Russian registry of hypothalamic and pituitary tumors: clinical and statistical analysis for 01.01.2023

BACKGROUND: Pituitary adenomas are the third most common intracranial neoplasm. Clinical significance is determined by the nature of growth and hormonal activity, leading to disruption of various parts of metabolism. Medical registries are a valuable tool for assessing clinical and epidemiological parameters and obtaining data on real-life clinical practice.

AIM: To analyze the epidemiological and clinical characteristics of four pituitary disorders in Russian Hypothalamic and Pituitary Tumor Registry (OGGO): acromegaly, Cushing’s disease, prolactin-secreting and non-functioning pituitary adenomas

MATERIALS AND METHODS: The object of the study is the database of the OGGO registry, which includes 84 regions of the Russian Federation, as of 01/01/2023.

RESULTS: The maximum prevalence of acromegaly in the Russian Federation was noted in Karelia (9.48/100 thousand) and Chuvash Republic (10.8/100 thousand) and in Tyumen region (8.9/100 thousand). The incidence of acromegaly in 2021 was 0.6/1 million population. The maximum prevalence of Cushing’s disease is observed in Karelia Republic (2.33/100 thousand), Chukotka (2.01/100 thousand), Kirov (1.79/100 thousand) and Tver region ( 1.79/100 thousand). The incidence of Cushing’s disease in 2021 was 0.1/1 million population. The maximum prevalence of prolactinomas is observed Karelia (13.33/100 thousand) and Chuvash Republic (12.35/100 thousand) and in Voronezh region (8.66/100 thousand). The incidence of prolactin-secreting adenomas in 2021 was 0.6/1 million population. The maximum prevalence of non-functioning pituitary adenomas is observed in the Karelia (15.08/100 thousand), Voronezh region (8.82/100 thousand), Tyumen region (8.11/100 thousand) and Sakha/Yakutia Republic (7.56 /100 thousand). The incidence of non-functioning pituitary adenomas in 2021 was 0.3/1 million population. The median age for acromegaly was 63.6 [53.2;71.5], for Cushing’s disease 49.2 [38.8;60.8], for prolactin-secreting adenomas 47.5 [38.3;59.7], for patients with non-functioning pituitary adenomas — 58.4 [43.8;67.9] . At the time of the last visit, remission was observed in 42.7% of patients with acromegaly, 62.5% with Cushing’s disease, 39.7% with prolactinomas and 76.6% of patients with non-active adenomas.

CONCLUSION: The OGGO registry is a valuable tool for assessing epidemiological, clinical data and treatments used. At the same time, the quality of the analysis directly depends on the quality and completeness of the entered data. Further research in the field of epidemiology of endocrinopathies should be aimed at improving the quality and ease of data entry, which will allow obtaining the most complete patient characterization.

1. Ostrom QT, Gittleman H, Farah P, et al. CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2006-2010. Neuro Oncol. 2013;15(suppl 2):ii1-ii56. doi:10.1093/neuonc/not151

2. McDowell BD, Wallace RB, Carnahan RM, Chrischilles EA, Lynch CF, Schlechte JA. Demographic differences in incidence for pituitary adenoma. Pituitary. 2011;14(1):23-30. doi:10.1007/s11102-010-0253-4

3. Daly AF, Beckers A. The Epidemiology of Pituitary Adenomas. Endocrinol Metab Clin North Am. 2020;49(3):347-355. doi:10.1016/j.ecl.2020.04.002

4. Gliklich RE, Dreyer NA, Leavy MB, editors. Registries for Evaluating Patient Outcomes: A User’s Guide [Internet]. 3rd edition. Rockville (MD): Agency for Healthcare Research and Quality (US); 2014 Apr. 20, Rare Disease Registries. Available from: https://.

5. Maione L, Chanson P. National acromegaly registries. Best Pract Res Clin Endocrinol Metab. 2019;33(2):101264. doi:10.1016/j.beem.2019.02.001

6. Valassi E, Tabarin A, Brue T, et al. High mortality within 90 days of diagnosis in patients with Cushing’s syndrome: results from the ERCUSYN registry. Eur J Endocrinol. 2019;181(5):461-472. doi:10.1530/EJE-19-0464

7. Федеральная служба государственной статистики. Доступно по: gks.ru. [Russian Federal State Statistics Service. Available from: www.gks.ru. (In Russ.)].

