eMedicine Specialties > General Surgery > Thorax

Cystosarcoma Phyllodes

Donald R Lannin, MD, Professor, Clinician-Educator Track, Department of Surgery, Section of Oncology; Executive Director, Co-Medical Director, Yale Comprehensive Breast Center
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

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.

Differential Diagnoses

Angiosarcoma
Breast Cancer

Workup

Laboratory Studies

No specific hematologic tumor markers or other blood tests can be used to diagnose cystosarcoma phyllodes.

Imaging Studies

Although mammography and ultrasonography generally are important in the diagnosis of breast lesions, they are notoriously unreliable in differentiating benign cystosarcoma phyllodes (CSP) from the malignant form of the condition or from fibroadenomas. Thus, findings on imaging studies are not definitively diagnostic of CSP.⁶

Procedures

Fine-needle aspiration for cytologic examination usually is inadequate for the diagnosis of phyllodes tumors. Core biopsy is more reliable, but there still can be sampling errors and difficulty in distinguishing the lesion from a fibroadenoma.
Open excisional breast biopsy for smaller lesions or incisional biopsy for large lesions is the definitive method for diagnosing phyllodes tumors.

Histologic Findings

All phyllodes tumors contain a stromal component that can vary significantly in histologic appearance from one lesion to another. In general, benign phyllodes tumors demonstrate a markedly increased number 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 an increased mitotic count, is almost always observed in the malignant form of cystosarcoma phyllodes. Ultrastructurally, in the benign and malignant forms of phyllodes tumors, nucleoli may reveal a coarsely meshed nucleolonema and abundant cisternae in the endoplasmic reticulum.

Treatment

Surgical Care

In most cases of cystosarcoma phyllodes, perform wide local excision, with a rim of normal tissue.^7,8,9 No absolute rules on margin size exist. However, a 2 cm margin for small (<5 cm) tumors and a 5 cm margin for large (>5 cm) tumors have been advocated.

The lesion should not be "shelled out," as might be done with a fibroadenoma, or the recurrence rate will be unacceptably high.⁴

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.⁸
Perform axillary lymph node dissection only for clinically suspicious nodes. However, virtually all of these nodes are reactive and do not contain malignant cells.¹⁰

Consultations

Consult a general surgery specialist.

Medication

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

Follow-up

Further Outpatient Care

Although specific guidelines regarding follow-up care for phyllodes tumors 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).
Carefully observe patients for any possible recurrence.

Complications

As with most breast surgery, postoperative complications from the surgical treatment of phyllodes tumors include the following:
- Infection
- Seroma formation
- Local and/or distant recurrence

Prognosis

Although cystosarcoma phyllodes is considered to be a clinically benign tumor, the possibility for local recurrence following excision always exists, particularly with lesions that display malignant histology.^9,11 Tumors that, after initial treatment by wide local excision, recur locally should ideally be treated with 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 the detection of phyllodes tumors. 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

Medicolegal Pitfalls

While most phyllodes tumors are benign, the possibility exists for underestimating their potential for malignancy. The difficulty in distinguishing between fibroadenoma, benign phyllodes tumors, and malignant cystosarcoma phyllodes may be vexing for even the most experienced pathologist.⁵ Nevertheless, complete excision, with accurate histologic examination and continued follow-up care, is the best way to treat this rare disease.

References

Hoover HC. Cystosarcomas of the breast. In: Raaf JH, ed. Soft Tissue Sarcomas: Diagnosis and Treatment. St Louis, Mo: Mosby; 1993:113-21.
Parker SJ, Harries SA. Phyllodes tumours. Postgrad Med J. Jul 2001;77(909):428-35. [Medline]. [Full Text].
Jones AM, Mitter R, Poulsom R, et al. mRNA expression profiling of phyllodes tumours of the breast: identification of genes important in the development of borderline and malignant phyllodes tumours. J Pathol. Dec 2008;216(4):408-17. [Medline].
Brooks HL, Priolo S, Waxman. Cystosarcoma phylloides: a case report of an 11-year survival and review of surgical experience. Contemp Surg. 1998;53:169-72.
Yohe S, Yeh IT. "Missed" diagnoses of phyllodes tumor on breast biopsy: pathologic clues to its recognition. Int J Surg Pathol. Apr 2008;16(2):137-42. [Medline].
Cole-Beuglet C, Soriano R, Kurtz AB. Ultrasound, x-ray mammography, and histopathology of cystosarcoma phylloides. Radiology. Feb 1983;146(2):481-6. [Medline]. [Full Text].
Chen WH, Cheng SP, Tzen CY, et al. Surgical treatment of phyllodes tumors of the breast: retrospective review of 172 cases. J Surg Oncol. Sep 1 2005;91(3):185-94. [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].
Pezner RD, Schultheiss TE, Paz IB. Malignant phyllodes tumor of the breast: local control rates with surgery alone. Int J Radiat Oncol Biol Phys. Jul 1 2008;71(3):710-3. [Medline].
Gullett NP, Rizzo M, Johnstone PA. National surgical patterns of care for primary surgery and axillary staging of phyllodes tumors. Breast J. Jan-Feb 2009;15(1):41-4. [Medline].
Jones AM, Mitter R, Springall R, et al. A comprehensive genetic profile of phyllodes tumours of the breast detects important mutations, intra-tumoral genetic heterogeneity and new genetic changes on recurrence. J Pathol. Apr 2008;214(5):533-44. [Medline].

Keywords

cystosarcoma phyllodes, breast tumor, breast lump, breast tumors, breast lumps, phyllodes tumor, phyllodes, cystosarcoma, phyllodes breast, cystosarcoma phylloides, fibroadenoma, giant fibroadenoma, benign breast tumor, breast mass, nonepithelial neoplasm of the breast, breast examination, breast self-examination, malignant phyllodes tumor, malignant CSP, nontender breast mass, open excisional breast biopsy

Contributor Information and Disclosures

Author

Donald R Lannin, MD, Professor, Clinician-Educator Track, Department of Surgery, Section of Oncology; Executive Director, Co-Medical Director, Yale Comprehensive Breast Center
Donald R Lannin, MD is a member of the following medical societies: Alpha Omega Alpha, American Cancer Society, and American College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Anastasios K Konstantakos, MD, Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic, Billings, Montana
Disclosure: Nothing to disclose.

John H Raaf, MD, PhD, Professor, Department of Surgery, Case Western Reserve University
John H Raaf, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association of Endocrine Surgeons, American College of Surgeons, American Society of Clinical Oncology, Central Surgical Association, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

Medical Editor

Brian James Daley, MD, MBA, FACS, Associate Program Director, Professor, Department of Surgery, Division of Trauma and Critical Care, University of Tennessee School of Medicine
Brian James Daley, MD, MBA, FACS is a member of the following medical societies: American Association for the Surgery of Trauma, American College of Chest Physicians, American College of Surgeons, American Medical Association, Association for Academic Surgery, Association for Surgical Education, Eastern Association for the Surgery of Trauma, Shock Society, Society of Critical Care Medicine, Southeastern Surgical Congress, and Tennessee Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Michael A Grosso, MD, Consulting Staff, Department of Cardiothoracic Surgery, St Francis Hospital
Michael A Grosso, MD is a member of the following medical societies: American College of Surgeons, Society of Thoracic Surgeons, and Society of University Surgeons
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chairman, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital
John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract
Disclosure: AMGEN Royalty Other

Further Reading

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