Unusual fallopian tube neoplasm in a 10-year-old patient with Proteus syndrome: a case report and review of the literature.

TitleUnusual fallopian tube neoplasm in a 10-year-old patient with Proteus syndrome: a case report and review of the literature.
Publication TypeJournal Article
Year of Publication2006
AuthorsFunk T, Frew P, Rodgers W, Troxell M
JournalPediatr Dev Pathol
Volume9
Issue4
Pagination316-20
Date Published2006 Jul-Aug
ISSN1093-5266
KeywordsBiomarkers, Tumor, CA-125 Antigen, Calbindin 2, Child, Epithelium, Fallopian Tube Neoplasms, Fallopian Tubes, Female, Humans, Hyperplasia, Proteus Syndrome, S100 Calcium Binding Protein G, Treatment Outcome
Abstract

We report a case of fallopian tube and peritoneal lesions resected from a 10-year-old girl with Proteus syndrome. The papillary fallopian tube tumor expressed CA-125 and demonstrated negative staining for CEA, CD15, calretinin, and CK 5/6. Nondilated portions of fallopian tube exhibited evidence of an in situ component. This patient was also found to have peritoneal mesothelial hyperplasia as well as cystic peritoneal lesions of mesothelial origin, with positive calretinin staining. This case provides direct evidence of both Müllerian and mesothelial lesions arising concurrently in a patient with Proteus syndrome and represents the 1st documented fallopian tube neoplasm. Previously reported lesions involving the female and male genital tracts in patients with Proteus syndrome are reviewed.

DOI10.2350/06-01-0016.1
Alternate JournalPediatr Dev Pathol
PubMed ID16944982
Related Faculty: 
William Rodgers, M.D., Ph.D.

Pathology & Laboratory Medicine 1300 York Avenue New York, NY 10065 Phone: (212) 746-6464
Surgical Pathology: (212) 746-2700