Multiple endocrine neoplasia(MEN-1) is also known as Wermer's syndrome. MEN-1 is a hereditory disorder .This type of hereditory disosorder generally passed from one generation to next generation. It affects the hormone producing glands.This disorder increases the development of tumors like cancerous and non cancerous tumors in glands.Non cancerous tumors may develop in parathyroid glands, Neuroendocrine system ,anterior pituitory glands and skin. The symptoms are tiredness, bone pain, fractures and kidney stones, as well as stomach or intestinal ulcers.
The clinical features of our patients are all consistent with MEN 2B. None of the MEN 2A cases examined in this period had evidence of neuromas or early onset of MTC. The codon 918 mutation was present in all of our patients and is the most common mutation. Cascade screening confirmed the mutation to be de novo in all of our cases. No cases due to the rare codon 883 or 922 mutations were identified, and cases with these codon mutations appear to have a similar phenotype to those with the common 918 mutation. In all cases, early prophylactic thyroidectomy is the treatment of choice before 6 months of age; given the aggressive progression in some cases, consideration should be given to thyroid surgery within the first month of life . Planned surgery after testing of cord blood or prenatal testing may be helpful in offspring of those with diagnosed cases; however, recognition of cases in early life may be difficult because all our cases were de novo mutations.