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A Rare Case of Ectopic Adrenocorticotropic Hormone Syndrome in Small Cell Carcinoma of the Vagina: A Case Report

Weberpals, Johanne MD, MSc, FRCSC1,2; Djordjevic, Bojana MD3; Khalifa, Mahmoud MD, FRCPC3; Oza, Amit MD, FRCPC4

Journal of Lower Genital Tract Disease: April 2008 - Volume 12 - Issue 2 - p 140-145
doi: 10.1097/LGT.0b013e31815cda1e
Case Reports

Objective. To describe ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) to increase awareness of this condition when treating patients with gynecological small cell carcinoma (SmCC).

Materials and Methods. This is a review of a 61-year-old woman with primary vaginal SmCC and an atypical presentation of Cushing syndrome. This case describes the molecular rationale, caveats in clinical presentation, pathological diagnosis, and management options for patients with this rare syndrome.

Results. After treatment with primary chemotherapy, the patient presented acutely with delirium and metabolic disturbances. This was associated with elevated ACTH and gastrin levels without any tumor staining for ACTH. The patient was initially managed with ketoconazole to control the EAS. Complications developed related to her Cushing syndrome, and she succumbed to complications of her disease.

Conclusions. Vaginal SmCC complicated by EAS is a rare paraneoplastic syndrome, and this case history outlines the management options for patients with this condition and reviews the pertinent literature.

A rare case of vaginal small cell carcinoma with ACTH production is reported, highlighting the molecular rationale, clinical presentation, caveats, pathologic diagnosis and management options.

1Division of Gynaecologic Oncology, The Ottawa Hospital, 2Centre for Cancer Therapeutics, Ottawa Health Research Institute, Ottawa, 3Department of Laboratory Medicine and Pathobiology, Sunnybrook Health Sciences Centre, Toronto, and 4Division of Medical Oncology and Hematology, Princess Margaret Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada

Reprint requests to: Johanne Weberpals, MD, MSc, FRCSC, Division of Gynaecologic Oncology, General Campus, The Ottawa Hospital, Room 8130, 501 Smyth Rd, Ottawa, Ontario, K1H 8L6 Canada. E-mail:

This work was supported by a Gynecological Cancer Research Award from the Mitchell Family Fund, National Ovarian Cancer Association and Cancer Care Ontario (J.W.).

©2008The American Society for Colposcopy and Cervical Pathology