Phyllodes Tumor (Cystosarcoma Phyllodes)

Updated: Jun 20, 2016
  • Author: Donald R Lannin, MD; Chief Editor: John Geibel, MD, DSc, MSc, AGAF  more...
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Overview

Background

Cystosarcoma phyllodes (from Greek kystis ["sac, bladder"], sarkoma ["fleshy tumor"], and phyllon ["leaf"]) is a rare, predominantly benign tumor that occurs almost exclusively in the female breast. [1, 2] 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 of these tumors are benign, the term cystosarcoma is potentially misleading. Accordingly, the term currently favored is phyllodes tumor.

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Pathophysiology and Etiology

Phyllodes tumor is the most commonly occurring nonepithelial neoplasm of the breast, though it represents only about 1% of tumors in the breast. [3]  It has a smooth, sharply demarcated texture and typically is freely movable. It is a relatively large tumor, with an average size of 5 cm (though lesions larger than 30 cm have been reported). The etiology of phyllodes tumors is unknown.

Because of limited data, the relative percentages of benign and malignant phyllodes tumors are not well defined. Reports have suggested, however, that about 85-90% of phyllodes tumors are benign and that approximately 10-15% are malignant. [4]

Although benign phyllodes tumors do not metastasize, they have a tendency to grow aggressively and can recur locally. [3]  Like other sarcomas, malignant phyllodes 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 of the disease

Although most phyllodes tumors are benign, it is nonetheless important not to underestimate the potential of these lesions for malignancy. Moreover, some juvenile fibroadenomas in teenagers can look like phyllodes tumors on histologic examination; however, they behave in a benign fashion similar to that of other fibroadenomas. The difficulty of distinguishing among fibroadenomas, benign phyllodes tumors, and malignant phyllodes tumors may be vexing for even the most experienced pathologist. [7]

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Epidemiology

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

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Prognosis

If a phyllodes tumor is benign, the long-term prognosis is excellent after adequate local excision. However, the possibility for local recurrence after excision always exists, particularly with lesions that display malignant histology. [8, 9, 10]  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]

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Patient Education

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

For patient education information, see the Women's Health Center and the Cancer Center, as well as Breast Cancer and Breast Self-Exam.

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