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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. Britvin
Moscow Regional Research and Clinical Institute (MONIKI)
Russian 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
Moscow Regional Research and Clinical Institute (MONIKI)
Russian Federation

Bondarenko Ekaterina V. - Research Fellow of the Department of Morphological Diagnostics, Department of Oncology.

Moscow


Competing Interests: not


A. V. Krivosheev
Moscow Regional Research and Clinical Institute (MONIKI)
Russian Federation

Krivosheev Aleksey V. - Junior Research Fellow of the Department of Surgical Endocrinology.

Moscow


Competing Interests: not


O. A. Nechaeva
Moscow Regional Research and Clinical Institute (MONIKI)
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
Moscow Regional Research and Clinical Institute (MONIKI)
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
Moscow Regional Research and Clinical Institute (MONIKI)
Russian Federation

Podrez Dmitry V. - Junior Research Fellow of the Radiology Department.

Moscow


Competing Interests: not


L. E. Gurevich
Moscow Regional Research and Clinical Institute (MONIKI)
Russian Federation

Gurevich Larisa E. - Dr. of Sci. (Biol.), Professor, Leading Research Fellow of the Department of Morphological Diagnostics, Department of Oncology.

Moscow

Competing Interests: not


P. A. Korosteleva
Moscow Regional Research and Clinical Institute (MONIKI)
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

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