The treatment of sporadic versus MEN1-related pituitary adenomas

J Intern Med. 2003 Jun;253(6):599-605. doi: 10.1046/j.1365-2796.2003.01164.x.

Abstract

The treatment of pituitary tumours strongly depends on their clinical presentation. In general, the treatment aims are reducing tumour volume and/or decreasing hormone hypersecretion. It relies on single or a combination of three different methods: surgery, medication and radiotherapy. The rationale for deciding the treatment are many but include the aggressiveness of the tumour. The aetiologies of sporadic pituitary adenomas are not fully understood. However, several causes have been identified resulting in specific familial phenotypes like multiple endocrine neoplasia type I (MEN1). MEN1 is related to mutations in the MEN1 gene, a tumour suppressor gene localized on chromosome 11q13 and which encodes menin, a 610 amino acid protein. During the last years, an evidence progressively emerged that MEN1-related adenomas were more aggressive and less responsive to therapy than their sporadic counterparts. In this article, we review the differences between sporadic and MEN1-related adenomas and suggest specific ways of treatment and follow-up for MEN1-related tumours.

MeSH terms

  • Acromegaly / drug therapy
  • Acromegaly / radiotherapy
  • Acromegaly / surgery
  • Adenoma / drug therapy
  • Adenoma / radiotherapy
  • Adenoma / surgery*
  • Antineoplastic Agents, Hormonal / therapeutic use
  • Humans
  • Multiple Endocrine Neoplasia Type 1 / drug therapy
  • Multiple Endocrine Neoplasia Type 1 / radiotherapy
  • Multiple Endocrine Neoplasia Type 1 / surgery*
  • Octreotide / therapeutic use
  • Pancreatic Neoplasms / drug therapy
  • Pancreatic Neoplasms / radiotherapy
  • Pancreatic Neoplasms / surgery*
  • Patient Selection
  • Peptides, Cyclic / therapeutic use
  • Prolactinoma / drug therapy
  • Prolactinoma / surgery
  • Somatostatin / analogs & derivatives*
  • Somatostatin / therapeutic use

Substances

  • Antineoplastic Agents, Hormonal
  • Peptides, Cyclic
  • lanreotide
  • Somatostatin
  • Octreotide