ACTH-secreting Crooke cell carcinoma of the pituitary

Eur J Clin Invest. 2013 Jan;43(1):20-6. doi: 10.1111/eci.12010. Epub 2012 Nov 7.

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 (90) Yttrium-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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • ACTH-Secreting Pituitary Adenoma / pathology*
  • ACTH-Secreting Pituitary Adenoma / therapy
  • Adolescent
  • Adrenalectomy
  • Carcinoma / pathology*
  • Carcinoma / therapy
  • Diagnosis, Differential
  • Fatal Outcome
  • Female
  • Humans
  • Ki-67 Antigen / analysis
  • Liver Neoplasms / pathology
  • Liver Neoplasms / secondary
  • Nelson Syndrome / pathology*
  • Neoplasm Metastasis / pathology
  • Pituitary ACTH Hypersecretion / etiology
  • Pituitary Gland / metabolism
  • Pituitary Gland / pathology*
  • Pituitary Neoplasms / pathology*
  • Pituitary Neoplasms / therapy
  • Tumor Suppressor Protein p53 / analysis
  • Young Adult

Substances

  • Ki-67 Antigen
  • Tumor Suppressor Protein p53