[Pheochromocytoma associated with primary hyperparathyroidism and type 1 neurofibromatosis]. / Feokhromotsitoma v sochetanii s pervichnym giperparatireozom i neirofibromatozom tipa 1.

Neurofibromatosis type 1 (NF-1) is a predominantly inherited genetic disease with prevalence of 1 per 2500-3000 newborns and based on clear clinical criteria. In addition to common neurofibromas and gliomas of the visual pathways, these patients have a higher risk of various benign and malignant tumors throughout their lives including tumors of the central nervous system, membranes of peripheral nerves, gastrointestinal stromal tumors and leukemia. Endocrine diseases and neoplasms also occur in patients with NF-1 and can include extrarenal paraganglioma, primary hyperparathyroidism, gastroenteropancreatic neuroendocrine tumors, thyroid tumors and other adrenal neoplasms. We demonstrate multiple neuroendocrine neoplasia (MEN 2A) of neurofibromatosis type 1 combined with pheochromocytoma and primary hyperparathyroidism in a woman with a long history of palpitations, paroxysmal hypertension and osteoporosis. Biochemical analysis revealed severe hypercalcemia with elevated parathyroid hormone indicating primary hyperparathyroidism, as well as high fractionated normetanephrine and metanephrine in the urine as a sign of catecholamine-releasing pheochromocytoma/paraganglioma. Further scintigraphy revealed signs of solitary parathyroid adenoma causing primary hyperparathyroidism and right-sided pheochromocytoma. Clinical diagnosis of MEN-2 syndrome is based on combination of at least two major MEN-2-associated endocrine tumors. Resection of parathyroid adenoma and pheochromocytoma normalized biochemical parameters and blood pressure. Combination of pheochromocytoma with primary hyperparathyroidism and type 1 neurofibromatosis is discussed.

[Oncological alertness in Graves disease]. / Onkologicheskaya nastorozhennost' pri bolezni Greivsa.

OBJECTIVE: To assess the risk of thyroid cancer in patients with Graves' disease (GD) after surgical treatment. MATERIAL AND METHODS: We retrospectively evaluated 121 patients with GD after thyroidectomy between December 2015 and January 2020. Thyroid cancer was confirmed by morphological analysis. Thyroid cancer occurred in 34 (28.1%) patients with GD after thyroidectomy. Preoperative ultrasound examination revealed nodular goiter in 62 (51.2%) patients. Nodular lesions were not found in other 59 (48.8%) patients with GD. RESULTS: Incidence of thyroid cancer was significantly higher in patients with nodular lesions (38% vs. 16%; p=0.009). Papillary thyroid cancer was detected in 32 out of 34 cases, follicular thyroid cancer - in 2 patients. Among 32 patients with papillary thyroid cancer, 28 ones had classical type, 2 patients - follicular variant of papillary cancer, 1 patient - oncocytic cancer, 1 patient - columnar cell variant of PTC. CONCLUSION: Risk of cancer is higher in patients with GD and nodes. In addition to standard examination of patients with GD, we performed ultrasound with examination of regional lymph nodes and analysis of further surgical tactics.