problendoПроблемы ЭндокринологииProblems of Endocrinology0375-96602308-1430Endocrinology Research Centre10.14341/probl12758problendo-12758Research ArticleКлиническая эндокринологияClinical endocrinologyРедкий случай гормонально-активной гонадотропиномы, ассоциированной со вторичным эритроцитозом, у мужчины в пожилом возрастеA rare case of a functioning gonadotroph tumor accompanied by erythrocytosis in an elderly manhttps://orcid.org/0000-0002-9783-3599МамедоваЕ. О.MamedovaE. O.

Мамедова Елизавета Октаевна - кандидат медицинских наук, старший научный сотрудник отделения нейроэндокринологии и остеопатий.

117036, Москва, ­ул. Дм. Ульянова, д. 11.

eLibrary SPIN: 3904-6017

Elizaveta O. Mamedova - MD, PhD.

11 Dm. Ulyanova street, 117036 Moscow.

eLibrary SPIN: 3904-6017

lilybet@mail.ruhttps://orcid.org/0000-0001-6891-0009СеливановаЛ. С.SelivanovaL. S.

Селиванова Лилия Сергеевна - кандидат медицинских наук.

117036, Москва, ­ул. Дм. Ульянова, д. 11.

eLibrary SPIN: 5151-3675

Liliya S. Selivanova - MD, PhD.

11 Dm. Ulyanova street, 117036 Moscow.

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liselivanova89@yandex.ruhttps://orcid.org/0000-0002-1967-8626ПотаповаК. А.PotapovaK. A.

Потапова Кристина Александровна.

117036, Москва, ­ул. Дм. Ульянова, д. 11.

eLibrary SPIN: 4892-6792

Kristina A. Potapova - MD.

11 Dm. Ulyanova street, 117036 Moscow.

eLibrary SPIN: 4892-6792

kris.potockaya.95@mail.ruhttps://orcid.org/0000-0001-9065-7791БурякинаС. А.BuryakinaS. A.

Бурякина Светлана Алексеевна - кандидат медицинских наук.

117036, Москва, ­ул. Дм. Ульянова, д. 11.

eLibrary SPIN: 5675-0651

Svetlana A. Buryakina - MD, PhD.

11 Dm. Ulyanova street, 117036 Moscow.

eLibrary SPIN: 5675-0651

sburyakina@yandex.ruhttps://orcid.org/0000-0001-9718-6099АзизянВ. Н.AzizyanV. N.

Азизян Вилен Неронович - кандидат медицинских наук.

117036, Москва, ­ул. Дм. Ульянова, д. 11.

eLibrary SPIN: 7666-5950

Vilen N. Azizyan - MD, PhD.

11 Dm. Ulyanova street, 117036 Moscow.

eLibrary SPIN: 7666-5950

vazizyan@mail.ruhttps://orcid.org/0000-0002-9575-4520ГригорьевА. Ю.GrigorievA. Yu.

Григорьев Андрей Юрьевич .

117036, Москва, ­ул. Дм. Ульянова, д. 11.

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Andrey Yu. Grigoriev - MD, PhD.

11 Dm. Ulyanova street, 117036 Moscow.

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medway26@gmail.comhttps://orcid.org/0000-0002-6674-6441БелаяЖ. Е.BelayaZh. E.

Белая Жанна Евгеньевна - доктор медицинских наук.

117036, Москва, ­ул. Дм. Ульянова, д. 11.

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Zhanna E. Belaya - MD, PhD.

11 Dm. Ulyanova street, 117036 Moscow.

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jannabelaya@gmail.comНациональный медицинский исследовательский центр эндокринологииРоссияEndocrinology Research CentreRussian FederationНациональный медицинский исследовательский центр эндокринологииРоссияEndocrinology Research CenterRussian Federation2021130620216733744Copyright © Мамедова Е.О., Селиванова Л.С., Потапова К.А., Бурякина С.А., Азизян В.Н., Григорьев А.Ю., Белая Ж.Е., 20212021Мамедова Е.О., Селиванова Л.С., Потапова К.А., Бурякина С.А., Азизян В.Н., Григорьев А.Ю., Белая Ж.Е.Mamedova E.O., Selivanova L.S., Potapova K.A., Buryakina S.A., Azizyan V.N., Grigoriev A.Y., Belaya Z.E.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/12758

