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

The manifestations of multiple endocrine neoplasia type 1 usually develop in the first, second or third decade of life and the age at which multiple endocrine neoplasia type 1 can begin to cause symptoms can vary from one family member to another. 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 manifestations of multiple endocrine neoplasia type 1 usually develop in the first/ second or third decade of life and the age at which multiple endocrine neoplasia type 1 can begin to cause symptoms can vary from one family member to another.
• Multiple endocrine neoplasia type 1 cannot be cured, but regular testing can help in early diagnosis and treatment. Even after treatment, recurrence can occur. Most people with multiple endocrine neoplasia type 1 have a long and productive life.

Complications

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

• Necrolytic migratory erythema
• Hypoglycemia
• Hypokalemia
• Achlorhydria
• Hypercalcemia
• Diabetes mellitus
• Cushing's syndrome
• Gynecomastia
• Gigantism
• Acromegaly
• Paresis
• Ataxia
• Convulsions
• Infertility
• Impotence
• Kidney stones
• Venous thrombosis
• Glossitis
• Anemia

Prognosis

• The prognosis of multiple endocrine neoplasia type 1 is good with treatment.
• Early death can occur due to the effect of hormones produced by these tumors.
• Although many tumors associated with multiple endocrine neoplasia type 1 are benign, about half of people with multiple endocrine neoplasia type 1 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.