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									Cystosarcoma Phyllodes                                           Race
Introduction                                                     A racial predilection does not appear to exist for phyllodes
Background                                                       tumors.
Cystosarcoma phyllodes is a rare, predominantly benign           Sex
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tumor that occurs almost exclusively in the female breast. Its   Phyllodes tumors occur almost exclusively in females. Rare
name is derived from the Greek words sarcoma, meaning            case reports have been described in males.
fleshy tumor, and phyllo,meaning leaf. Grossly, the tumor        Age
displays characteristics of a large, malignant sarcoma, takes    Phyllodes tumors can occur in people of any age; however,
on a leaflike appearance when sectioned, and displays            the median age is the fifth decade of life.
epithelial, cystlike spaces when viewed histologically (hence    Some juvenile fibroadenomas in teenagers can look
the name). Because most tumors are benign, the name may          histologically like phyllodes tumors; however, they behave in
be misleading. Thus, the favored terminology is now              a benign fashion similar to that of other fibroadenomas.
phyllodes tumor.                                                 Clinical
Pathophysiology                                                  History
Phyllodes tumor is the most commonly occurring                   1. Patients typically present with a firm, mobile, well-
nonepithelial neoplasm of the breast, although it represents          circumscribed, nontender breast mass.
                                            2
only about 1% of tumors in the breast. It has a smooth,          2. A small mass may rapidly increase in size in the few
sharply demarcated texture and typically is freely movable. It        weeks before the patient seeks medical attention.
is a relatively large tumor, with an average size of 5 cm.       3. Tumors rarely involve the nipple-areola complex or
However, lesions of more than 30 cm have been reported.               ulcerate to the skin.
Frequency                                                        4. Patients with metastases may present with such
United States                                                         symptoms as dyspnea, fatigue, and bone pain.
No difference in phyllodes tumor frequency appears to exist      Physical
between patients from the United States and those from           1. A firm, mobile, well-circumscribed, nontender breast
other countries. Phyllodes tumors account for approximately           mass is appreciated.
                            2
1% of all breast neoplasms.                                      2. Curiously, cystosarcoma phyllodes tends to involve the
Mortality/Morbidity                                                   left breast more commonly than the right one.
Because of limited data, the percentage of benign vs             3. Overlying skin may display a shiny appearance and be
malignant phyllodes tumors is not well defined. Reports               translucent enough to reveal underlying breast veins.
suggest, however, that about 85-90% of phyllodes tumors are      4. Physical findings (ie, the occurrence of mobile masses
                                                       3
benign and that approximately 10-15% are malignant.                   with distinct borders) are similar to those of
                                                                                       5
Although the benign tumors do not metastasize, they have a            fibroadenoma.
                                                       2
tendency to grow aggressively and can recur locally. Similar     5. Phyllodes tumors generally manifest as larger masses
to other sarcomas, the malignant tumors metastasize                   and display rapid growth.
hematogenously. Unfortunately, the pathologic appearance         6. Mammographic findings (ie, the appearance of round
of a phyllodes tumor does not always predict the neoplasm's           densities with smooth borders) are also similar to those
clinical behavior; in some cases, therefore, there is a degree        of fibroadenoma.
of uncertainty about the lesion's classification. The            7. Recurrent malignant tumors seem to be more aggressive
characteristics of a malignant phyllodes tumor include the            than the original tumor.
following:                                                       8. The lungs are the most common metastatic site,
1. Recurrent malignant tumors seem to be more aggressive              followed by the skeleton, heart, and liver.
     than the original tumor.                                    9. Symptoms from metastatic involvement usually arise in a
2. The lungs are the most common metastatic site,                     few months but may occur as late as 12 years after the
     followed by the skeleton, heart, and liver.                      initial therapy.
3. Symptoms from metastatic involvement can arise from           10. Most patients with metastases die within 3 years of the
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     as early as a few months to as late as 12 years after the        initial treatment.
     initial therapy.                                            11. No cures exist for systemic metastases.
4. Most patients with metastases die within 3 years of the       12. Roughly 30% of patients with malignant phyllodes
                        4
     initial treatment.                                               tumors die from the disease.
