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Annals of Clinical & Laboratory Science 36:469-474 (2006)
© 2006 Association of Clinical Scientists

Case Report

Morphological Characterization of the Breast in Proteus Syndrome Complicated by Ductal Carcinoma In Situ

Jabed Iqbal1, Gary He1, Leslie G. Biesecker2, Peter Rosen3, Paul H. Duray4, Douglas Schwartzentruber5, Mansor Beg1 and Ellen Kahn1
1 North Shore University Hospital, NYU School of Medicine, Manhasset, NY; 2 National Human Genome Research Institute, NIH, Bethesda, MD; 3 New York Presbyterian Hospital, New York University, New York, NY; 4 Clinical Center, Department of Pathology, NIH, Bethesda, MD; and 5 Goshen Health System Cancer Center, Goshen, IN

Address correspondence to Ellen Kahn, M.D., 9 Sheep Pasture Road, Huntington, NY 11743, USA; tel 613 692 8548, e-mail ellenkahn{at}optonline.net.

Proteus syndrome (PS) is a severe, variable, and rare disorder with asymmetric and disproportionate overgrowth of body parts, cerebriform connective tissue nevi, epidermal nevi, dysregulated adipose tissue, and vascular malformations. It is associated with benign and occasionally malignant tumors. We report the first case of ductal carcinoma in situ (DCIS) in a 28-yr-old woman with PS who underwent a mastectomy for asymmetric overgrowth. The cut surface of the tissue showed a discrete, white, lobulated, solid mass with multiple cysts with occasional small polypoid nodules. Microscopically, the tissue was characterized by neoplastic and non-neoplastic changes. The former consisted of multiple intraductal papillomas and low-grade intraductal papillary, solid, and cribriform carcinoma. The non-neoplastic changes were characterized by cysts of various sizes, lined by cuboidal or apocrine cells, focally with epithelial papillary proliferation; the lumens contained eosinophilic, mucicarmine-positive, and PAS-positive material. Variable ductal proliferation and periductal, peri- and intra-lobular fibrosis with loose fibrous connective tissue was present. The carcinoma was positive for ER, PR, CK7, and MIB-1 (40%), and negative for p53 and CK20 staining. We conclude that DCIS may be one of the tumors associated with PS and that the proliferative phenotype serves as an initiator for carcinogenesis. This case highlights the difficulty of recognizing small foci of carcinoma in an asymmetrical overgrowth of the breast in a young woman with PS.

Keywords: Proteus syndrome, intraductal carcinoma, intraductal papilloma, breast proliferative changes






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