the malignant form
of the condition or from fibroadenomas. (The phyllodes tumors mammographic appearance, as a round
density with smooth borders, is similar to that of fibroadenoma.) Thus, findings on imaging studies are not
definitively diagnostic of phyllodes tumors.[8]

Tumor Excision and Mastectomy

Complete excision, with accurate histologic examination and continued follow-up care, is the best way to
treat phyllodes tumors. In most cases of cystosarcoma phyllodes, perform wide local excision, with a rim

of normal tissue included.[9, 10, 11] No absolute rules on margin size exist. However, a 2cm margin for small
(< 5cm) tumors and a 5cm margin for large (>5cm) tumors have been advocated.
The lesion should not be "shelled out," as might be done with a fibroadenoma, or the recurrence rate will
be unacceptably high.[6]
If the tumor-to-breast ratio is sufficiently high to preclude a satisfactory cosmetic result by segmental
excision, total mastectomy, with or without reconstruction, is an alternative. More radical procedures are
generally not warranted.[10]
Perform axillary lymph node dissection only for clinically suspicious nodes. However, virtually all
of these nodes are reactive Consultations
Consult a general surgery specialist.

Adjuvant therapy
There is no proven role for adjuvant chemotherapy or radiation therapy in the treatment of phyllodes
tumors. Response to chemotherapy and radiotherapy for recurrences and metastases has been poor,
and no success with hormonal manipulation has been documented.

Postoperative complications
As with most breast surgery, postoperative complications from the surgical treatment of phyllodes tumors
include the following:

Seroma formation
Local and/or distant recurrence

If the tumor is benign, the long-term prognosis is excellent following adequate local excision. However,
the possibility for local recurrence following excision always exists, particularly with lesions that display
malignant histology.[11, 13] If the tumor recurs locally after excision, further local excision or
total mastectomy is typically curative. Metastatic disease is typically observed in the lung, mediastinum,
and skeleton.[5]e and do not contain malignant cells.[12]

Outpatient Care
Although specific guidelines regarding follow-up care for phyllodes tumors are limited because of the
rarity of these lesions, regular, long-term follow-up care should be performed to detect possible local
recurrences.An initial visit 1-2 weeks after surgery to detect any initial complications should be followed
by periodic visits as determined by the patient's surgeon. A reasonable schedule might be physical
examinations every 6 months and mammograms yearly for at least 5 years. Carefully observe patients for
any possible recurrence.