Non-androgen secreting adrenocortical carcinoma in preadolescence: a case report and literature review
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Hiroko Narumi
,
Kazuyuki Waki
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.
Acknowledgments
We thank to Drs. Maiko Shimomura (Department of Pediatrics, Yamaguchi University Graduate School of Medicine), Takashi Iwai, and Yuichi Ishikawa (Division of Pediatrics, Yamaguchi-ken Saiseikai Shimonoseki General Hospital) for management.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission. Hiroko Narumi, Shunji Hasegawa, and Shouichi Ohga were the principal investigators taking primary responsibility for the paper. Kazuyuki Waki and Ken Fukuda confirmed the clinical and laboratory data. Kazuyuki Waki, Ken Fukuda, Takuya Ichimura, Yousuke Fujimoto, and Yuji Ohnishi supported the clinical study with helpful discussions. Shunsaku Katsura performed surgical intervention. Hiroo Kawano and Eiji Ikeda performed pathological examination. Satoshi Okada performed the genetic tests. Hiroko Narumi and Shunji Hasegawa wrote the first draft of the manuscript.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
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©2016 Walter de Gruyter GmbH, Berlin/Boston
Artikel in diesem Heft
- Frontmatter
- Editorial
- Puberty – genes, environment and clinical issues
- Puberty
- Increased levels of bisphenol A (BPA) in Thai girls with precocious puberty
- Efficacy and safety of triptorelin 6-month formulation in patients with central precocious puberty
- Monitoring treatment of central precocious puberty using basal luteinizing hormone levels and practical considerations for dosing with a 3-month leuprolide acetate formulation
- Original Articles
- Association between the triglyceride to high-density lipoprotein cholesterol ratio and insulin resistance in Korean adolescents: a nationwide population-based study
- Progression from impaired glucose tolerance to type 2 diabetes in obese children and adolescents: a 3–6-year cohort study in southern Thailand
- Investigation of the inflammatory biomarkers of metabolic syndrome in adolescents
- A 10-year experience using combined lipid-lowering pharmacotherapy in children and adolescents
- Prevalence of medically treated children with ADHD and type 1 diabetes in Germany – Analysis of two representative databases
- Association of short stature with life satisfaction and self-rated health in children and adolescents: the CASPIAN-IV study
- Case Reports
- Central precocious puberty in a boy with 22q13 deletion syndrome and NOTCH-1 gene duplication
- Non-androgen secreting adrenocortical carcinoma in preadolescence: a case report and literature review
- Efficacy of growth hormone therapy in Kearns-Sayre syndrome: the KIGS experience
- A unique case of Shwachman-Diamond syndrome presenting with congenital hypopituitarism
Artikel in diesem Heft
- Frontmatter
- Editorial
- Puberty – genes, environment and clinical issues
- Puberty
- Increased levels of bisphenol A (BPA) in Thai girls with precocious puberty
- Efficacy and safety of triptorelin 6-month formulation in patients with central precocious puberty
- Monitoring treatment of central precocious puberty using basal luteinizing hormone levels and practical considerations for dosing with a 3-month leuprolide acetate formulation
- Original Articles
- Association between the triglyceride to high-density lipoprotein cholesterol ratio and insulin resistance in Korean adolescents: a nationwide population-based study
- Progression from impaired glucose tolerance to type 2 diabetes in obese children and adolescents: a 3–6-year cohort study in southern Thailand
- Investigation of the inflammatory biomarkers of metabolic syndrome in adolescents
- A 10-year experience using combined lipid-lowering pharmacotherapy in children and adolescents
- Prevalence of medically treated children with ADHD and type 1 diabetes in Germany – Analysis of two representative databases
- Association of short stature with life satisfaction and self-rated health in children and adolescents: the CASPIAN-IV study
- Case Reports
- Central precocious puberty in a boy with 22q13 deletion syndrome and NOTCH-1 gene duplication
- Non-androgen secreting adrenocortical carcinoma in preadolescence: a case report and literature review
- Efficacy of growth hormone therapy in Kearns-Sayre syndrome: the KIGS experience
- A unique case of Shwachman-Diamond syndrome presenting with congenital hypopituitarism