Гормонально-активные гонадотропиномы — это редкие опухоли гипофиза, секретирующие один или два гонадотропных гормона (фолликулостимулирующий гормон (ФСГ) и/или лютеинизирующий гормон (ЛГ)), которые обладают биологической активностью. В большинстве случаев гонадотропиномы являются «молчащими» и составляют более половины гормонально-неактивных аденом гипофиза. В статье представлено описание редкого клинического наблюдения: ЛГ/ФСГ-секретирующей макроаденомы гипофиза с развитием битемпоральной гемианопсии у 62-летнего мужчины. Пациенту была проведена трансназальная транссфеноидальная аденомэктомия, позволившая достичь ремиссии заболевания. Отличительной особенностью данного случая являлось наличие вторичного эритроцитоза, развившегося вследствие эндогенной гиперандрогении, что потребовало проведения процедур эксфузий крови с целью нормализации уровня гематокрита перед проведением оперативного вмешательства. Примечательно, что клинические признаки эритроцитоза у пациента были выявлены задолго до развития зрительных нарушений. Представленный клинический случай демонстрирует сложность ранней диагностики гормонально-активных гонадотропином.

Functioning gonadotroph adenomas are rare pituitary tumors secreting one or two gonadotropins (follicle-stimulating hormone (FSH) and/or luteinizing hormone (LH)), which are hormonally active. In the majority of cases, gonadotroph tumors are endocrinologically “silent” and make up more than a half of non-functioning pituitary adenomas. In this article we describe a rare clinical case of LH/FSH-secreting pituitary macroadenoma with bitemporal hemianopsia in a 62-year-old man. The patient underwent transnasal transsphenoidal adenomectomy, leading to remission. The distinctive feature of this case is the presence of secondary erythrocytosis due to endogenous hyperandrogenism, which required several blood exfusions to normaliza the level of hematocrit before surgery. It is noteworthy that clinical signs of erythrocytosis were present long before visual impairment. This clinical case demonstrates difficulties in the early diagnosis of functioning gonadotroph adenomas.

