The table below summarises the three main types of multiple endocrine neoplasia (MEN). MEN is inherited as an autosomal dominant disorder.

2 P's Parathyroid (60%) Phaeochromocytoma | Medullary thyroid cancer • prophylactic thyroidectomy within first months of life 1 P Phaeochromocytoma Marfanoid body habitus Neuromas | | MEN1 gene Most common presentation = hypercalcaemia | RET oncogene | RET oncogene |

Multiple Endocrine Neoplasia (MEN)

All patients with new MTC require serum calcitonin and carcinoembryonic antigen, neck ultrasound (evaluation for regional metastases), genetic testing for germline RET mutations, and evaluation for coexisting tumors (hyperparathyroidism, pheochromocytomas)

MEN-1

Wermer's syndrome

MEN 2A (Sipple syndrome)

Polyglandular Autoimmune Syndrome

Essentially, 2 types of PGA exist, type I and the more common type II, also known as Schmidt syndrome. A third type (type III), which occurs in adults, has been described. Type III does not involve the adrenal cortex, but it includes 2 of the following: thyroid deficiency, pernicious anemia, type 1A diabetes mellitus, vitiligo, and alopecia.

PGA-I, or autoimmune polyglandular failure type 1, also known as autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) or as Whitaker syndrome, is associated with candidiasis, hypoparathyroidism, and adrenal failure. Sporadic or AR. No HLA association

PGA-II, most common of the immunoendocrinopathy syndromes, is characterized by the obligatory occurrence of autoimmune Addison disease in combination with thyroid autoimmune diseases and/or type 1 DM. Primary hypogonadism, myasthenia gravis, and celiac disease also are commonly observed in this syndrome. AD with variable  expressibility; HLA DR3/4 association