ACTH-producing carcinoma of the pituitary with refractory Cushing's Disease and hepatic metastases: a case report and review of the literature.
BACKGROUND
Pituitary carcinomas are rare neuroendocrine tumors affecting the adenohypophysis. The hallmark of these lesions is the demonstration of distant metastatic spread. To date, few well-documented cases have been reported in the literature.


METHODS
Here, we report the case of a fatal pituitary carcinoma evolving within two years from an adrenocorticotrophic hormone (ACTH)-secreting macroadenoma and review the global literature regarding this rare neuroendocrine tumor.


CONCLUSIONS
Pituitary carcinomas are extremely rare neoplasms, representing only 0.1% to 0.2% of all pituitary tumors. To date, little is understood about the molecular basis of malignant transformation. The latency period between initial presentation of a pituitary adenoma and the development of distal metastases marking carcinoma is extremely variable, and some patients may live well over 10 years with pituitary carcinoma.
Pinchot
Scott N
SN
Section of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, WI, USA. spinchot@uwhealth.org
Sippel
Rebecca
R
Chen
Herbert
H
D049913:ACTH-Secreting Pituitary Adenoma / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005260:Female; D016158:Genes, p53; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010281:Parathyroid Hormone / Q000097:blood; D047748:Pituitary ACTH Hypersecretion / Q000150:complications*
D010281:Parathyroid Hormone