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Borderline Ovarian Mucinous Neoplasm Recurring as Small Cell Carcinoma of Hypercalcemic Type: Evidence for an Epithelial Histogenesis and Relationship With Ovarian Mucinous Tumors for This Enigmatic Neoplasm

Mansor, Sorsiah; Nagarajan, Sri; Sumathi, Vaiyapuri P.; McCluggage, W. Glenn

International Journal of Gynecological Pathology: July 2011 - Volume 30 - Issue 4 - p 380–385
doi: 10.1097/PGP.0b013e318209aebc

Ovarian small cell carcinoma of hypercalcemic type (OSCCHT) is an uncommon neoplasm of uncertain histogenesis. As far as we are aware, this neoplasm has never been reported in association with another primary ovarian tumor. We report a case in a 33-year-old patient in whom an extraovarian pericolonic neoplasm with the morphological features and immunohistochemical profile of OSCCHT developed 5 years after removal of an ovarian mucinous borderline tumor of the intestinal type. Together with the observation that mucinous epithelium is seen in some OSCCHTs, this case raises the possibility that this enigmatic neoplasm is of epithelial origin and is related to primary ovarian mucinous neoplasms. The pericolonic tumor exhibited an unusual immunophenotype with positive staining with CD99 and FLI-1, suggesting the possibility of a neoplasm in the Ewing family of tumors. This was excluded by molecular studies that showed no evidence of EWS/FLI-1 or EWS/ERG translocation.

Department of Pathology (S.M., W.G.M.), Belfast Health and Social Care Trust, Belfast, Northern Ireland

Department of Pathology (S.N.), The James Cook University Hospital, Middlesbrough

Department of Musculoskeletal Pathology (V.P.S.), The Royal Orthopaedic Hospital NHS Foundation Trust, England

Address correspondence and reprint requests to W. Glenn McCluggage, Department of Pathology, Royal Group of Hospitals Trust, Grosvenor Road, Belfast BT12 6BA, Northern Ireland. e-mail:

©2011International Society of Gynecological Pathologists