title: A rare adolescent case of female pseudohermaphroditism with adrenocortical carcinoma and synchronous teratoma.
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.
authors.affiliation: Cancer Center, Division of Abdominal Cancer, West China Hospital of Sichuan University, Chengdu, China.
authors.forename: Xiao-Feng
authors.initials: XF
authors.lastname: He
authors.forename: Xing-Chen
authors.initials: XC
authors.lastname: Peng
authors.forename: Meng
authors.initials: M
authors.lastname: Qiu
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000150:complications* / Q000473:pathology; D000306:Adrenal Cortex Neoplasms / Q000150:complications* / Q000473:pathology; D018268:Adrenocortical Carcinoma / Q000150:complications* / Q000473:pathology; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications* / Q000473:pathology; D013724:Teratoma / Q000150:complications* / Q000473:pathology