title: Non-androgen secreting adrenocortical carcinoma in preadolescence: a case report and literature review.
abstract: Adrenocortical carcinoma (ACC) is a rare malignancy in childhood. Affected children with ACC mostly present with virilization, but not the pure form of Cushing's syndrome. A 9-year-old Japanese girl was hospitalized, because of the unstable emotions and excessive weight gain. She was diagnosed as having Cushing's syndrome and a left adrenal tumor. The adrenalectomy led to the pathological diagnosis of ACC without metastasis. There was no mutation of PRKACA in the tumor-derived DNA, or p53 in peripheral blood-derived DNA. Testosterone and dehydroepiandrosterone sulfate (DHEA-S) levels were normal throughout the clinical course. On the other hand, these levels were elevated in all five reported cases of preadolescent ACC children with isolated Cushing's syndrome. The exceptional secretory behavior of ACC gave a diagnostic precaution of the rare pediatric cancer.
authors: Hiroko
authors: H
authors: Narumi
authors: Shunji
authors: S
authors: Hasegawa
authors: Kazuyuki
authors: K
authors: Waki
authors: Ken
authors: K
authors: Fukuda
authors: Yuji
authors: Y
authors: Ohnishi
authors: Takuya
authors: T
authors: Ichimura
authors: Yousuke
authors: Y
authors: Fujimoto
authors: Shunsaku
authors: S
authors: Katsura
authors: Hiroo
authors: H
authors: Kawano
authors: Eiji
authors: E
authors: Ikeda
authors: Satoshi
authors: S
authors: Okada
authors: Shouichi
authors: S
authors: Ohga
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000523:psychology / Q000601:surgery; D000311:Adrenal Glands / Q000000981:diagnostic imaging* / Q000601:surgery; D000315:Adrenalectomy; D018268:Adrenocortical Carcinoma / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000523:psychology / Q000601:surgery; D000342:Affective Symptoms / Q000209:etiology / Q000517:prevention & control; D000728:Androgens / Q000097:blood; D002648:Child; D003480:Cushing Syndrome / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D006963:Hyperphagia / Q000209:etiology / Q000517:prevention & control; D007564:Japan; D016896:Treatment Outcome
chemical_list: D000728:Androgens