Get access

ACTH-secreting Crooke cell carcinoma of the pituitary

Authors


Márta Korbonits, Professor of Endocrinology and Metabolism, Department of Endocrinology, Barts and the London Medical School, Charterhouse Square, London EC1M 6BQ, UK. Tel.: +44 20 7882 6238; fax: +44 20 7882 6197; e-mail: m.korbonits@qmul.ac.uk

Abstract

Eur J Clin Invest 2012

Abstract

Purpose  While pituitary adenomas are common, pituitary carcinomas are rare. It is unclear whether pituitary carcinomas arise de novo or evolve from adenomas.

Methods  We studied the clinical characteristics and tissue samples from eight pituitary surgeries and the autopsy from a patient with pituitary carcinoma. A 16-year-old female patient was diagnosed with an aggressive Crooke cell macroadenoma. Following transsphenoidal surgery, clinical signs of Cushing disease quickly reappeared. During the 14-year course of the illness, eight pituitary surgeries, three courses of extracranial irradiation and two 90Yttrium-DOTATOC treatments were undertaken. A bilateral adrenalectomy was performed. The patient died of metastatic disease and uncontrolled hypercortisolism due to an adrenal remnant. A systematic morphologic study (histologic staining, electron microscopy) of all available surgical and autopsy specimens was undertaken.

Results  Brisk mitotic activity, high Ki-67 and p53 immunolabelling were present in the pituitary samples from the onset. High proportion of tumour cells showed irregular nuclei and large nucleoli, and gradual increase in MGMT staining was observed. The tumour remained of Crooke cell type throughout the course. Autopsy disclosed a postirradiation sarcoma in the pituitary area.

Conclusions  The question whether pituitary carcinomas arise de novo or transform from an adenoma cannot be answered at present with certainty.

Ancillary