National clinical recomendations
Russian clinical practice guidelines are dedicated to the management of patients with pheochromocytoma and paraganglioma.
Clinical practice
Clinical Case
Syndrome Sipple is the second name of the syndrome of multiple endocrine neoplasia type II (MEN2A). In 1961 J.H. Sipple described several relatives with medullary thyroid cancer, feohromotsytoma and hyperparathyroidism. The following observation is presented.
Given the unique observation of atypical MEN1 (mixed version) of a man 48 years old, due to the presence 9 of multiple tumors and hyperplasia in 8 different organs (parathyroid glands, pancreatic and thyroid glands, duodenum, lung, stomach, skin and adrenal glands). The peculiarity of this case is medullary carcinoma of the thyroid gland that is usually associated with the syndrome of MEN2. The patient had typical clinical hypoglycemic syndrome, however, among all the investigated neuroendocrine tumors of the insulinproducing cells were not available, that doesn’t mean there are not revealed (possibly very small) tumors of the pancreas. At the same time, it is known that clinical manifestations of MEN1 may be due to the cumulative effect of all complex of the hormones produced simultaneously by several multiple tumors.

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