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Phyllodes tumors of the breast

Authors
Ana M Grau, MD, FACS
A Bapsi Chakravarthy, MD, FASTRO
Rashmi Chugh, MD
Section Editors
Lori J Pierce, MD
Daniel F Hayes, MD
Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
Deputy Editor
Wenliang Chen, MD, PhD

INTRODUCTION

Phyllodes tumors are uncommon fibroepithelial breast tumors that are capable of a diverse range of biologic behavior. In their least aggressive form, they behave similarly to benign fibroadenomas, although with a propensity to recur locally following excision without wide margins. At the other end of the spectrum are tumors that metastasize distantly, sometimes degenerating histologically into sarcomatous lesions that lack an epithelial component [1,2].

The terminology of phyllodes tumors has evolved. All such tumors were originally called "cystosarcoma phyllodes" by Johannes Muller in 1838 [1]. However, they only occasionally have cystic components, and they are not true sarcomas by either cellular origin or biologic behavior. The term "phyllodes", which means leaf-like, describes the typical papillary projections that are seen on pathologic examination. Since the original description, over 60 synonyms have been applied to this entity until the adoption of the term phyllodes tumors by the World Health Organization [3,4].

EPIDEMIOLOGY AND RISK FACTORS

Phyllodes tumors account for fewer than 0.5 percent of all breast malignancies [4,5]. Given their rarity, epidemiologic data are scant. In a study from Los Angeles county over a 17 year period, the average annual incidence rate was 2.1 per million women, and there was a higher incidence in Latina whites, as compared to non-Latina whites, Asians, and African American women [6].

The vast majority occur in women, in whom the median age at presentation is 42 to 45 (range 10 to 82 years) [3,6-8]. Data indicate that the tumor grade increases with mean age at diagnosis [9]. Some case reports describe these tumors in men, usually in association with gynecomastia [3].

No etiologic or predisposing factors have been associated with phyllodes tumors, with the exception of Li-Fraumeni syndrome, a rare autosomal dominant condition that is characterized by the development of multiple tumors [10]. (See "Overview of hereditary breast and ovarian cancer syndromes" and "Li-Fraumeni syndrome".)

                

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Literature review current through: Nov 2016. | This topic last updated: Thu Feb 20 00:00:00 GMT+00:00 2014.
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