ICEECE2012 Poster Presentations Endocrine tumours and neoplasia (112 abstracts)
Virilization due to adrenocortical carcinoma in a 7 years-old female: a case report
M. Furujo 1 , Y. Higuchi 1 , H. Usui 1 , T. Asai 1 , T. Goto 1 , Y. Shinno 1 , I. Yamadori 1 , K. Homma 2 , T. Hasegawa 3 & T. Kubo 1
1National Hospital Organization Okayama Medical Center, Okayama, Japan; 2Keio University Hospital, Tokyo, Japan; 3Keio University School of Medicine, Tokyo, Japan.
Adrenal cortical carcinoma (ACC) is a rare malignancy, especially in children, and the annual worldwide incidence has been calculated as 0.3 cases per million children younger than 15 years old. We present a case of functioning ACC in a 7 years-old female child who manifested with virilization. She presented with low-pitched voice, excessive pubic hair growth, growth acceleration, clitoral enlargement and increased muscle strength at the age of six. Her blood pressure was normal, and her breast was Tanner stage 1, but her bone age was advanced. Hormonal findings were characterized as an increase of androgens, especially serum testsosterone, DHEAS and urinary 17-ketosteroid (17-KS). The abdominal magnetic resonance imaging revealed a large right suprarenal mass sized 55×50×40 mm. The brain, lung and liver were free from metastases in MRI and CT. She had a right adrenalectomy without any complications. The histological diagnosis was ACC according to the criteria of Weiss: nuclear atypia, atypical architecture and atypical mitosis; and Weiss criteria three out of nine. The labeling index of Ki67 staining was 10–15%, and DNA analysis of her peripheral leukocytes for the p53 tumor suppressor gene was negative. After histopathological confirmation of the diagnosis she received mitotane treatment (2 g/day) and radiation therapy. Two weeks after adrenalectomy, her serum testosterone and DHEAS and urinary 17-KS rapidly decreased to the normal level. We must pay attention to recurrences because little is known of ACC’s long-term natural history.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.
Article tools
My recent searches
My recently viewed abstracts
Authors
Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
© Bioscientifica 2024 |
Privacy policy |
Cookie settings
BiosciAbstracts
Bioscientifica Abstracts is the gateway to a series of products that provide a permanent, citable record of abstracts for biomedical and life science conferences.
Find out more