Elsevier

Surgical Oncology

Volume 12, Issue 1, July 2003, Pages 27-37
Surgical Oncology

Malignant phyllodes tumor of the breast: review of the literature and case report of stromal overgrowth

https://doi.org/10.1016/S0960-7404(03)00005-7Get rights and content

Abstract

Cystosarcoma phyllodes constitutes only 0.3–0.9% of all breast tumors. The term “sarcoma” was initially used because of its fleshy appearance, a more modern term is Phyllodes tumor (PT). The behavior of PT constitutes a spectrum from benign and locally recurrent to malignant and metastatic. In a general surgical series, 6.2% of the tumors were malignant. The microscopic appearance of PT is that of epithelial elements and connective tissue stroma. Malignancy is determined by characteristics of the stroma. The metastatic spread of malignant PT is mainly hematogenous to lung, with infrequent lymphatic involvement. Wide local excision with 2 cm margins is the treatment of choice. In 20% of both benign and malignant cases, PT will locally recur. There is no proven benefit of radiation or chemotherapy, although radiotherapy may be useful in selected cases. We present a case of a sarcomatous overgrowth in a malignant phyllodes tumor involving multiple histologic types.

Introduction

Breast cancer affects 1 in 9 women and is the 2nd leading cause of cancer related deaths in females. The great majority of these cases correspond to epithelial malignancies, the most frequent being ductal carcinomas. The most frequently encountered non-epithelial malignancies of the breast are lymphomas, sarcomas, and malignant melanomas. One of the most infrequent neoplasms is cystosarcoma phyllodes (CSP) which constitutes only 0.3–0.9% of all breast tumors and 2–3% of fibroepithelial neoplasms [1]. Johannes Muller first described CSP in 1838, choosing the term “sarcoma” for its gross fleshy appearance [2]. However, it was not until 1931 that the first case of metastatic phyllodes tumor (PT) was identified [3]. Although, the term cystosarcoma denotes a malignant nature, the majority of cases are benign. Taking this fact into consideration and wanting to establish a neutral term, the World Health Organization coined the term “phyllodes tumor” in 1981 [4].

In earlier studies, CSP had been described as either benign, borderline or malignant by histology [5]. As described by Kario et al. the most important clinical problem is that histological parameters do not correlate with the clinical course [6]. Benign and malignant phyllodes tumors recur locally, and high grade tumors have a metastatic incidence rate of 25% [1]. A study investigating the histological features and clinical outcomes of 32 patients determined that tumor recurrence is determined by the presence of tumor at the margins of excision, rather than the histology [7]. They found that all of the benign tumors that recurred were due to incomplete excision during their initial treatment and that none of the completely excised tumors recurred [7]. Unlike other breast tumors, there are no predisposing factors and the etiology of phyllodes tumor is unknown. Studies have demonstrated that large tumors are more likely to be malignant, have stromal overgrowth and develop local recurrence [6]. In other studies, it was determined that stromal overgrowth is the most important predictor of aggressive behavior [8]. In this case report we discuss a patient who developed a rapidly expanding malignant phyllodes tumor with a sarcomatous overgrowth by multiple histologic types.

Section snippets

Case report

A 96 year-old female presented to the surgical clinic with of an expanding left breast mass. The patient had been initially seen 18 months before for evaluation of a concerning mass on the left breast. On initial examination a firm well-circumscribed mass was palpable in the left breast. An ultrasound study revealed a 1.5×2.8 cm lobulated solid mass and mammography indicated a 2.1×3.2 cm high-density subareolar mass with partially defined margins. A core biopsy of the breast mass was subsequently

Histopathology

Phyllodes tumors are well-circumscribed, painless, mobile masses that are surrounded by a pseudocapsule of compressed breast parenchyme [1]. These tumors range in size from 1 to 41 cm, with a median size of 5 cm [9]. One study found that 73% of benign phyllodes tumors were under 5 cm, while all malignant PT were over 7 cm [6]. Grossly, smaller tumors resemble fibroadenomas with a gray-white fibrous appearance, while larger ones appear fleshy, resembling sarcomas [10]. Both benign and malignant

Epidemiology

Phyllodes tumor comprises less than 1% of all breast tumors. The incidence rate of phyllodes tumor is 1 in 100,000 [24] with an overall risk of malignancy of 2.1 per 1 million women [25]. The average age of onset is 41–44 years (range: 9–85 years) [1], [10], [24], [26], [27] and the risk of this tumor peaks between 45 and 49 years of age [25]. One study has shown that Latina women are at higher risk of phyllodes tumor than other ethnic groups. They also indicated that women born in Mexico and

Clinical manifestations

Patients typically present with a firm, seldom-painful breast mass that is smooth, mobile and well circumscribed [9], [29]. The tumor exhibits continuous growth, but it may also rapidly increase in size. This may result in shiny stretched skin that becomes translucent to display underlying veins [29]. As a result of stretching and increased pressure; the attenuated skin ulcerates secondary to ischemia [11]. Even so, it has been observed that pain, retraction of the nipple and skin fixation

Diagnosis

In a series of 18 patients, investigators determined that preoperative diagnosis of phyllodes tumor occurred in only 10–20% of the cases [1]. A major diagnostic problem involving phyllodes tumor is that fine needle aspiration, mammography and ultrasound studies are not able to distinguish between fibroadenoma and phyllodes tumor. For example, mammography unveils large round masses with smooth borders that resemble either a fibroadenoma or a well-circumscribed carcinoma [1]. A confirmed

