TY - JOUR
T1 - Non-androgen secreting adrenocortical carcinoma in preadolescence
T2 - A case report and literature review
AU - Narumi, Hiroko
AU - Hasegawa, Shunji
AU - Waki, Kazuyuki
AU - Fukuda, Ken
AU - Ohnishi, Yuji
AU - Ichimura, Takuya
AU - Fujimoto, Yousuke
AU - Katsura, Shunsaku
AU - Kawano, Hiroo
AU - Ikeda, Eiji
AU - Okada, Satoshi
AU - Ohga, Shouichi
N1 - Publisher Copyright:
© 2016 Walter de Gruyter GmbH, Berlin/Boston.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2016/11/1
Y1 - 2016/11/1
N2 - 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.
AB - 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.
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U2 - 10.1515/jpem-2016-0145
DO - 10.1515/jpem-2016-0145
M3 - Review article
C2 - 27771624
AN - SCOPUS:84994908291
SN - 0334-018X
VL - 29
SP - 1313
EP - 1317
JO - Journal of Pediatric Endocrinology and Metabolism
JF - Journal of Pediatric Endocrinology and Metabolism
IS - 11
ER -
