Benign Brenner Tumor in an Ectopic Ovary: A Case Report and Review of Literature.

Supernumerary ectopic ovaries are very rare, with fewer than 40 cases of isolated supernumerary ovaries reported in the literature since their discovery in 1864. Tumors arising in ectopic ovaries are also extremely rare, with only a handful of reports in the literature. Given the rarity of this combination of findings, we report a case of a 68-yr-old woman incidentally found to have a 4.7 cm solid retroperitoneal mass adjacent to the liver, diagnosed as a benign Brenner tumor arising in a supernumerary ectopic ovary. To our knowledge, there has been only one previously reported case of Brenner tumor arising in this unusual setting.

Familial Adenomatous Polyposis Syndrome: An Update and Review of Extraintestinal Manifestations.

CONTEXT.­: Familial adenomatous polyposis (FAP) is a rare genetic disorder with autosomal dominant inheritance, defined by numerous adenomatous polyps, which inevitably progress to colorectal carcinoma unless detected and managed early. Greater than 70% of patients with this syndrome also develop extraintestinal manifestations, such as multiple osteomas, dental abnormalities, and a variety of other lesions located throughout the body. These manifestations have historically been subcategorized as Gardner syndrome, Turcot syndrome, or gastric adenocarcinoma and proximal polyposis of the stomach. Recent studies, however, correlate the severity of gastrointestinal disease and the prominence of extraintestinal findings to specific mutations within the adenomatous polyposis coli gene (APC), supporting a spectrum of disease as opposed to subcategorization. Advances in immunohistochemical and molecular techniques shed new light on the origin, classification, and progression risk of different entities associated with FAP. OBJECTIVE.­: To provide a comprehensive clinicopathologic review of neoplastic and nonneoplastic entities associated with FAP syndrome, with emphasis on recent developments in immunohistochemical and molecular profiles of extraintestinal manifestations in the thyroid, skin, soft tissue, bone, central nervous system, liver, and pancreas, and the subsequent changes in classification schemes and risk stratification. DATA SOURCES.­: This review will be based on peer-reviewed literature and the authors' experiences. CONCLUSIONS.­: In this review we will provide an update on the clinicopathologic manifestations, immunohistochemical profiles, molecular features, and prognosis of entities seen in FAP, with a focus on routine recognition and appropriate workup of extraintestinal manifestations.