Results of treatment of pituitary disease in multiple endocrine neoplasia, type I

Neurosurgery. 1996 Aug;39(2):273-8; discussion 278-9. doi: 10.1097/00006123-199608000-00008.


Objective: The aim of the present study was to examine the clinical and pathological features of pituitary disease in patients with multiple endocrine neoplasia, Type I (MEN I) and to assess the prognosis.

Methods: Fifty-two patients with pituitary disease and MEN I were studied retrospectively. Medical records were reviewed, and all of the patients known to be alive were sent a questionnaire to ascertain current disease status.

Results: In 12 patients, pituitary disease was the initial manifestation of MEN I. The most common lesion was prolactinoma, followed, in frequency, by acromegaly and nonsecretory adenoma. Thirty-four of the patients had surgical treatment at the Mayo Clinic, Rochester, MN, as primary treatment, 3 had radiotherapy, and 12 received no specific therapy. Twelve patients had adjunctive radiotherapy postoperatively. Of the 34 patients receiving surgical treatment, 33 had adenoma and 1 had adenoma and pituitary hyperplasia. Immunocytochemical examination demonstrated that many tumors showed reactivity for more than one pituitary hormone. On survival analysis, no excess pituitary-related mortality was found, either in the surgically treated group or in the group as a whole.

Conclusion: On the basis of this study, we conclude that pituitary disease is frequently the initial manifestation of MEN I; that adenomas, particularly prolactinomas, are the rule and hyperplasia is rare; that a significant proportion of tumors are plurihormonal; and that excess pituitary-related mortality is not a factor in patients with MEN I.

MeSH terms

  • Acromegaly / mortality
  • Acromegaly / pathology
  • Acromegaly / radiotherapy
  • Acromegaly / surgery
  • Adenoma / mortality
  • Adenoma / pathology
  • Adenoma / radiotherapy
  • Adenoma / surgery*
  • Adolescent
  • Adult
  • Aged
  • Combined Modality Therapy
  • Female
  • Humans
  • Hypophysectomy
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 1 / mortality
  • Multiple Endocrine Neoplasia Type 1 / pathology
  • Multiple Endocrine Neoplasia Type 1 / radiotherapy
  • Multiple Endocrine Neoplasia Type 1 / surgery*
  • Paraneoplastic Endocrine Syndromes / mortality
  • Paraneoplastic Endocrine Syndromes / pathology
  • Paraneoplastic Endocrine Syndromes / radiotherapy
  • Paraneoplastic Endocrine Syndromes / surgery
  • Pituitary Gland / pathology
  • Pituitary Irradiation
  • Pituitary Neoplasms / mortality
  • Pituitary Neoplasms / pathology
  • Pituitary Neoplasms / radiotherapy
  • Pituitary Neoplasms / surgery*
  • Prolactinoma / mortality
  • Prolactinoma / pathology
  • Prolactinoma / radiotherapy
  • Prolactinoma / surgery
  • Radiotherapy, Adjuvant
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome