ABSTRACT
MEN IIa is the rare disorder consisted of thyroid medullary carcinoma, pheochromocytoma, and hyperparathyroidism. We experienced the case in which 42 year-old male patient with thyroid medullary carcinoma and pheochromocytoma complicated by acute myocardial infarction. During the process of conventional treatment of acute myocardial infarction, paroxysmal hypertension occurred for several times. We sought for the cause of paroxysmal hypertension, and found pheochromocytoma by the radiologic imaging study and the biochemical study and we found the 4X4 cm sized neck mass by palpation. After stabilizing his blood pressure by the use of phenoxybenzamine, we removed the pheochromocytoma in right adrenal gland and the medullary thyroid cancer, by right adrenalectomy and total thyroidectomy respectively. Thereafter, his subjective symptoms and objective signs were improved. We report the case with review of literatures.
Subject(s)
Adult , Humans , Male , Adrenal Glands , Adrenalectomy , Blood Pressure , Carcinoma, Medullary , Hyperparathyroidism , Hypertension , Multiple Endocrine Neoplasia Type 2a , Multiple Endocrine Neoplasia , Myocardial Infarction , Neck , Palpation , Phenoxybenzamine , Pheochromocytoma , Thyroid Gland , Thyroid Neoplasms , ThyroidectomyABSTRACT
Multiple endocrine neoplasia (MEN) Ila is an inherited disease characterized by the development of medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. It has been shown to be associated with germ-line mutatians in the RET proto-oncogene. Presymptomatic screening of medullary thyroid carcinoma in MEN IIa families enables the early diagnosis of this tumor with its significant morbidity, We describe a 19-year-old woman fmm a MEN IIa family who was founded by DNA analysis to be a gene carrier of MEN IIa and then was diagnosed, using a pentagastrin stimulation test, as having presymptomatie medullary thyroid carcinoma She underwent thyroidectomy and histologic examination confirmed medullary thyroid carcinoma. It is cancluded that direct genetic analysis for mutations in the RET proto-oncogene should be the diagnstlc test of choice for identifying family members at risk for MEN IIa and thyroidectomy on the basis of genetic analysis is a rational course of action.