Dr Sheraz saleem z Multiple Endocrine Neoplasia z

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Dr. Sheraz saleem z Multiple Endocrine Neoplasia

Dr. Sheraz saleem z Multiple Endocrine Neoplasia

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z MEN type 1 Autosomal dominant condition with prevalence of 1 in 10, 000

z MEN type 1 Autosomal dominant condition with prevalence of 1 in 10, 000 of the population, Characterized by • • • Parathyroid tumours / hyperplasia Anterior Pituitary adenomas Pancreatic neuroendocrine tumours Requires 2 out of above 3 for the diagnosis or 1 of the above in the context of a family history MEN – 1 gene is located on chromosome 11 and is a tumour suppressor gene

z Clinical Features § The disease has been reported to manifest itself from the

z Clinical Features § The disease has been reported to manifest itself from the ages of 5 to 80 years § High mortality due to aggressive tumours § Commonest presentation ------ Primary hyperparathyroidism manifests as hypercalcemia § 2 nd most common presentation --- insulinomas or prolactinomas

z Management of MEN type 1 § Primary Hyperparathyroidism Surgical management is the gold

z Management of MEN type 1 § Primary Hyperparathyroidism Surgical management is the gold standard § Pituitary Tumours Surgery, medical therapies or radiotherapy § Gastrinoma Non Metastatic ----- Surgery Metastatic ------ Poor prognosis

z MEN Type 2 MEN-2 may be divided into two forms, § MEN-2 A

z MEN Type 2 MEN-2 may be divided into two forms, § MEN-2 A comprises of familial medullary thyroid carcinoma in combination with phaeochromocytoma and parathyroid tumours § MEN-2 B is combination of FMTC in association with phaeochromocytoma, mucosal neuromas and a marfanoid habitus § MEN – 2 is an autosomal dominant condition § C-RET proto-oncogene is located on long arm of chromosome 10

z Clinical Features---MEN-2 A § Initial manifestation is often with medullary carcinoma of thyroid

z Clinical Features---MEN-2 A § Initial manifestation is often with medullary carcinoma of thyroid and is generally multifocal § Calcitonin is generally elevated however hypocalcemia is rare § Phaeochromocytoma and parathyroid disease typically develop later

z Clinical Features---MEN - 2 B § Mucosal neuromas are found on distal tongue,

z Clinical Features---MEN - 2 B § Mucosal neuromas are found on distal tongue, conjunctiva and throughout the GIT ( causing malabsorption ) § A marfanoid habitus is typically evident

z Management of MEN type 2 § Medullary carcinoma of thyroid Definitive treatment is

z Management of MEN type 2 § Medullary carcinoma of thyroid Definitive treatment is total thyroidectomy along with lymph node dissection § Phaeochromocytoma Best treated medically with alpha and beta blockade followed by surgical removal § Primary hyperparathyroidism Symptomatic or surgical removal

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