Multiple endocrine neoplasia type 1 natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.

Natural History

The symptoms of multiple endocrine neoplasia type 1 usually develop in the first/ second/ third decade of life and the age at which MEN1 can begin to cause endocrine gland overactivity can differ strikingly from one family member to another. One person may have only mild hyperparathyroidism beginning at age 50, while a relative may develop complications from tumors of the parathyroid, pancreas, and pituitary by age 18. MEN1 cannot be cured, but regular testing can detect many of the problems caused by MEN1 tumors many years before serious complications develop. Even after treatment, residual tissue can grow back or different glands may become affected. Most people with MEN1 have a long and productive life.

Complications

  • Complications that can develop as a result of multiple endocrine neoplasia type 1 are as below.[1]

Prognosis

  • The prognosis of multiple endocrine neoplasia type 1 is good with treatment.
  • Most people with MEN1 have a long and productive life.
  • Early death can occur due to the effect of hormones produced by these tumors.
  • Although many tumors associated with MEN1 are benign, about half of people with MEN1 will eventually develop a cancerous tumor.
  • The presence of pancreatic tumors is associated with a particularly poor prognosis among patients with multiple endocrine neoplasia type 1.

References

  1. Falchetti A, Marini F, Luzi E, Giusti F, Cavalli L, Cavalli T; et al. (2009). "Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors". Genet Med. 11 (12): 825–35. doi:10.1097/GIM.0b013e3181be5c97. PMID 19904212.