title: ACTH-secreting Crooke cell carcinoma of the pituitary.
abstract: OBJECTIVE
  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.
authors.affiliation: Division of Endocrinology, Department of Internal Medicine, Military Hospital State Health Center, Budapest, Hungary.
authors.forename: Gábor L
authors.initials: GL
authors.lastname: Kovács
authors.forename: Miklós
authors.initials: M
authors.lastname: Góth
authors.forename: Fabio
authors.initials: F
authors.lastname: Rotondo
authors.forename: Bernd W
authors.initials: BW
authors.lastname: Scheithauer
authors.forename: Eivind
authors.initials: E
authors.lastname: Carlsen
authors.forename: Ali
authors.initials: A
authors.lastname: Saadia
authors.forename: Erika
authors.initials: E
authors.lastname: Hubina
authors.forename: László
authors.initials: L
authors.lastname: Kovács
authors.forename: István
authors.initials: I
authors.lastname: Szabolcs
authors.forename: Pál
authors.initials: P
authors.lastname: Nagy
authors.forename: Sándor
authors.initials: S
authors.lastname: Czirják
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Hanzély
authors.forename: Kálmán
authors.initials: K
authors.lastname: Kovács
authors.forename: Éva
authors.initials: É
authors.lastname: Horváth
authors.forename: Márta
authors.initials: M
authors.lastname: Korbonits
mesh_terms: D049913:ACTH-Secreting Pituitary Adenoma / Q000473:pathology* / Q000628:therapy; D000293:Adolescent; D000315:Adrenalectomy; D002277:Carcinoma / Q000473:pathology* / Q000628:therapy; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D019394:Ki-67 Antigen / Q000032:analysis; D008113:Liver Neoplasms / Q000473:pathology / Q000556:secondary; D009347:Nelson Syndrome / Q000473:pathology*; D009362:Neoplasm Metastasis / Q000473:pathology; D047748:Pituitary ACTH Hypersecretion / Q000209:etiology; D010902:Pituitary Gland / Q000378:metabolism / Q000473:pathology*; D010911:Pituitary Neoplasms / Q000473:pathology* / Q000628:therapy; D016159:Tumor Suppressor Protein p53 / Q000032:analysis; D055815:Young Adult
chemical_list: D019394:Ki-67 Antigen; D016159:Tumor Suppressor Protein p53