eMedicine Specialties > General Surgery > Thorax

Cystosarcoma Phyllodes

Author: Donald R Lannin, MD, Professor, Clinician-Educator Track, Department of Surgery, Section of Oncology; Executive Director, Co-Medical Director, Yale Comprehensive Breast Center
Coauthor(s): Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic, Billings, Montana; John H Raaf, MD, PhD, Professor, Department of Surgery, Case Western Reserve University
Contributor Information and Disclosures

Updated: Jan 30, 2009

Introduction

Background

Cystosarcoma phyllodes is a rare, predominantly benign tumor that occurs almost exclusively in the female breast.¹ 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, 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 5 cm. However, lesions of more than 30 cm have been reported.

Frequency

United States

No difference in phyllodes tumor frequency appears to exist between patients from the United States and those from other countries. Phyllodes tumors account for approximately 1% of all breast neoplasms.²

Mortality/Morbidity

Because of limited data, the percentage of benign vs malignant phyllodes tumors is not well defined. Reports suggest, however, that about 85-90% of phyllodes tumors are benign and that approximately 10-15% are malignant.³

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:

• 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 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.⁴
• 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 for phyllodes tumors.

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; however, they behave in a benign fashion similar to that of other fibroadenomas.

Clinical

History

• Patients typically present with a firm, mobile, well-circumscribed, nontender breast mass.
• 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

• 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.⁵
• Phyllodes tumors generally manifest as larger masses and display rapid growth.
• Mammographic findings (ie, the appearance of round densities with smooth borders) are also similar to those of fibroadenoma.
• 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 usually arise in a few months but may occur as late as 12 years after the initial therapy.
• Most patients with metastases die within 3 years of the initial treatment.⁴
• No cures exist for systemic metastases.
• Roughly 30% of patients with malignant phyllodes tumors die from the disease.

Causes

The etiology of cystosarcoma phyllodes is unknown.

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