аденома гипофизагонадотропиномаЛГФСГгиперандрогенияэритроцитозpituitary adenomagonadotroph tumorLHFSHhyperndrogenismerythrocytosisReferencesNtali G, Capatina C, Grossman A, Karavitaki N. Functioning gonadotroph adenomas. J Clin Endocrinol Metab. 2014;99(12):4423-4433. doi: https://doi.org/10.1210/jc.2014-2362Ntali G, Capatina C, Grossman A, Karavitaki N. Functioning gonadotroph adenomas. J Clin Endocrinol Metab. 2014;99(12):4423-4433. doi: https://doi.org/10.1210/jc.2014-2362Cote DJ, Smith TR, Sandler CN, et al. Functional gonadotroph adenomas: case series and report of literature. Neurosurgery. 2016;79(6):823-831. doi: https://doi.org/10.1227/NEU.0000000000001188Cote DJ, Smith TR, Sandler CN, et al. Functional gonadotroph adenomas: case series and report of literature. Neurosurgery. 2016;79(6):823-831. doi: https://doi.org/10.1227/NEU.0000000000001188Chamoun R, Layfield L, Couldwell WT. Gonadotroph adenoma with secondary hypersecretion of testosterone. World Neurosurg. 2013;80(6):900.e7-11. doi: https://doi.org/10.1016/j.wneu.2012.11.069Chamoun R, Layfield L, Couldwell WT. Gonadotroph adenoma with secondary hypersecretion of testosterone. World Neurosurg. 2013;80(6):900.e7-11. doi: https://doi.org/10.1016/j.wneu.2012.11.069Snyder PJ. Gonadotroph cell adenomas of the pituitary. Endocr Rev. 1985;6:552-563. doi: https://doi.org/10.1210/edrv-6-4-552Snyder PJ. Gonadotroph cell adenomas of the pituitary. Endocr Rev. 1985;6:552-563. doi: https://doi.org/10.1210/edrv-6-4-552Yamada S, Ohyama K, Taguchi M, et al. A study of the correlation between morphological findings and biological activities in clinically nonfunctioning pituitary adenomas. Neurosurgery. 2007;61:580-584. doi: https://doi.org/10.1227/01.NEU.0000290906.53685.79Yamada S, Ohyama K, Taguchi M, et al. A study of the correlation between morphological findings and biological activities in clinically nonfunctioning pituitary adenomas. Neurosurgery. 2007;61:580-584. doi: https://doi.org/10.1227/01.NEU.0000290906.53685.79Gryngarten MG, Braslavsky D, Ballerini MG, et al. Spontaneous Ovarian Hyperstimulation Syndrome Caused by a Follicle-Stimulating Hormone-Secreting Pituitary Macroadenoma in an Early Pubertal Girl. Horm Res Paediatr. 2010;73(4):293-298. doi: https://doi.org/10.1159/000284395Gryngarten MG, Braslavsky D, Ballerini MG, et al. Spontaneous Ovarian Hyperstimulation Syndrome Caused by a Follicle-Stimulating Hormone-Secreting Pituitary Macroadenoma in an Early Pubertal Girl. Horm Res Paediatr. 2010;73(4):293-298. doi: https://doi.org/10.1159/000284395Ambrosi B, Bassetti M, Ferrario R, et al. Precocious puberty in a boy with a PRL-, LH- and FSH-secreting pituitary tumour: hormonal and immunocytochemical studies. Acta Endocrinol (Copenh). 1990;122(5):569-576. doi: https://doi.org/10.1530/acta.0.1220569Ambrosi B, Bassetti M, Ferrario R, et al. Precocious puberty in a boy with a PRL-, LH- and FSH-secreting pituitary tumour: hormonal and immunocytochemical studies. Acta Endocrinol (Copenh). 1990;122(5):569-576. doi: https://doi.org/10.1530/acta.0.1220569Clemente M, Caracseghi F, Gussinyer M, et al. Macroorchidism and panhypopituitarism: two different forms of presentation of FSH-secreting pituitary adenomas in adolescence. Horm Res Paediatr. 2011;75(3):225-230. doi: https://doi.org/10.1159/000322211Clemente M, Caracseghi F, Gussinyer M, et al. Macroorchidism and panhypopituitarism: two different forms of presentation of FSH-secreting pituitary adenomas in adolescence. Horm Res Paediatr. 2011;75(3):225-230. doi: https://doi.org/10.1159/000322211Zhao Y, Lian W, Xing B, et al. Functioning gonadotroph adenoma. Chin Med J (Engl). 2019;132(8):1003-1004. doi: https://doi.org/10.1097/CM9.0000000000000184Zhao Y, Lian W, Xing B, et al. Functioning gonadotroph adenoma. Chin Med J (Engl). 2019;132(8):1003-1004. doi: https://doi.org/10.1097/CM9.0000000000000184Cooper O, Geller JL, Melmed S. Ovarian hyperstimulation syndrome caused by an FSH-secreting pituitary adenoma. Nat Clin Pract Endocrinol Metab. 2008;4(4):234-238. doi: https://doi.org/10.1038/ncpendmet0758Cooper O, Geller JL, Melmed S. Ovarian hyperstimulation syndrome caused by an FSH-secreting pituitary adenoma. Nat Clin Pract Endocrinol Metab. 