problendoПроблемы ЭндокринологииProblems of Endocrinology0375-96602308-1430Endocrinology Research Centre10.14341/probl201359319-26problendo-6476Research ArticleСтатьиArticlesФеохромоцитома/параганглиома: клинико-генетические аспектыPheochromocytoma/paraganglioma: clinical and genetic aspectsIukinaM Iukuronova@yandex.ruTroshinaE A-Bel'tsevichD G-Tiul'pakovA N-LysenkoM A-201315062013593ТОМ 59, №3 (2013)1926Copyright © Iukina M.I., Troshina E.A., Bel'tsevich D.G., Tiul'pakov A.N., Lysenko M.A., 20132013Iukina M.I., Troshina E.A., Bel'tsevich D.G., Tiul'pakov A.N., Lysenko M.A.Iukina M.I., Troshina E.A., Bel'tsevich D.G., Tiul'pakov A.N., Lysenko M.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.probl-endojournals.ru/jour/article/view/6476

Проведено ретроспективное описательно-аналитическое исследование 167 пациентов, разделенных на три группы: две группы пациентов с генетически-детерминированной феохромоцитомой (1-я - синдром множественной эндокринной нео­плазии - МЭН 2-го типа и 2-я - болезнь фон Гиппеля-Линдау) и 3-я группа со спорадической феохромоцитомой. Медиана возраста выявления феохромоцитомы в 1-й группе составила 25 лет, во 2-й группе - 18 лет, в 3-й группе - 47 лет. Для пациентов 1-й группы характерен преимущественный адреналиновый тип секреции, для 2-й - норадреналиновый, для 3-й группы - смешанный. В 1-й группе выявлена положительная корреляция между размером опухоли и экскрецией метилированных катехоламинов; у 82% пациентов имелось двустороннее поражение надпочечников и у 58% - мультифокальное поражение. Во всех случаях опухоль локализовалась вне надпочечников. Во 2-й группе вненадпочечниковая локализация выявлена у 16,4% пациентов, у 6,6% выявлены метастазы феохромоцитомы. В 1-й и в 3-й группах метастазов феохромоцитомы не выявлено. Пациентам моложе 40 лет, пациентам с двусторонней и мультицентрической феохромоцитомой, с отягощенным семейным анамнезом необходимо исключение генетической причины заболевания с учетом синдромальных проявлений. При изолированной экскреции норметанефрина обследование в отношении синдрома МЭН 2-го типа нецелесообразно. При синдроме МЭН 2-го типа и/или при изолированной экскреции метанефрина поиск метастазов и вненадпочечниковой локализации феохромоцитомы нецелесообразен.

The present retrospective descriptive and analytical study included 167 patients divided into three groups. Two groups were comprised of the patients with genetically predetermined pheochromocytoma (type 2 MEN syndrome and von Hippel-Lindau disease respectively), the third croup consisted of the patients presenting with sporadic pheochromocytoma. The median age at which pheochromocytoma was diagnosed was 25 years in group 1, 18 years in group 2, and 47 years in group 3. Adrenalin-type secretion was predominated in group 1 in contrast to the noradrenalin-type and mixed-type secretion in the patients of groups 2 and 3 respectively. The patients of group 1 showed positive correlation between the tumour size and excretion of methylated catecholamines. 82% of these patients exhibited bilateral adrenal lesions and 58% suffered multifocal lesions; no cases of extra-adrenal localization of the tumour were documented. The extra-adrenal localization of pheochromocytomas was observed in 16.4% of the patients in group 2, where 6.6% of the patients had metastases. No metastases of pheochromocytoma were found in the patients of groups 1 and 3. It is concluded that the patients under the age of 40 presenting with bilateral and multicentral pheochromocytoma with the aggravated familial history need the genetic predetermination of the disease to be excluded taking into account syndromal symptoms. Examination for the detection of type 2 MEN is not required in the patients showing isolated normetanephrin excretion. Nor is the search for metastases of pheochromocytoma and its extra-adrenal localization is necessary in the patients presenting with type 2 MEN syndrome and/or exhibiting isolated metanephrin excretion.

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The authors declare that there are no conflicts of interest present.