Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A갈색세포종에 의한 이소성 부신피질자극호르몬 증후군을 동반한 제2형 다발성내분비 선종증 1예
- Other Titles
- 갈색세포종에 의한 이소성 부신피질자극호르몬 증후군을 동반한 제2형 다발성내분비 선종증 1예
- Authors
- 문지미; 김윤정; 서영진; 최혜윤; 김주형; 박주리; 이윤정; 김희영; 김신곤; 최동섭
- Issue Date
- Oct-2009
- Publisher
- 대한내분비학회
- Keywords
- ACTH syndrome; ectopic; multiple endocrine neoplasia type 2A; pheochromocytoma
- Citation
- Endocrinology and Metabolism, v.24, no.4, pp 265 - 271
- Pages
- 7
- Indexed
- KCI
- Journal Title
- Endocrinology and Metabolism
- Volume
- 24
- Number
- 4
- Start Page
- 265
- End Page
- 271
- URI
- https://scholarworks.korea.ac.kr/kumedicine/handle/2020.sw.kumedicine/35531
- ISSN
- 2093-596X
2093-5978
- Abstract
- Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome characterized by the
presence of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN 2A arises due
to a germline missense mutation of the RET proto-oncogene. Specific RET mutation analysis has
revolutionized the diagnosis and therapy of this disorder, and early thyroidectomy may have lowered the
morbidity and mortality associated with these diseases. Ectopic adrenocorticotropic hormone (ACTH) syndrome
is characterized by hypercortisolism due to the hypersecretion of ACTH outside of the pituitary gland; the
most common causes are malignancies, but rarely adrenal pheochromocytoma may be the cause.
We describe here a case of ectopic ACTH syndrome with bilateral pheochromocytoma in a 29-year-old man
with MEN 2A presenting with medullary thyroid carcinoma and hyperparathyroidism. MEN 2A was confirmed
by the detection of the RET proto-oncogene mutation. The laboratory results were compatible with ectopic
ACTH syndrome. Immunohistochemical studies of the pheochromocytoma tissue confirmed the etiology of the
ACTH secretion. This is the first confirmed case of the ectopic ACTH syndrome with bilateral
pheochromocytoma in a Korean patient with MEN 2A. (J Korean Endocr Soc 24:265~271, 2009)
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