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A Rare Adolescent Case of Female Pseudohermaphroditism With Adrenocortical Carcinoma and Synchronous Teratoma

He, Xiao-Feng MD*,†; Peng, Xing-Chen MD*; Qiu, Meng MD*

Journal of Pediatric Hematology/Oncology: July 2013 - Volume 35 - Issue 5 - p e183–e186
doi: 10.1097/MPH.0b013e318286d112
Online Articles: Original Articles

A patient with female pseudohermaphroditism is chromosomally and gonadally a female individual but has male or ambiguous external genitalia. In this paper, we report a 12-year-old Chinese girl who was diagnosed with female pseudohermaphroditism characterized by clitoridauxe, hirsutism, acne, hypertension, and karyotype 46 XX. Computed tomography scan revealed a huge left abdominal mass with distant metastases to bilateral lungs and a concomitant pelvic teratoma. Because the left abdominal mass was unresectable, the patient underwent a biopsy of the abdominal mass and a radical resection of the pelvic teratoma. Histopathology confirmed that the left abdominal mass was an adrenocortical carcinoma (ACC) and the pelvic teratoma was a mature cystic teratoma originating from the left ovary. After surgery, the patient received a transcatheter arterial chemoembolization of ACC, combined with 2 g mitotane daily for systemic treatment. It was a pity that she died 8 months later after diagnosis. So far, as we know, the simultaneous occurrence of pseudohermaphroditism, ACC, and ovarian teratomas has not been reported in the literatures before.

*Cancer Center, Division of Abdominal Cancer, West China Hospital of Sichuan University

Department of Medical Oncology, The First People's Hospital of Longquanyi District, Chengdu, China

The authors declare no conflict of interest.

Reprints: Meng Qiu, MD, Cancer Center, Division of Abdominal Cancer, West China Hospital of Sichuan University, 37 Guoxue Xiang Street, Chengdu, Sichuan Province, China 610041 (e-mail: qiumeng33@hotmail.com).

Received March 23, 2012

Accepted January 8, 2013

© 2013 by Lippincott Williams & Wilkins.