Authors
Grickevich A.A.1, Bajtman T.P.1, Chevina A.A.1, Borukaev A.Yu.1, Karelskaya N.A.1, Oganyan V.A.1, Grickevich E.Yu.2, Petrosyan K.A.2, Zhivotov V.A.3, Poddubnyj E.I.3
1 National Medical Research Center for Surgery named after A. Vishnevsky, Moscow
2 Russian National Research Medical University named after N.I. Pirogov, Moscow
3 Pirogov National Medical and Surgical Center, Moscow
Abstract
A clinical case of a 30-year-old patient with type 2A multiple endocrine neoplasia syndrome (MEN 2A) manifesting with arterial hypertension is considered. At the beginning of the survey, blood pressure (BP), which had been periodically rising for 15 years, reached 230; 100 mm Hg. Computed tomography (MSCT) revealed a tumour of the right adrenal gland with a diameter of 92 mm, with cystic transformation, several tumours of the left adrenal gland, with a diameter of 6–20 mm. The results of laboratory studies confirmed the hormonal activity of the neoplasm. The mother and the sister of the patient’s mother have tumors of the thyroid gland. According to the ultrasound of the patient’s thyroid gland, echographic signs of multinodular goiter (EU-TIRADS 5). Fine-needle aspiration biopsy of a focal thyroid tumour revealed an increase in the level of calcitonin by more than 2000 pg/ml in a wash from a needle.
The right-sided adrenalectomy was performed in October 2021, after conservative preoperative preparation with a selective blocker of postsynaptic α1-adrenergic receptors doxazosin and stabilization of BP. The normalization of the BP was noted in the postoperative period, which made it possible to cancel the antihypertensive therapy. The postoperative period proceeded without any complications.
Thyroidectomy with central lymphadenectomy, total parathyroidectomy for medullary thyroid cancer, primary hyperparathyroidism was performed in April 2022, and dynamic monitoring of the left adrenal gland is continuing.
The clinical case demonstrates the importance of careful history taking, laboratory and instrumental examination of patients with adrenal masses. Timely and complete diagnosis of the MEN 2A syndrome allows to determine the tactics of treatment, to avoid typical peri- and intraoperative complications, and significantly improves the quality of life and prognosis of the patient.
Keywords: syndrome of multiple endocrine neoplasias, pheochromocytoma, medullary thyroid cancer, mutation of the RET proto-oncogene, clinical case.
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