Symptoms of Multiple Endocrine Neoplasia

Cancer Facts

According to the National Cancer Institute, around 1 in 30,000 people are affected with each of the two major types of multiple endocrine neoplasia.

Multiple Endocrine Neoplasia Type 1

MEN1 tumors may result in the overproduction of hormones in your body. When specific tumors form in specific endocrine glands, those glands often make too much hormones, which may cause the following:

  • Parathyroid gland – hyperparathyroidism and hypercalcemia
  • Pituitary – oligomenorrhea/amenorrhea and galactorrhea (females) or sexual dysfunction (males)
  • Gastrointestinal tract
    • Gastrinoma – Zollinger-Ellison syndrome (upper abdominal pain, diarrhea, esophageal reflux, acid-peptic ulcers)
    • Insulinoma – hypoglycemia
    • Glucagonoma – hyperglycemia, anorexia, glossitis, anemia, diarrhea, venous thrombosis, skin rash
    • VIPoma – watery diarrhea, hypokalemia, achlorhydria syndrome (lack of hydrochloric acid in the stomach)
  • Carcinoid
    • Diarrhea
    • Facial flushing
    • Wheezing
  • Adrenocortical – hypercortisolism (Cushing’s syndrome due to adrenal tumor) or hyperaldosteronism

MEN1 also may cause the following non-endocrine tumors:

  • Facial angiofibromas – benign blood vessel/connective tissue tumor
  • Collagenomas – cutaneous nodule
  • Lipomas – benign fatty tissue tumor
  • Meningiomas – benign brain tumor
  • Ependymomas – malignant tumor of the central nervous system
  • Leiomyomas – benign smooth muscle tumor

Multiple Endocrine Neoplasia Type 2

In most cases, patients with MEN2 develop medullary thyroid carcinoma (MTC). Signs or symptoms of MTC include:

  • Mass or lump in the neck
  • Goiter (enlarged thyroid)
  • Diarrhea

Around half of patients with MEN2A and MEN2B develop pheochromocytomas; about a third develop primary hyperparathyroidism.

A subset of patients with MEN2A may also develop:

  • Hirschsprung disease
  • Pruritic cutaneous lichen amyloidosis

In many cases, patients with MEN2B will develop the following symptoms:

  • Neuromas and/or ganglioneuromatosis of gastric system
  • Reduced upper/lower body ratio
  • Marfanoid habitus
  • Enlarged lips/bumpy tongue
  • Skeletal abnormalities
  • Joint laxity

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