Skip Navigation LinksHome > July 2013 - Volume 35 - Issue 5 > A Rare Adolescent Case of Female Pseudohermaphroditism With...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e318286d112
Online Articles: Original Articles

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

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

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Abstract

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.

Copyright © 2013 by Lippincott Williams & Wilkins

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