Bilateral asynchronous adrenocortical adenoma in a girl with Beckwith-Wiedemann syndrome

Michiyo Mizota, Izumi Tamada, Kazuko Hizukuri, Kiyoko Otsubo, Siu Arima, Yoshifumi Kawano, Seigo Ono, Yoshihiro Hayashida, Tatsuru Kaji, Hideo Takamatsu, Hironobu Sasano

研究成果: Article査読

2 被引用数 (Scopus)

抄録

We report a case of asynchronous occurrence of bilateral adrenocortical adenoma in a 13-yr-old girl with Beckwith-Wiedemann syndrome. A right virilizing adrenal adenoma was surgically removed at age 6, following clinical manifestation of virilization such as acne, voice change, clitoris hypertrophy and overgrowth. Histopathological examination of the resected specimen revealed an adrenocortical adenoma predominantly composed of eosinophilic tumor cells expressing all the steroidogenic enzymes. High serum levels of DHEA-S (6,380 ng/ml) and testosterone (547 ng/dl) were noted prior to the operation. Postoperative course was unremarkable. Menstruation started at age 11, with a regular interval. At the age of 13 yr old, a high serum level of DHEA-S (8,250 ng/ml) was detected. In contrast to the episode of virilization at age 6, however, the serum testosterone level was not so high (122 ng/dl), and no clinical symptoms of virilization were apparent. Abdominal ultrasonography demonstrated the presence of a left adrenocortical adenoma. Pathological examination of the resected specimen revealed a circumscribed and well encapsulated tumor with essentially the same histological features as the tumor previously removed, except that the tumor cells showed a more prominent morphological similarity to the fetal adrenal cortex and did not express 3βHSD. The absence of virilization at the second episode was due to the relatively low serum level of testosterone compared with that of DHEA-S. Copyright

本文言語English
ページ(範囲)23-26
ページ数4
ジャーナルClinical Pediatric Endocrinology
14
1
DOI
出版ステータスPublished - 2005

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康
  • 内分泌学、糖尿病および代謝内科学
  • 内分泌学

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