Management

Due to the properties of CSP, surgical excision is the mainstay treatment. Wide local excision with at least 1–2 cm margins is routine, [21], [24], [27], [30] except for lesions larger than 10 cm in which total mastectomy is recommended [6], [24]. Though, removal of the pectoralis fascia or muscle is not routinely indicated unless they are involved [9]. If initial excison of the tumor fails to produce >1 cm margin, it is recommended that the patient should undergo reexcision to obtain a wider

Prognosis

Benign and malignant phyllodes tumors recur locally and both have the potential to metastasize [1]. Pathologists are able to distinguish benign phyllodes tumors, which very rarely metastasize, from malignant phyllodes tumors, which metastasize in one fourth of the patients [40]. However, determining the probability of recurrence by histology still remains undetermined. Data demonstrates that tumors larger than 10 cm (incidence 7 times greater than smaller tumors) and involved excisional margins

Sarcomatous overgrowth

Sarcomatous differentiation is an extremely rare event in PT. Review of the literature back to 1979 yields a total of 30 documented cases. The most common histologic type is liposarcoma, accounting for a total of 23 cases [42], [43], [44], [45], [46], [47], [48], [49], [50] (adipose differentiation ranged from mature fat to liposarcoma). MFH has been identified in 3 cases [51], [52], while rhabdomyosarcoma [53] and chondrosarcoma [54] in one case of malignant PT each. An even more unusual event

Conclusion

The rare finding of a CSP tumor with sarcomatous overgrowth adds to the quandary of what treatment modality improves survival and recurrence. Moreover, another obstacle is encountered in trying to identify a breast mass as a phyllodes tumor. As mentioned, the presentation and histological findings of phyllodes tumor is similar to those of fibroadenoma. Since a fibroadenoma is the most common tumor in young women, it would be impractical to aggressively treat these masses because of the slight

Marlon Alex Guerrero received his undergraduate education at California State University at Hayward, where he received his Bachelor of Science in Biology. He is currently a fourth year medical student at Meharry Medical College in Nashville, TN and will graduate as in May 2003. He has been the reciepient of multiple honors and awards throughout his education including the Alpha Omega Alpha Honor Medical Society, Dean's List during all semesters, Henry Nehemiah Cooper, M.D. Memorial

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    Marlon Alex Guerrero received his undergraduate education at California State University at Hayward, where he received his Bachelor of Science in Biology. He is currently a fourth year medical student at Meharry Medical College in Nashville, TN and will graduate as in May 2003. He has been the reciepient of multiple honors and awards throughout his education including the Alpha Omega Alpha Honor Medical Society, Dean's List during all semesters, Henry Nehemiah Cooper, M.D. Memorial Scholorship, Jonathon Wayne House Memorial Scholorship, Hears Endowed Scholorship, NIH Funded Minority Biomedical Research Support Scholar, Golden Key Honor Society, Latino Academic Society, Retired Officers Society Outstanding Leadership Award, Airforce ROTC Academic Excellence Award, and Arnold Air Academic Society. He has been actively involved in research as a Research Assistant at Vanderbilt University. He has presented his research at the Annual NIH-MBRS National Research Symposium as well as at the Vanderbilt University Diabetes Center Student Research Symposium, and at the Southern Medical Association Annual Scientific Assembly. He has also been actively involved in the community as the National Coordinator for AMSA Minority Affairs and has co-founded and is currently the president of the American Latino Medical Association.

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    Billy R. Ballard, DDS, MD, is Professor and Chair of the Department of Pathology and Associate Dean for Graduate Medical Education at Meharry Medical College, Nashville, Tennessee. He has a Bachelor of Science degree from Southern University, Baton Rouge, Louisiana, a DDS, and MD, from Meharry Medical College, Nashville, Tennessee. His postdoctoral studies include residency trainning in pathology at George W. Hubbard Hospital, Nashville, Tennessee, State University of New York. He completed a Fellowship in Surgical and Cytopathology at Roswell Park Memorial Cancer Institute and the University of Mississippi Medical Center, Jackson, Mississippi. He has received fellowships from the National Cancer Institute, the National Institutes of Health and the American Cancer Society, New York Division. Doctor Ballard is a Diplomat of the American Board of Oral Pathology, and a Diplomat of the American Board of Pathology. His honors include Alpha Omega Alpha Honor Medical Society, Omicron Kappa Upsilon Honor Dental Society, Fellow of the American Society of Clinical Pathologists, and a Fellow of the College of American Pathologists, and the American Academy of Oral Pathology.

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    Dr. Ana M. Grau received her Medical School education at the Universidad Catolica de Chile, where she graduated with Maximum Distinction. She trained as a general surgeon first in Chile and then at the University of Arizona, Tucson Hospitals. During her residency she participated in a one-year Surgical Oncology Research Fellowship at the University of Texas M.D. Anderson Cancer Center, where she later returned for a two-year Surgical Oncology Fellowship. She is currently Assistant Professor of Surgery at Meharry Medical College and Vanderbilt University. She also serves as Chief, Surgical Oncology Section and Director, Breast Health Center at Meharry Medical College. Dr. Grau is a Diplomat of the American Board of Surgery, and a member of the Society of Clinical Oncology, American Association for Cancer Research, Society of Surgical Oncology, and Association for Academic Surgery. She serves in the International Relations Committee, American College of Surgeons ACOSOG and as Cancer Physician Liaison, American College of Surgeons, Commission on Cancer. Her research interests are in the role of transcriptional factors in cancer, with emphasis in Transforming Growth factor β and Nuclear Factor κ B, and also in health disparities in breast cancer outcomes.

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