WHEN EVEN THE CLASSICS REQUIRE CLOSE SURVEILLANCE – A TYPICAL CASE OF MEN SYNDROME

Abstract

The genetic syndrome known in literature as the Multiple Endocrine Neoplasia type 1 (MEN 1) is transmitted in a dominant manner. The primary discovery of more than two endocrine tumor formations representative for MEN 1 should guide the doctor to the suspicion and subsequent confirmation of the diagnosis. Statistics state a long association of the MEN 1 syndrome with the tumors discovered in the small intestine and the pancreas with the adenomas encountered in the parathyroid and pituitary glandular tissueWhile in postmenopausal women, the primary hyperparathyroidism is diagnosed mostly with abnormal levels of parathyroid hormone and serum calcium specifically. The most useful preoperative imaging tools are neck ultrasound scanning and technetium-99m sestamibi scintigraphy. Using bilateral neck exploration, subtotal, and parathyroidectomy are proposed. Our case consists of a 54 years old female, diagnosed with right inferior parathyroid adenoma. With three first degree relatives operated for neuroendocrine pancreatic tumors, her medical history includes distal corporeal-caudal pancreatectomy for a neuroendocrine tumor four years ago. Laboratory tests unveil increased calcium (12, 41 mg/dl) and glucose (150 mg/dl) levels. During hospitalization, the patient’s representative parameters for monitoring hyperparathyroidism maintained at high levels. Parathyroid ultrasound and the 99mTctetrofosmin/99mTc-pertechnetate parathyroid scintigraphy sustained the diagnosis. Consequently, the patient was proposed for a subtotal parathyroidectomy with a pathology examination. It is highly recommended the interdisciplinary cooperation for the monitoring and management of patients with suspicion of MEN 1. The annual assessment of serum calcium level, together with the parathyroid hormones, is proposed as a screening tool in early diagnosis.

Keywords

• men syndrome
• parathyroid adenomas
• corporeal-caudal pancreatectomy