Table 1.
Basic facts about the different endocrine neoplasia familial syndromes
Fanilial Endocrine Syndrome | Chromosome | Gene | Pattern of inheritance | Glands Involved | Extraglandular manifestations | Cause of death |
---|---|---|---|---|---|---|
MEN-1 | 11 | Menin | Autosomal dominant | Parathyroid Pancreas Pituitary Thyroid Adrenal |
Bronchus Thymus Stomach Lipomas Skin tumors |
Pancreatic NET, Bronchial or thymic NET |
MEN-2a | 10 | RET | Autosomal dominant | Thyroid Adrenal Parathyroid |
None | MTC |
MEN-2b (MEN-3) | 10 | RET | Autosomal dominant | Thyroid Adrenal |
Marfanoid habitus, Mucosal neuromas, Corneal nerve hypertrophy, Intestinal ganglioneruomatosis. | MTC |
FMTC | 10 | RET | Autosomal dominant | Thyroid | None | Natural causes |
MEN-4 | 12 | CDKN1B/p27Kip1 | Autosomal dominant | Parathyroid GEP pituitary |
none | unclear |