AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

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.

Frequency

United States

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

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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.
• Rarely do tumors involve the nipple-areola complex or ulcerate to the skin.
• Patients with metastases may present with symptoms such as dyspnea, fatigue, and bone pain.

Physical

• A firm, mobile, well-circumscribed, nontender breast mass is appreciated.
• Curiously, CSP tends to involve the left breast more commonly than the right breast.
• Overlying skin may display a shiny appearance and be translucent enough that underlying breast veins are visible.
• Physical findings are similar to fibroadenoma, ie, mobile masses with distinct borders.
• Phyllodes tumors generally manifest as larger masses and with rapid growth.
• Mammographic findings are also similar to fibroadenoma, ie, round densities with smooth borders.
• 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 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.

Causes

The etiology of CSP is unknown.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Juvenile fibroadenoma
Giant fibroadenoma
Inflammatory carcinoma
Sclerosing adenosis
Radial scar
Fat necrosis
Fibrocystic change
Breast abscess
Mastitis

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• No specific hematologic tumor markers or other blood tests can be used to diagnose CSP.

Imaging Studies

• While mammography and ultrasonography are important in the diagnosis of breast lesions in general, they are notoriously unreliable in differentiating benign CSP from fibroadenomas and malignant CSP. Thus, findings on imaging studies are not definitively diagnostic of this disease.

Procedures

• Fine-needle aspiration (FNA) for cytologic examination usually is inadequate for diagnosis. Core biopsy is more reliable, but there still can be sampling errors and difficulty distinguishing the lesion from a fibroadenoma.
• Open excisional breast biopsy for smaller lesions or incisional biopsy for large lesions is the definitive method to diagnose this lesion.

Histologic Findings

All CSP tumors contain a stromal component that can vary significantly in histologic appearance from one tumor to another. In general, benign CSP lesions demonstrate markedly increased numbers of regular fusiform fibroblasts in the stroma. Occasionally, highly anaplastic cells with myxoid changes are observed. A high degree of cellular atypia with increased stromal cellularity and increased mitotic count are nearly always observed in the malignant form of CSP. Ultrastructurally, nucleoli may reveal a coarsely meshed nucleolonema and abundant cisternae in the endoplasmic reticulum in both benign and malignant forms.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Surgical Care

In most cases, perform wide local excision with a rim of normal tissue. The lesion should not be "shelled out" as might be done with a fibroadenoma, or the recurrence rate will be unacceptably high.

• If the tumor-to-breast ratio is sufficiently high to preclude a satisfactory cosmetic result by segmental excision, total mastectomy, with or without reconstruction, is an alternative.
• More radical procedures are not generally warranted.
• Perform axillary lymph node dissection only for clinically suspicious nodes. However, virtually all of these nodes are reactive and do not contain malignant cells.

Consultations

Consult a general surgery specialist.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

There is no proven role for adjuvant chemotherapy or radiation therapy. Response to chemotherapy and radiotherapy for recurrences and metastases has been poor, and no success with hormonal manipulation has been documented.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• Although specific guidelines regarding follow-up care for CSP are limited because of the rarity of these lesions, regular, long-term follow-up care should be performed to detect possible local recurrences.
• An initial visit 1-2 weeks after surgery to detect any initial complications should be followed by periodic visits as determined by the patient's surgeon. A reasonable schedule might be physical examinations every 6 months and mammograms yearly for at least 5 years (see Complications below).
• Carefully observe patients for any possible recurrence.

Complications

• As with most breast surgery, postoperative complications include the following:
• • Infection
  • Seroma formation
  • Local and/or distant recurrence

Prognosis

• Although CSP is considered a clinically benign tumor, the possibility for local recurrence following excision always exists, particularly with lesions that display malignant histology. Tumors initially treated by wide local excision that recur locally should ideally be treated by total mastectomy.
• Metastatic disease is typically observed in the lung, mediastinum, and skeleton.
• The clinical course is variable.
• • If the tumor is benign, the long-term prognosis is excellent following adequate local excision.
  • If the tumor recurs locally after excision, further local excision or total mastectomy is typically curative.

Patient Education

• As with all breast neoplasms, self-examination remains the most important initial mechanism for detection of lesions. Appropriate teaching of this procedure is vital for early detection of all breast neoplasms.
• For excellent patient education resources, visit eMedicine's Women's Health Center and Cancer and Tumors Center. Also, see eMedicine's patient education articles Breast Cancer and Breast Self-Exam.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• While most CSP lesions are benign, the possibility exists for underestimating their potential for malignancy. The difficulty in distinguishing between fibroadenoma, benign CSP, and malignant CSP may be vexing for even the most experienced pathologist. Nevertheless, complete excision with accurate histologic examination and continued follow-up care is the best way to treat this rare disease.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Brooks HL, Priolo S, Waxman. Cystosarcoma phylloides: a case report of an 11-year survival and review of surgical experience. Contemp Surg. 1998;53:169-172.
• Chen WH, Cheng SP, Tzen CY. Surgical treatment of phyllodes tumors of the breast: retrospective review of 172 cases. J Surg Oncol. Sep 1 2005;91(3):185-94.
• Cole-Beuglet C, Soriano R, Kurtz AB. Ultrasound, x-ray mammography, and histopathology of cystosarcoma phylloides. Radiology. Feb 1983;146(2):481-6. [Medline].
• Contarini O, Urdaneta LF, Hagan W. Cystosarcoma phylloides of the breast: a new therapeutic proposal. Am Surg. Apr 1982;48(4):157-66. [Medline].
• Hoover HC. Cystosarcomas of the breast. In: Raaf JH, ed. Soft Tissue Sarcomas: Diagnosis and Treatment. St. Louis, Mo:. Mosby;1993:113-121.
• Parker SJ, Harries SA. Phyllodes tumours. Postgrad Med J. Jul 2001;77(909):428-35.

Article Last Updated: Jun 12, 2006