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Table 5. MEN Syndromes with Associated Clinical and Genetic Alterations

Syndrome Clinical Features/Tumors Genetic Alterations 
MEN type 1: Werner syndrome [2]Parathyroid 11q13 (MEN1 gene)
Pancreatic islets: Gastrinoma11q13 (MEN1 gene)
Insulinoma
Glucagonoma
VIPoma
Pituitary: Prolactinoma11q13 (MEN1 gene)
Somatotrophinoma
Corticotropinoma
Other associated tumors: Carcinoid: bronchial and thymic11q13 (MEN1 gene)
Adrenocortical
Lipoma
MEN type 2A: Sipple syndrome Medullary thyroid carcinoma 10q11.2 (RET gene)
Pheochromocytoma
Parathyroid gland
MEN type 2B Medullary thyroid carcinoma 10q11.2 (RET gene)
Pheochromocytoma
Mucosal neuromas
Intestinal ganglioneuromatosis
Marfanoid habitus
Familial medullary thyroid carcinoma Medullary thyroid carcinoma 10q11.2 (RET gene)

References

  1. Thakker RV: Multiple endocrine neoplasia--syndromes of the twentieth century. J Clin Endocrinol Metab 83 (8): 2617-20, 1998.  [PUBMED Abstract]