8. Drange MR, Fram NR, Herman-Bonert V, Melmed S. Pituitary Tumor Registry: A Novel Clinical Resource 1. J Clin Endocrinol Metab. 2000;85(1):168-174. doi:10.1210/jcem.85.1.6309

9. Berkmann S, Schütz P, Röthlisberger M, et al. The Swiss Pituitary Registry—SwissPit. In: ; 2018. doi:10.1055/s-0038-1660716

10. Khamseh ME, Mohajeri Tehrani MR, Mousavi Z, et al. Iran Pituitary Tumor Registry: Description of the Program and Initial Results. Arch Iran Med. 2017;20(12):746-751. http://www.ncbi.nlm.nih.gov/pubmed/29664314

11. Lavrentaki A, Paluzzi A, Wass JAH, Karavitaki N. Epidemiology of acromegaly: review of population studies. Pituitary. 2017;20(1):4-9. doi:10.1007/s11102-016-0754-x

12. Petrossians P, Daly AF, Natchev E, et al. Acromegaly at diagnosis in 3173 patients from the Liège Acromegaly Survey (LAS) Database. Endocr Relat Cancer. 2017;24(10):505-518. doi:10.1530/ERC-17-0253

13. Colao A, Grasso LFS, Giustina A, et al. Acromegaly. Nat Rev Dis Prim. 2019;5(1):20. doi:10.1038/s41572-019-0071-6

14. Capatina C, Wass JAH. 60 YEARS OF NEUROENDOCRINOLOGY: Acromegaly. J Endocrinol. 2015;226(2):T141-T160. doi:10.1530/JOE-15-0109

15. Giuffrida G, Crisafulli S, Ferraù F, et al. Global Cushing’s disease epidemiology: a systematic review and meta-analysis of observational studies. J Endocrinol Invest. 2022;45(6):1235-1246. doi:10.1007/s40618-022-01754-1

16. Valassi E. Clinical presentation and etiology of Cushing’s syndrome: Data from <scp>ERCUSYN</scp>. J Neuroendocrinol. 2022;34(8). doi:10.1111/jne.13114

17. Stroud A, Dhaliwal P, Alvarado R, et al. Outcomes of pituitary surgery for Cushing’s disease: a systematic review and meta-analysis. Pituitary. 2020;23(5):595-609. doi:10.1007/s11102-020-01066-8

18. Valassi E, Chiodini I, Feelders RA, et al. Unmet needs in Cushing’s syndrome: the patients’ perspective. Endocr Connect. 2022;11(7). doi:10.1530/EC-22-0027

19. Young J, Bertherat J, Vantyghem MC, et al. Hepatic safety of ketoconazole in Cushing’s syndrome: results of a Compassionate Use Programme in France. Eur J Endocrinol. 2018;178(5):447-458. doi:10.1530/EJE-17-0886

20. Lacroix A, Gu F, Gallardo W, et al. Efficacy and safety of once-monthly pasireotide in Cushing’s disease: a 12 month clinical trial. Lancet Diabetes Endocrinol. 2018;6(1):17-26. doi:10.1016/S2213-8587(17)30326-1

21. Ntali G, Hakami O, Wattegama M, Ahmed S, Karavitaki N. Mortality of Patients with Cushing’s Disease. Exp Clin Endocrinol Diabetes. 2021;129(03):203-207. doi:10.1055/a-1197-6380

22. Chanson P, Maiter D. The epidemiology, diagnosis and treatment of Prolactinomas: The old and the new. Best Pract Res Clin Endocrinol Metab. 2019;33(2):101290. doi:10.1016/j.beem.2019.101290

23. Ntali G, Wass JA. Epidemiology, clinical presentation and diagnosis of non-functioning pituitary adenomas. Pituitary. 2018;21(2):111-118. doi:10.1007/s11102-018-0869-3

24. Ntali G, Capatina C, Fazal-Sanderson V, et al. Mortality in patients with non-functioning pituitary adenoma is increased: systematic analysis of 546 cases with long follow-up. Eur J Endocrinol. 2016;174(2):137-145. doi:10.1530/EJE-15-0967