A rare case of a functioning gonadotroph tumor accompanied by erythrocytosis in an elderly man
https://doi.org/10.14341/probl12758
Abstract
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.
About the Authors
E. O. MamedovaRussian Federation
Elizaveta O. Mamedova - MD, PhD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 3904-6017
Competing Interests:
No
L. S. Selivanova
Russian Federation
Liliya S. Selivanova - MD, PhD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 5151-3675
Competing Interests:
No
K. A. Potapova
Russian Federation
Kristina A. Potapova - MD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 4892-6792
Competing Interests:
No
S. A. Buryakina
Russian Federation
Svetlana A. Buryakina - MD, PhD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 5675-0651
Competing Interests:
No
V. N. Azizyan
Russian Federation
Vilen N. Azizyan - MD, PhD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 7666-5950
Competing Interests:
No
A. Yu. Grigoriev
Russian Federation
Andrey Yu. Grigoriev - MD, PhD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 8910-8130
Competing Interests:
No
Zh. E. Belaya
Russian Federation
Zhanna E. Belaya - MD, PhD.
11 Dm. Ulyanova street, 117036 Moscow.
eLibrary SPIN: 4746-7173
Competing Interests:
No
References
1. Ntali 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.2014-2362
2. Cote 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.0000000000001188
3. Chamoun 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.2012.11.069
4. Snyder PJ. Gonadotroph cell adenomas of the pituitary. Endocr Rev. 1985;6:552-563. doi: https://doi.org/10.1210/edrv-6-4-552
5. Yamada 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.0000290906.53685.79
6. Gryngarten 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/10.1159/000284395
7. Ambrosi 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.0.1220569
8. Clemente 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/10.1159/000322211
9. Zhao 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/CM9.0000000000000184
10. Cooper 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/ncpendmet0758
11. Halupczok 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/10.3109/09513590.2014.934668
12. Hirano 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.EJ19-0066
13. Broughton 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-18-0123
14. Graillon 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.2018.11.007
15. Gorbacheva 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/probl10178
16. Dizon 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.8.3.225
17. Ceccato 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/BF03401328
18. Heseltine 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.x
19. Pigny 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.81.6.8964889
20. Dahlqvist 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-z
21. Snyder 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-42-3-544
22. Zárate 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.0.1130029
23. Whitaker 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.x
24. Thakkar 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/10.1155/2014/194716
25. Roman 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-58-2-313
26. Peterson 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-52-4-692
27. McMullin 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.x
28. Mithoowani 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
Supplementary files
|
1. Figure 1. MRI of the brain of patient G. Pituitary macroadenoma with supra-, infra-, retro-, parasellar (D, S) spread (Knosp IV): a) T2-weighted image (weighted image), axial projection. Pituitary adenoma (arrow) b) T1-VI, coronal projection; c) T2-VI, coronal projection. Compression of the optic chiasm (arrow); d) T1-VI, sagittal projection. The spread of adenoma infrasellarly into the main sinus (thick arrow) and retrosellarly into the pontine cistern (thin arrow). | |
Subject | ||
Type | Данные | |
View
(536KB)
|
Indexing metadata ▾ |
|
2. Figure 2. Results of perimetry before surgery (A, top row) and 6 months after surgery (B, bottom row). OS - left eye, OD - right eye. | |
Subject | ||
Type | Данные | |
View
(102KB)
|
Indexing metadata ▾ |
|
3. Figure 3. Microscopic structure of the tumor. Staining with hematoxylin and eosin. Magnification x100. | |
Subject | ||
Type | Данные | |
View
(255KB)
|
Indexing metadata ▾ |
|
4. Figure 4. Expression of luteinizing hormone by tumor cells. Magnification x100. | |
Subject | ||
Type | Данные | |
View
(355KB)
|
Indexing metadata ▾ |
|
5. Figure 5. Expression of follicle-stimulating hormone by tumor cells. Magnification x100. | |
Subject | ||
Type | Данные | |
View
(387KB)
|
Indexing metadata ▾ |
|
6. Figure 6. Expression of SSTR2A by tumor cells. Magnification x100. | |
Subject | ||
Type | Данные | |
View
(256KB)
|
Indexing metadata ▾ |
|
7. Figure 7. Expression of SSTR5 by tumor cells. Magnification x100. | |
Subject | ||
Type | Данные | |
View
(328KB)
|
Indexing metadata ▾ |
|
8. Figure 8. Index of tumor proliferative activity (by Ki-67 expression). | |
Subject | ||
Type | Данные | |
View
(335KB)
|
Indexing metadata ▾ |
Review
For citations:
Mamedova E.O., Selivanova L.S., Potapova K.A., Buryakina S.A., Azizyan V.N., Grigoriev A.Yu., Belaya Zh.E. A rare case of a functioning gonadotroph tumor accompanied by erythrocytosis in an elderly man. Problems of Endocrinology. 2021;67(3):37-44. https://doi.org/10.14341/probl12758

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND 4.0).