2008;4(4):234-238. doi: https://doi.org/10.1038/ncpendmet0758Halupczok J, Bidzińska-Speichert B, Lenarcik-Kabza A, et al. Gonadotroph adenoma causing ovarian hyperstimulation syndrome in a premenopausal woman. Gynecol Endocrinol. 2014;30(11):774-777. doi: https://doi.org/10.3109/09513590.2014.934668Halupczok J, Bidzińska-Speichert B, Lenarcik-Kabza A, et al. Gonadotroph adenoma causing ovarian hyperstimulation syndrome in a premenopausal woman. Gynecol Endocrinol. 2014;30(11):774-777. doi: https://doi.org/10.3109/09513590.2014.934668Hirano M, Wada-Hiraike O, Miyamamoto Y, et al. A case of functioning gonadotroph adenoma in a reproductive aged woman. Endocr J. 2019;66(7):653-656. doi: https://doi.org/10.1507/endocrj.EJ19-0066Hirano M, Wada-Hiraike O, Miyamamoto Y, et al. A case of functioning gonadotroph adenoma in a reproductive aged woman. Endocr J. 2019;66(7):653-656. doi: https://doi.org/10.1507/endocrj.EJ19-0066Broughton C, Mears J, Williams A, Lonnen K. A clinically functioning gonadotroph adenoma presenting with abdominal pain, ovarian hyperstimulation and fibromatosis. Endocrinol Diabetes Metab Case Reports. 2018;2018. doi: https://doi.org/10.1530/EDM-18-0123Broughton C, Mears J, Williams A, Lonnen K. A clinically functioning gonadotroph adenoma presenting with abdominal pain, ovarian hyperstimulation and fibromatosis. Endocrinol Diabetes Metab Case Reports. 2018;2018. doi: https://doi.org/10.1530/EDM-18-0123Graillon T, Castinetti F, Chabert-Orsini V, et al. Functioning gonadotroph adenoma with severe ovarian hyperstimulation syndrome: A new emergency in pituitary adenoma surgery? Surgical considerations and literature review. Ann Endocrinol (Paris). 2019;80(2):122-127. doi: https://doi.org/10.1016/j.ando.2018.11.007Graillon T, Castinetti F, Chabert-Orsini V, et al. Functioning gonadotroph adenoma with severe ovarian hyperstimulation syndrome: A new emergency in pituitary adenoma surgery? Surgical considerations and literature review. Ann Endocrinol (Paris). 2019;80(2):122-127. doi: https://doi.org/10.1016/j.ando.2018.11.007Горбачева А.М., Пржиялковская Е.Г., Азизян В.Н., и др. Клинически активная гонадотропинома c развитием синдрома гиперстимуляции яичников у молодой женщины // Проблемы эндокринологии. — 2019. — Т. 65. — №4. — С. 278-288. doi: https://doi.org/10.14341/probl10178Gorbacheva AM, Przhiyalkovskaya EG, Azizyan VN, et al. An ovarian hyperstimulation syndrome caused by gonadotropinoma in a young woman. Problems of Endocrinology. 2019;65(4):278-288. (In Russ.). doi: https://doi.org/10.14341/probl10178Dizon MN, Vesely DL. Gonadotropin-secreting pituitary tumor associated with hypersecretion of testosterone and hypogonadism after hypophysectomy. Endocr Pract. 2002;8(3):225-231. doi: https://doi.org/10.4158/EP.8.3.225Dizon MN, Vesely DL. Gonadotropin-secreting pituitary tumor associated with hypersecretion of testosterone and hypogonadism after hypophysectomy. Endocr Pract. 2002;8(3):225-231. doi: https://doi.org/10.4158/EP.8.3.225Ceccato F, Occhi G, Regazzo D, et al. Gonadotropin secreting pituitary adenoma associated with erythrocytosis: case report and literature review. Hormones (Athens). 2014;13(1):131-139. doi: https://doi.org/10.1007/BF03401328Ceccato F, Occhi G, Regazzo D, et al. Gonadotropin secreting pituitary adenoma associated with erythrocytosis: case report and literature review. Hormones (Athens). 2014;13(1):131-139. doi: https://doi.org/10.1007/BF03401328Heseltine D, White MC, Kendall-Taylor P, et al. Testicular enlargement and elevated serum inhibin concentrations occur in patients with pituitary macroadenomas secreting follicle stimulating hormone. Clin Endocrinol (Oxf). 1989;31:411-423. doi: https://doi.org/10.1111/j.1365-2265.1989.tb01265.xHeseltine D, White MC, Kendall-Taylor P, et al. Testicular enlargement and elevated serum inhibin concentrations occur in patients with pituitary macroadenomas secreting follicle stimulating hormone. Clin Endocrinol (Oxf). 1989;31:411-423. doi: https://doi.org/10.1111/j.1365-2265.1989.tb01265.xPigny P, Henric B, Lahlou N, et al. A gonadotroph adenoma with a high proportion of basic FSH isohormones by chromatofocusing. J Clin Endocrinol Metab. 