Surgical treatment of a patient with multiple endocrine neoplasia type 2A

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...

Full description

Saved in:
Bibliographic Details
Main Authors: T. A. Britvin, E. V. Bondarenko, A. V. Krivosheev, O. A. Nechaeva, T. S. Tamazyan, D. V. Podrez, L. E. Gurevich, P. A. Korosteleva
Format: Article
Language:Russian
Published: Pavlov First Saint Petersburg State Medical University 2020-11-01
Series:Вестник хирургии имени И.И. Грекова
Subjects:
Online Access:https://www.vestnik-grekova.ru/jour/article/view/1522
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849771860587184128
author T. A. Britvin
E. V. Bondarenko
A. V. Krivosheev
O. A. Nechaeva
T. S. Tamazyan
D. V. Podrez
L. E. Gurevich
P. A. Korosteleva
author_facet T. A. Britvin
E. V. Bondarenko
A. V. Krivosheev
O. A. Nechaeva
T. S. Tamazyan
D. V. Podrez
L. E. Gurevich
P. A. Korosteleva
author_sort T. A. Britvin
collection DOAJ
description 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.
format Article
id doaj-art-06b1fa37618c4e5da5c1fe2fcfb04ba4
institution DOAJ
issn 0042-4625
language Russian
publishDate 2020-11-01
publisher Pavlov First Saint Petersburg State Medical University
record_format Article
series Вестник хирургии имени И.И. Грекова
spelling doaj-art-06b1fa37618c4e5da5c1fe2fcfb04ba42025-08-20T03:02:29ZrusPavlov First Saint Petersburg State Medical UniversityВестник хирургии имени И.И. Грекова0042-46252020-11-011794798610.24884/0042-4625-2020-179-4-79-861163Surgical treatment of a patient with multiple endocrine neoplasia type 2AT. A. Britvin0E. V. Bondarenko1A. V. Krivosheev2O. A. Nechaeva3T. S. Tamazyan4D. V. Podrez5L. E. Gurevich6P. A. Korosteleva7Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)Moscow Regional Research and Clinical Institute (MONIKI)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.https://www.vestnik-grekova.ru/jour/article/view/1522sipple’s syndrome, medullary thyroid carcinoma, pheochromocytoma, ret-gene, surgical treatment
spellingShingle T. A. Britvin
E. V. Bondarenko
A. V. Krivosheev
O. A. Nechaeva
T. S. Tamazyan
D. V. Podrez
L. E. Gurevich
P. A. Korosteleva
Surgical treatment of a patient with multiple endocrine neoplasia type 2A
Вестник хирургии имени И.И. Грекова
sipple’s syndrome, medullary thyroid carcinoma, pheochromocytoma, ret-gene, surgical treatment
title Surgical treatment of a patient with multiple endocrine neoplasia type 2A
title_full Surgical treatment of a patient with multiple endocrine neoplasia type 2A
title_fullStr Surgical treatment of a patient with multiple endocrine neoplasia type 2A
title_full_unstemmed Surgical treatment of a patient with multiple endocrine neoplasia type 2A
title_short Surgical treatment of a patient with multiple endocrine neoplasia type 2A
title_sort surgical treatment of a patient with multiple endocrine neoplasia type 2a
topic sipple’s syndrome, medullary thyroid carcinoma, pheochromocytoma, ret-gene, surgical treatment
url https://www.vestnik-grekova.ru/jour/article/view/1522
work_keys_str_mv AT tabritvin surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT evbondarenko surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT avkrivosheev surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT oanechaeva surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT tstamazyan surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT dvpodrez surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT legurevich surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a
AT pakorosteleva surgicaltreatmentofapatientwithmultipleendocrineneoplasiatype2a