Endocrine Abstracts

MEN (multiple endocrine neoplasia) I disease represents a compilation of multiple endocrine neoplasms affecting a patient in the course of a life-time. It thus represents a phenotype which has survived over many years. Pituitary neoplasms, lipomas and parathyroid hyperplasia are observed in the disease. The aim was to describe a patient with MEN I disease who presented with lipomas and acromegaly in his early adulthood and developed primary hyperparathyroidism in his senior years. A patient presented with lipomas at the age of 35 years. At the age of 36 years acromegaly was diagnosed as the patient had increased perspiration, weight gain, increased growth of his hands and feet and headaches. A diagnostic evaluation revealed the presence of acromegaly due to a pituitary tumor in the left side of the pituitary gland. The pituitary tumor was excised transsphenoidally. Following the surgical removal of the pituitary adenoma the patient improved dramatically and lost 10 kg of weight within a few weeks. Thirty-six years later a thyroid ultrasound was performed which showed a nodule inferior to the left thyroid lobe. A diagnostic evaluation revealed increased PTH levels 76 pg/ml, mildly elevated blood calcium levels 10.1 mg/dl and normal 24 h urinary calcium levels. Bone mineral density was normal. An abdominal ultrasound was performed which did not reveal the presence of kidney stones. The diagnosis of mild hyperparathyroidism was made. Conservative management was decided. MEN I disease is a genetic disease which has been transmitted in families allowing the survival of the patients and the genotype and corresponding phenotype. Moreover, primary hyperparathyroidism in the case of MEN I is due to parathyroid hyperplasia. Additionally, some of the tumors present in MEN I patients may be completely silent for many years. It is therefore proposed that the management of hyperparathyroidism should preferably be conservative as it is due to parathyroid hyperplasia and may be mild. Hence, surgical removal, if decided should lead to the excision of more than one parathyroid glands. Additionally, complete removal of the parathyroid glands leads to hypoparathyroidism, a disease known for its notoriously difficult management. Thus, in the management of tumors diagnosed in the course of MEN I conservative management should be considered, if clinical findings are mild and the tumor does not cause obstructive or other size related manifestations.