5. No cures for systemic metastases exist.                       Causes
6. Roughly 30% of patients with malignant phyllodes              The etiology of cystosarcoma phyllodes is unknown.
     tumors die from the disease.                                Differential Diagnoses
                                                                 Angiosarcoma
                                                                 Breast Cancer
Other Problems to Be Considered                                  1.  If the tumor to breast ratio is sufficiently high to
Juvenile fibroadenoma                                                preclude a satisfactory cosmetic result by segmental
Giant fibroadenoma                                                   excision, total mastectomy, with or without
Inflammatory carcinoma                                               reconstruction, is an alternative.
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Sclerosing adenosis                                              2. More radical procedures are not generally warranted.
Radial scar                                                      3. Perform axillary lymph node dissection only for clinically
Fat necrosis                                                         suspicious nodes. However, virtually all of these nodes
                                                                                                                      10
Fibrocystic change                                                   are reactive and do not contain malignant cells.
Breast abscess                                                   Consultations
Adenocarcinoma                                                   Consult a general surgery specialist.
Mastitis                                                         Medication
Workup                                                           There is no proven role for adjuvant chemotherapy or
Laboratory Studies                                               radiation therapy in the treatment of phyllodes tumors.
No specific hematologic tumor markers or other blood tests       Response to chemotherapy and radiotherapy for recurrences
can be used to diagnose cystosarcoma phyllodes.                  and metastases has been poor, and no success with
Imaging Studies                                                  hormonal manipulation has been documented.
Although mammography and ultrasonography generally are           Follow-up
important in the diagnosis of breast lesions, they are           Further Outpatient Care
notoriously unreliable in differentiating benign cystosarcoma    1. Although specific guidelines regarding follow-up care
phyllodes (CSP) from the malignant form of the condition or          for phyllodes tumors are limited because of the rarity of
from fibroadenomas. Thus, findings on imaging studies are            these lesions, regular, long-term follow-up care should
                                    6
not definitively diagnostic of CSP.                                  be performed to detect possible local recurrences.
Procedures                                                       2. An initial visit 1-2 weeks after surgery to detect any
1. Fine-needle aspiration for cytologic examination usually          initial complications should be followed by periodic visits
     is inadequate for the diagnosis of phyllodes tumors.Core        as determined by the patient's surgeon. A reasonable
     biopsy is more reliable, but there still can be sampling        schedule might be physical examinations every 6 months
     errors and difficulty in distinguishing the lesion from a       and mammograms yearly for at least 5 years
     fibroadenoma.                                                   (seeComplications).
2. Open excisional breast biopsy for smaller lesions or          3. Carefully observe patients for any possible recurrence.
     incisional biopsy for large lesions is the definitive       Complications
     method for diagnosing phyllodes tumors.                     As     with      most      breast     surgery,   postoperative
Histologic Findings                                              complications from the surgical treatment of phyllodes
All phyllodes tumors contain a stromal component that can        tumors include the following:
vary significantly in histologic appearance from one lesion to                 o Infection
another. In general, benign phyllodes tumors demonstrate a                     o Seroma formation
markedly increased number of regular fusiform fibroblasts in                   o Local and/or distant recurrence
the stroma. Occasionally, highly anaplastic cells with myxoid    Prognosis
changes are observed. A high degree of cellular atypia, with     1. Although cystosarcoma phyllodes is considered to be a
increased stromal cellularity and an increased mitotic               clinically benign tumor, the possibility for local
count, is almost always observed in the malignant form of            recurrence following excision always exists, particularly
cystosarcoma phyllodes. Ultrastructurally, in the benign and                                                          9,11
                                                                     with lesions that display malignant histology. Tumors
malignant forms of phyllodes tumors, nucleoli may reveal a           that, after initial treatment by wide local excision, recur
coarsely meshed nucleolonema and abundant cisternae in               locally should ideally be treated with total mastectomy.
the endoplasmic reticulum.                                       2. Metastatic disease is typically observed in the lung,
Treatment                                                            mediastinum, and skeleton.
Surgical Care                                                    3. The clinical course is variable.
In most cases of cystosarcoma phyllodes, perform wide local                o If the tumor is benign, the long-term prognosis
                                      7,8,9
excision, with a rim of normal tissue. No absolute rules on                    is excellent following adequate local excision.
margin size exist. However, a 2 cm margin for small (<5 cm)                o If the tumor recurs locally after excision, further
tumors and a 5 cm margin for large (>5 cm) tumors have                         local excision or total mastectomy is typically
been advocated.                                                                curative.
The lesion should not be "shelled out," as might be done with
a fibroadenoma, or the recurrence rate will be unacceptably
      4
high.

								
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