Cushing's disease in a child caused by a corticotropin-releasing hormone-secreting intrasellar gangliocytoma associated with an adrenocorticotropic hormone-secreting pituitary adenoma.
Cushing's disease resulting from intrasellar corticotropin-releasing hormone (CRH)-secreting gangliocytomas is very rare, and only two such cases have been reported in the literature to date. The authors present a third case in which an adrenocorticotropic hormone-secreting pituitary adenoma was found in addition to a gangliocytoma in a 10-year-old girl with clinical and endocrinological symptoms of Cushing's disease. Computed tomographic and magnetic resonance imaging scans showed a suprasellar and parasellar tumor. A green-colored, heterogeneous tumor and a small adenoma were removed transsphenoidally. Histological examination revealed a large gangliocytoma immunoreactive for CRH and a small, mucoid cell pituitary adenoma immunoreactive for ACTH. This is the first case of such a tumor causing Cushing's disease in a child. It might exemplify induction of an ACTH-secreting pituitary adenoma by means of chronic overstimulation of CRH.
Puchner
M J
MJ
Department of Neurosurgery, University of Hamburg, Germany.
Lüdecke
D K
DK
Valdueza
J M
JM
Saeger
W
W
Willig
R P
RP
Stalla
G K
GK
Odink
R J
RJ
D000236:Adenoma / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D002648:Child; D003346:Corticotropin-Releasing Hormone / Q000378:metabolism*; D003480:Cushing Syndrome / Q000097:blood / Q000473:pathology / Q000601:surgery*; D005260:Female; D005729:Ganglioneuroma / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007124:Immunoenzyme Techniques; D008279:Magnetic Resonance Imaging; D016609:Neoplasms, Second Primary / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D010901:Pituitary Function Tests; D010902:Pituitary Gland / Q000473:pathology; D010911:Pituitary Neoplasms / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D011183:Postoperative Complications / Q000097:blood
D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D006854:Hydrocortisone