Disease Information for Breast cystosarcoma phyllodes

Cystosarcoma phyllodes (CSP) is a rare, predominantly benign tumor that occurs almost exclusively in the female breast; Its name is derived from the Greek words sarcoma, 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 (hence the name); Because most tumors are benign, the name may be misleading; Thus, the favored terminology is now phyllodes tumor; Pathophysiology: Phyllodes tumor is the most commonly occurring nonepithelial neoplasm of the breast but represents only about 1% of tumors in the breast; It has a sharply demarcated smooth texture and is typically freely movable; It is a relatively large tumor, and the average size is 5 cm; However, lesions more than 30 cm in size have been reported; In the US: No difference in phyllodes tumor frequency appears to exist between patients from the United States and patients from other countries; Phyllodes tumors comprise less than 1% of all breast neoplasms;

Mortality/Morbidity: About 90% of phyllodes tumors are benign and about 10% are malignant; The benign tumors, although they will not metastasize, do have a tendency to grow aggressively and recur locally; The malignant tumors metastasize hematogenously similar to other sarcomas; Unfortunately, the pathologic appearance does not always predict the clinical behavior, so there is always some uncertainty about the classification of some cases; 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 from metastatic involvement may arise from a few months to as late as 12 years after initial therapy; Most patients with metastases die within 3 years of initial treatment; No cures for systemic metastases exist; Roughly 30% of patients with malignant phyllodes tumors die from the disease; Race: A racial predilection does not appear to exist; Sex: Phyllodes tumors occur almost exclusively in females; Rare case reports have been described in males; Age: Phyllodes tumors can occur in people of any age; however, the median age is the fifth decade of life; Some juvenile fibroadenomas in teenagers can look histologically like phyllodes tumors, but they behave in a benign fashion similar to other fibroadenomas; --------------[EMedicine website 2007]-------------Median age 54 year old female

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