Non-androgen secreting adrenocortical carcinoma in preadolescence: A case report and literature review

Hiroko Narumi, Shunji Hasegawa, Kazuyuki Waki, Ken Fukuda, Yuji Ohnishi, Takuya Ichimura, Yousuke Fujimoto, Shunsaku Katsura, Hiroo Kawano, Eiji Ikeda, Satoshi Okada, Shouichi Ohga

Research output: Contribution to journalReview article

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.

Original languageEnglish
Pages (from-to)1313-1317
Number of pages5
JournalJournal of Pediatric Endocrinology and Metabolism
Volume29
Issue number11
DOIs
Publication statusPublished - Nov 1 2016

Fingerprint

Adrenocortical Carcinoma
Cushing Syndrome
Virilism
Neoplasms
Dehydroepiandrosterone Sulfate
Glandular and Epithelial Neoplasms
DNA
Adrenalectomy
Weight Gain
Testosterone
Emotions
Pediatrics
Neoplasm Metastasis
Mutation

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

Cite this

Non-androgen secreting adrenocortical carcinoma in preadolescence : A case report and literature review. / Narumi, Hiroko; Hasegawa, Shunji; Waki, Kazuyuki; Fukuda, Ken; Ohnishi, Yuji; Ichimura, Takuya; Fujimoto, Yousuke; Katsura, Shunsaku; Kawano, Hiroo; Ikeda, Eiji; Okada, Satoshi; Ohga, Shouichi.

In: Journal of Pediatric Endocrinology and Metabolism, Vol. 29, No. 11, 01.11.2016, p. 1313-1317.

Research output: Contribution to journalReview article

Narumi, H, Hasegawa, S, Waki, K, Fukuda, K, Ohnishi, Y, Ichimura, T, Fujimoto, Y, Katsura, S, Kawano, H, Ikeda, E, Okada, S & Ohga, S 2016, 'Non-androgen secreting adrenocortical carcinoma in preadolescence: A case report and literature review', Journal of Pediatric Endocrinology and Metabolism, vol. 29, no. 11, pp. 1313-1317. https://doi.org/10.1515/jpem-2016-0145
Narumi, Hiroko ; Hasegawa, Shunji ; Waki, Kazuyuki ; Fukuda, Ken ; Ohnishi, Yuji ; Ichimura, Takuya ; Fujimoto, Yousuke ; Katsura, Shunsaku ; Kawano, Hiroo ; Ikeda, Eiji ; Okada, Satoshi ; Ohga, Shouichi. / Non-androgen secreting adrenocortical carcinoma in preadolescence : A case report and literature review. In: Journal of Pediatric Endocrinology and Metabolism. 2016 ; Vol. 29, No. 11. pp. 1313-1317.
@article{7afde0d550594c439b9b881f01b6c2f5,
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.",
author = "Hiroko Narumi and Shunji Hasegawa and Kazuyuki Waki and Ken Fukuda and Yuji Ohnishi and Takuya Ichimura and Yousuke Fujimoto and Shunsaku Katsura and Hiroo Kawano and Eiji Ikeda and Satoshi Okada and Shouichi Ohga",
year = "2016",
month = "11",
day = "1",
doi = "10.1515/jpem-2016-0145",
language = "English",
volume = "29",
pages = "1313--1317",
journal = "Journal of Pediatric Endocrinology and Metabolism",
issn = "0334-018X",
publisher = "Walter de Gruyter GmbH & Co. KG",
number = "11",

}

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

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.

UR - http://www.scopus.com/inward/record.url?scp=84994908291&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84994908291&partnerID=8YFLogxK

U2 - 10.1515/jpem-2016-0145

DO - 10.1515/jpem-2016-0145

M3 - Review article

C2 - 27771624

AN - SCOPUS:84994908291

VL - 29

SP - 1313

EP - 1317

JO - Journal of Pediatric Endocrinology and Metabolism

JF - Journal of Pediatric Endocrinology and Metabolism

SN - 0334-018X

IS - 11

ER -