1996;81:2407-2408. doi: https://doi.org/10.1210/jcem.81.6.8964889Pigny P, Henric B, Lahlou N, et al. A gonadotroph adenoma with a high proportion of basic FSH isohormones by chromatofocusing. J Clin Endocrinol Metab. 1996;81:2407-2408. doi: https://doi.org/10.1210/jcem.81.6.8964889Dahlqvist P, Koskinen LO, Brännström T, Hägg E. Testicular enlargement in a patient with a FSH-secreting pituitary adenoma. Endocrine. 2010;37:289-293. doi: https://doi.org/10.1007/s12020-009-9302-zDahlqvist P, Koskinen LO, Brännström T, Hägg E. Testicular enlargement in a patient with a FSH-secreting pituitary adenoma. Endocrine. 2010;37:289-293. doi: https://doi.org/10.1007/s12020-009-9302-zSnyder PJ, Sterling FH. Hypersecretion of LH and FSH by a pituitary adenoma. J Clin Endocrinol Metab. 1976;42:544-550. doi: https://doi.org/10.1210/jcem-42-3-544Snyder PJ, Sterling FH. Hypersecretion of LH and FSH by a pituitary adenoma. J Clin Endocrinol Metab. 1976;42:544-550. doi: https://doi.org/10.1210/jcem-42-3-544Zárate A, Fonseca ME, Mason M, et al. Gonadotropin-secreting pituitary adenoma with concomitant hypersecretion of testosterone and elevated sperm count. Treatment with LRH agonist. Acta Endocrinol (Copenh). 1986;113:29-34. doi: https://doi.org/10.1530/acta.0.1130029Zárate A, Fonseca ME, Mason M, et al. Gonadotropin-secreting pituitary adenoma with concomitant hypersecretion of testosterone and elevated sperm count. Treatment with LRH agonist. Acta Endocrinol (Copenh). 1986;113:29-34. doi: https://doi.org/10.1530/acta.0.1130029Whitaker MD, Prior JC, Scheithauer B, et al. Gonadotrophin-secreting pituitary tumour: report and review. Clin Endocrinol (Oxf). 1985;22:43-48. doi: https://doi.org/10.1111/j.1365-2265.1985.tb01063.xWhitaker MD, Prior JC, Scheithauer B, et al. Gonadotrophin-secreting pituitary tumour: report and review. Clin Endocrinol (Oxf). 1985;22:43-48. doi: https://doi.org/10.1111/j.1365-2265.1985.tb01063.xThakkar A, Kannan S, Hamrahian A, et al. Testicular “Hyperstimulation” Syndrome: A Case of Functional Gonadotropinoma. Case Rep Endocrinol. 2014;2014:1-4. doi: https://doi.org/10.1155/2014/194716Thakkar A, Kannan S, Hamrahian A, et al. Testicular “Hyperstimulation” Syndrome: A Case of Functional Gonadotropinoma. Case Rep Endocrinol. 2014;2014:1-4. doi: https://doi.org/10.1155/2014/194716Roman SH, Goldstein M, Kourides IA, et al. Luteinizing hormone-releasing hormone (LHRH) agonist [D-Trp6-Pro9-NEt]LHRH increased rather than lowered LH and alpha-subunit levels in a patient with an LH-secreting pituitary tumor. J Clin Endocrinol Metab. 1984;58(2):313-319. doi: https://doi.org/10.1210/jcem-58-2-313Roman SH, Goldstein M, Kourides IA, et al. Luteinizing hormone-releasing hormone (LHRH) agonist [D-Trp6-Pro9-NEt]LHRH increased rather than lowered LH and alpha-subunit levels in a patient with an LH-secreting pituitary tumor. J Clin Endocrinol Metab. 1984;58(2):313-319. doi: https://doi.org/10.1210/jcem-58-2-313Peterson RE, Kourides IA, Horwith M, et al. Luteinizing hormone- and alpha-subunit-secreting pituitary tumor: positive feedback of estrogen. J Clin Endocrinol Metab. 1981;52(4):692-698. doi: https://doi.org/10.1210/jcem-52-4-692Peterson RE, Kourides IA, Horwith M, et al. Luteinizing hormone- and alpha-subunit-secreting pituitary tumor: positive feedback of estrogen. J Clin Endocrinol Metab. 1981;52(4):692-698. doi: https://doi.org/10.1210/jcem-52-4-692McMullin MF, Bareford D, Campbell P, et al. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br J Haematol. 2005;130(2):174-195. doi: https://doi.org/10.1111/j.1365-2141.2005.05535.xMcMullin MF, Bareford D, Campbell P, et al. Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis. Br J Haematol. 2005;130(2):174-195. doi: https://doi.org/10.1111/j.1365-2141.2005.05535.xMithoowani S, Laureano M, Crowther MA, Hillis CM. Investigation and management of erythrocytosis. CMAJ. 2020;192:913-918. doi: https://doi.org/10.1503/cmaj.191587Mithoowani S, Laureano M, Crowther MA, Hillis CM. Investigation and management of erythrocytosis. CMAJ. 2020;192:913-918. doi: https://doi.org/10.1503/cmaj.191587

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