HCG-secreting pineal germinoma - the cause of gonadotropin-independent precocious sexual development: description of the clinical case and literature review


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Abstract

Background. Gonadotropin-independent precocious sexual development (PSD) against the background of human chorionic gonadotropin (HCG)-secreting tumors is casuistically rare in the practice of pediatric endocrinologists and oncologists. Description of the clinical case. A clinical case of PSD caused by HCG-secreting pineal germinoma in 6-year-old boy is described. Clinical manifestation of the tumor was characterized by the appearance and rapid progression of the pubic hair, acne, low voice, an increasing the penis size, as well as a parallel increase in the cerebral and focal neurological symptoms during the month. High testosterone and ß-HCG levels against the background of low luteinizing (LH) and follicle-stimulating hormone (FSH) levels allowed to confirm the gonadotropin-independent genesis of PSD. The range of clinical endocrinologicl manifestations in HCG-secreting tumors is diverse and depends on gender, age of tumor manifestation and the presence of aromatase expression in tumor tissue in girls, and is also likely to be related to the level of β-HCG elevation. Conclusion. Awareness of doctors of various specialties about the first clinical symptoms that may appear several months before a diagnosis is established will help in the timely diagnosis and improvement of the prognosis of HCG-secreting tumors in children.

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About the authors

E. S Astashova

Ural State Medical University

Alexey V. Kiyaev

Ural State Medical University; Regional Children's Clinical Hospital

Email: thyroend@mail.ru
MD, Associate Professor at the Department of Outpatient Pediatrics and Pediatrics FATPRS, Chief Freelance Children’s Endocrinologist of the Ministry of Healthcare of the Russian Federation in the Urals Federal District, Chief Freelance Children’s Endocrinologist of the Ministry of Healthcare of the Sverdlovsk Region

References

  1. Федеральные клинические рекомендации (протоколы) по ведению детей с эндокринными заболеваниями. Под ред. И.И. Дедова, В.А. Петерковой. М., 2014
  2. Kuo H.-C., Sheen J.-M., Wu K.-S., et al. Precocious puberty due to human chorionic gonadotropin-secreting pineal tumor Chang Gung Med J. 2006;29(2):321-24
  3. Cohen A.R., Wilson J.A., Sadeghi-Nejad A. Gonadotropin-secreting pineal teratoma causing precocious puberty. Neurosurgery. 1991;28(4):597-602.
  4. Starzyk J., Starzyk B., Bartnik-Mikuta A., et al. Gonadotropin releasing hormone-independent precocious puberty in a 5 year-old girl with suprasellar germ cell tumor secreting beta-hCG and alpha-fetoprotein. J Pediatr Endocrinol Metab. 2001;14(6):789-96
  5. Ahmed S.R., Shalet S.M., Price D.A., Pearson D. Human chorionic gonadotrophin secreting pineal germinoma and precocious puberty. Arch Dis Child. 1983;58(9):743-45.
  6. Nogueira K., Liberman B., Pimentel-Filho F.R., et al. hCG-secreting pineal teratoma causing precocious puberty: report of two patients and review of the literature. J. Pediatr Endocrinol Metab. 2002;15(8):1195-201.
  7. Blüme l.P., Grümayer E.R., Machacek E., Stögmann W. Beta-HCG-producing choriocarcinoma of the pineal area as a cause of precocious puberty. Helv Paediatr Acta. 1985;40(6):473- 79.
  8. Hammadur Rahaman S.K., Khandelwal D., Khadgawat R., et al. Peripheral Precocious Puberty Caused by Human Chorionic Gonadotropin Producing Pineal Gland Tumor. Ind Pediatr. 2018;55(3):254-56.
  9. Kitanaka C., Matsutani M., Sora S., et al. Precocious puberty in a girl with an hCG-secreting suprasellar immature teratoma. Case report. J. Neurosurg. 1994;81(4):601-4.
  10. Takahashi H., Tokuda N., Kariya H. Precocious puberty in a seven-year-old boy due to human chorionic gonadotropin producing pineal tumor detected by nuclear magnetic resonance computed tomographic scanning. Acta Paediatr Jpn. 1990;32(1):88-93.
  11. Inamo Y., Hiyoshi K., Hanawa Y., Okuni M. Precocious puberty caused by an hCG-producing tumor of the septum pellucidum. Acta Paediatr Jpn. 1991;33(5):681-84.
  12. Lin C.-M., Lee C.-T., Tung Y-C., et al. Endocrine dysfunction in Taiwanese children with human chorionic gonadotropin-secreting germ cell tumors. J. Formosan Med Ass. 2014;113:102-5.
  13. Claudia Lozano C., Marcela Molina P. Beta-hCG-producing thymic teratoma: an uncommon cause of peripheral precocious puberty. Rev Chil Pediatr 2018;89(3):373- 79.
  14. Senniappan S., Wood D., Hakeem V., et al. Gonadotrophin-independent precocious puberty associated with later diagnosis of testicular embryonal carcinoma. Horm Res Paediatr. 2014;82(4):272-77. doi: 10.1159/000362187.
  15. Королева Д.Н., Олина Т.С., Коваленко Т.В. ХГЧ-секретирующая гепатобластома. Проблемы эндокринологии. 2017;63(2):127-29
  16. Heimann A., White P.F., Riely C.A., et al. Hepatoblastoma presenting as isosexual precocity The clinical importance of histologic and serologic parameters. J Clin Gastroenterol. 1987; 9(1):105-10.
  17. Beach R., Betts P., Radford M., Millward-Sadler H. Production of human chorionic gonadotrophin by a hepatoblastoma resulting in precocious puberty. J Clin Pathol. 1984;37(7): 734-37.
  18. Flores F., Solano A., Rebeil R., et al. Isosexual precocious puberty in a male infant with hepatoblastoma. Rev Invest Clin. 1979; 31(3):251-55.
  19. Bode H.H., Schimana W., Swai B., Bode U. Sexual precocity associated with an abdominal tumor in an African boy. J Pediatr Endocrinol Metab. 2008;21(3):275-77.
  20. Butenandt O., Knorr D., Hecker W.C., Löhrs U. Precocious puberty in a boy with HcG-producing hepatoma. Case report. Helv Paediatr Acta. 1980;35(2):155-63.
  21. Gajendra S., Chopra A., Kumar R. β-hCG-Secreting Anaplastic Large Cell Lymphoma. Turk J Haematol. 2015 Apr 27. doi: 10.4274/tjh.2014.0145.
  22. Senba M., Watanabe M. Ectopic production of beta-subunit of human chorionic gonadotropin in malignant lymphoma. Zentralbl Pathol. 1991;137(5):402-4.
  23. O'Marcaigh A.S., Ledger G.A., Roche P.C., et al. Aromatase expression in human germinomas with possible biological effects. J Clin Endocrinol Metab. 1995;80(12):3763-66.
  24. Fuqua J.S. Treatment and Outcomes of Precocious Puberty: An Update. J Clin Endocrinol Metab. 2013;98(6):2198-207. doi: 10.1210/jc.2013-1024.

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