Multiple endocrine neoplasm type III. Report of a case.

Contact Person: Alberto G Pizarro (rediegal@homonet.com.mx)

Introduction

Multiple endocrine neoplasia type III (MEN III), also known as MEN IIb or mucosal neuroma syndrome, can often be recognized at an early age by its typical facies, marfanoid habitus, and characteristic mucosal neuromas. These features are usually present before development of the more serious, life-threatening complications that consist of medullary thyroid carcinoma (MTC) and pheochromocytoma.(1,2,3,4,5,6)

Pheochromocytoma is the most frequent cause of adrenal sudden death, which is the unique sign in 1.5% of cases. Pheochromocytoma is an explosive clinical syndrome that may be characterized by either severe hypertension associated with cerebral, cardiac, and renal complications or hypotension, or even shock and sudden death. Other emergencies include lactic acidosis, hypoglycemia, hypercalcemic crisis, severe hypokalemic alkalosis, and acute bowel obstruction due to bowel ischemia, necessitating prompt surgical intervention.(7)

Many difficulties are encountered by clinicians in attempting to diagnose pheochromocytomas. Saudi (8) describe several patients with unusual clinical features. These include sudden death, cerebral hemorrhage, refractory congestive heart failure, acute abdominal pain, and hypercalcemia.

The condition may be familial, having a pattern of inheritance consistent with autosomal dominance. (9,10,11,12,13,14) Von Recklinghausen´s Neurofibromatosis is inherited as an autosomal dominant. It is characterized by a wide variety of cutaneous neurologic, endocrine, skeletal, ocular and visceral manifestations. The findings in the skin provide the best means of diagnosis. These mat range from café au lait spots, usually more than six, to multiple neurofibromas. Spinal and cranial nerves may be involved. Superficial neurofibromata seldom undergo malignant change : neurogenic sarcoma may arise.(10,15,16,17)

Pheochromocytomas are tumors of the adrenal gland which produce excess adrenaline. Pheochromocytoma, a tumor arising from the chromaffin tissue, is a rare and unique disease which is mainly characterized by a great deal of variability in its biological activity and therefore in its clinical manifestation. (11,13,15)

Pheochromocytomas arise from the central portion of the adrenal gland which is called the adrenal medulla. The adrenal medulla is responsible for the normal production of adrenaline which our body requires to help maintain blood pressure (15) Pheochromocytoma occurs with high incidence in this disease, your frequency is 5-20 %.

Pheochromocytomas are often referred to as the "ten percent tumor" because they do many things about ten percent of the time: malignant, bilateral, extraadrenal, in children, familial, recurrent associated with MEN syndromes, presnet with a stroke. (11,12,14,15,18,19,20)

The Multiple endocrine neoplams have neurofibromatosis and pheocromocytoma and others neoplams are MEN type III o IIB. (11,12,16)

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