Surgical treatment of a patient with multiple endocrine neoplasia type 2A
https://doi.org/10.24884/0042-4625-2020-179-4-79-86
Abstract
The diagnosis and treatment of multiple endocrine neoplasia type 2A syndrome or Sipple’s syndrome, as well as other endocrine hereditary syndromes, presents certain difficulties for several reasons. One of them is the rarity of this pathology and, as a result, the lack of awareness of physicians. In addition, the diagnosis requires a comprehensive examination and a high level of clinical, laboratory, radiation and morphological diagnostics. And finally, surgical treatment, which is essentially the only method, involves an individual approach to each patient. We present the clinical case of Sipple's syndrome in a 39-year-old man. The diagnosis was carried out of medical history, clinical examination, laboratory tests (24-hour urine excretion of metanephrines and normetanephrines; serum calcium, calcitonin and parathyroid hormone levels), neck ultrasound and abdominal CT scan and was confirmed by histopathological examination and genetic testing for RET mutation. The achievement of the endpoint in the form of normalization of blood pressure and the absence of adrenal insufficiency was due to radical surgical treatment.
About the Authors
T. A. BritvinRussian Federation
Britvin Timur A. - Dr. of Sci. (Med.), Head of the Department of Surgery.
61/2, Schepkina str., Moscow, 129110
Competing Interests: not
E. V. Bondarenko
Russian Federation
Bondarenko Ekaterina V. - Research Fellow of the Department of Morphological Diagnostics, Department of Oncology.
Moscow
Competing Interests: not
A. V. Krivosheev
Russian Federation
Krivosheev Aleksey V. - Junior Research Fellow of the Department of Surgical Endocrinology.
Moscow
Competing Interests: not
O. A. Nechaeva
Russian Federation
Nechaeva Olga A. - Cand. of Sci. (Med.), Senior Research Fellow of the Department of Therapeutic Endocrinology.
Moscow
Competing Interests: not
T. S. Tamazyan
Russian Federation
Tamazyan Tatyana S. - Assistant of the Department of Radiology Diagnostics of the Faculty of Continuing Medical Education.
Moscow
Competing Interests: not
D. V. Podrez
Russian Federation
Podrez Dmitry V. - Junior Research Fellow of the Radiology Department.
Moscow
Competing Interests: not
L. E. Gurevich
Russian Federation
Gurevich Larisa E. - Dr. of Sci. (Biol.), Professor, Leading Research Fellow of the Department of Morphological Diagnostics, Department of Oncology.
MoscowCompeting Interests: not
P. A. Korosteleva
Russian Federation
Korosteleva Polina A. – Pathologist.
Moscow
Competing Interests: not
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Review
For citations:
Britvin T.A., Bondarenko E.V., Krivosheev A.V., Nechaeva O.A., Tamazyan T.S., Podrez D.V., Gurevich L.E., Korosteleva P.A. Surgical treatment of a patient with multiple endocrine neoplasia type 2A. Grekov's Bulletin of Surgery. 2020;179(4):79-86. (In Russ.) https://doi.org/10.24884/0042-4625-2020-179-4-79-86