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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 1 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 4 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. 8 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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