Advanced treatment-resistant ovarian germ cell tumor (yolk sac tumor) with successful fertility realization 20 years after combined treatment: a case report

Background. Yolk sac tumors (YST) are classified as rare malignant germ cell tumors of the ovaries, most commonly found in young patients interested in preserving fertility. YST is characterized by large size, predominantly unilateral involvement, and increased production of alpha-fetoprotein, which can be used as an important diagnostic criterion for this condition. Among all germ cell tumors, YST has the least favorable prognosis. The primary goals of YST treatment are not only to improve prognosis and quality of life but also to enable the patient to fulfill her reproductive plans in the future.

Description of the clinical case. We present a clinical case of an aggressive course of yolk sac tumor in the ovary of a 15-year-old girl, requiring multiple surgeries, repeated courses of high-dose drug therapy, and bone marrow transplantation.

Conclusion. This is a rare case of YST with successful preservation of reproductive function due to modern medical technologies despite aggressive treatment.

1. Euscher E.D. Germ Cell Tumors of the Female Genital Tract. Surg Pathol Clin. 2019; 12(2): 621–49. doi: 10.1016/j.path.2019.01.005.

2. Reid B.M., Permuth J.B., Sellers T.A. Epidemiology of ovarian cancer: a review. Cancer Biol Med. 2017; 14(1): 9–32. doi: 10.20892/j.issn.2095-3941.2016.0084.

3. Solopova A.E., Sologub Yu.N., Makatsaria A.D., Ternovoy S.K. Ovarian germ cell tumors – modern approaches to diagnosis. Obstetrics and Gynecology. 2016; 12: 28–33. (in Russian). doi: 10.18565/aig.2016.12.28-33.

4. Dantkale K.S., Agrawal M. A Comprehensive Review of Current Trends in the Diagnosis and Treatment of Ovarian Germ Cell Tumors. Cureus. 2024; 16(1). doi: 10.7759/cureus.52650.

5. Soft Tissue and Bone Tumours: WHO Classifcation of Tumours (Medicine). 5th Edition, Vol. 3. World Health Organization, 2020.

6. Zong X., Zhang Y., Peng X., Cao D., Yu M., Wang J., Li H., Guo X., Liang H., Yang J. Analysis of the genomic landscape of yolk sac tumors reveals mechanisms of evolution and chemoresistance. Nat Commun. 2021; 12(1): 3579. doi: 10.1038/s41467-021-23681-0.

7. Stefanelli E., Talarico V., Scavone M., Carboni E., Stranieri G., Galati M.C., Salerno D., Raiola G. Acute abdominal pain in an adolescent girl with an ovarian yolk sac tumor. Acta Biomed. 2019; 90(4): 599–602. doi: 10.23750/abm.v90i4.9017.

8. Safdar N.S., Stall J.N., Young R.H. Malignant Mixed Germ Cell Tumors of the Ovary: An Analysis of 100 Cases Emphasizing the Frequency and Interrelationships of Their Tumor Types. Am J Surg Pathol. 2021; 45(6): 727–41. doi: 10.1097/PAS.0000000000001625.

9. Radswiki T., Baba Y., Weerakkody Y., et al. Ovarian yolk sac tumor. [Internet]. Reference article. Radiopaedia.org. doi: 10.53347/rID-15285. URL: https://radiopaedia.org/articles/ovarian-yolk-sac-tumour [cited 2024 Mar 22].

10. Anfelter P., Testa A., Chiappa V., Froyman W., Fruscio R., Guerriero S., Alcazar J.L., Mascillini F., Pascual M.A., Sibal M., Savelli L., Zannoni G.F., Timmerman D., Epstein E. Imaging in gynecological disease (17): ultrasound features of malignant ovarian yolk sac tumors (endodermal sinus tumors). Ultrasound Obstet Gynecol. 2020; 56(2): 276–84. doi: 10.1002/uog.22002. Erratum in: Ultrasound Obstet Gynecol. 2020; 56(6): 966. doi: 10.1002/uog.23526.

11. Eddaoualline H., Sami H., Rais H., Belbaraka R., El Omrani A., Khouchani M. Ovarian Yolk Sac Tumor: A Case Report and Literature Review. Clin Case Rep Int. 2018; 2: 1057.

12. Nonepithelial ovarian tumors. Clinical guidelines. Approved at the meeting of the Scientifc and Practical Council of the Ministry of Health of the Russian Federation (protocol of 20.12.2019, No. 10/2-3-4). 2020. 71 p. (in Russian).

13. Gutorov S.L., Novikova E.G., Rumyantsev A.A., Tryakin A.A., Tyulyanina A.S., Urmancheeva A.F., Khokhlova S.V. Malignant nonepithelial ovarian tumors: practical guidelines. Part 1. Malignant tumors. 2023; 13(3s2–1): 216–34. (in Russian). doi: 10.18027/2224-5057-2023-13-3s2-1-216-234.

14. Graham R., MacDonald N.D., Lockley M., Miller R., Butler J., Murali K., Sarker S.J., Banerjee S., Stoneham S., Shamash J., Liberale V., Berney D.M., Newton C. Surgical management and outcomes for stage 1 malignant ovarian germ cell tumours: A UK multicentre retrospective cohort study. Eur J Obstet Gynecol Reprod Biol. 2022; 271: 138–44. doi: 10.1016/j.ejogrb.2022.02.013.

15. Liu P., Li Z., Cheng X., Gao Q., Che Y., Zhang Z., Chu R., Chen Z., Zhang Y., Wang Q., Dou Z., Wei Y., Cui Z., Wang J., Xie X., Ma D., Yang X., Kong B., Song K. Assessment of prognostic and reproductive outcomes of omentectomy for patients with clinically apparent early-stage (I, II) malignant ovarian germ cell tumours: A multicentre retrospective study. BJOG. 2022; 129(s2): 23–31. doi: 10.1111/1471-0528.17325.

16. Pallavi V.R., Kansal Y., Rathod P.S., Shobha K., Kundargi R., Bafna U.D., Devi U., Vijay C.R. Impact of Optimal Therapy and Prognostic Factors in Malignant Germ Cell Tumors of Ovary: 20 Years’ Institutional Experience. Indian J Surg Oncol. 2022; 13(3): 633–40. doi: 10.1007/s13193-022-01537-3.

17. Uccello M., Boussios S., Samartzis E.P., Moschetta M. Systemic anti-cancer treatment in malignant ovarian germ cell tumours (MOGCTs): current management and promising approaches. Ann Transl Med. 2020; 8(24): 1713. doi: 10.21037/atm.2020.04.15.

18. Newton C., Murali K., Ahmad A., Hockings H., Graham R., Liberale V., Sarker S.J., Ledermann J., Berney D.M., Shamash J., Banerjee S., Stoneham S., Lockley M. A multicentre retrospective cohort study of ovarian germ cell tumours: Evidence for chemotherapy de-escalation and alignment of paediatric and adult practice. Eur J Cancer. 2019; 113: 19–27. doi: 10.1016/j.ejca.2019.03.001.

19. Park J.Y., Kim D.Y., Suh D.S., Kim J.H., Kim Y.M., Kim Y.T., Nam J.H. Outcomes of Surgery Alone and Surveillance Strategy in Young Women With Stage I Malignant Ovarian Germ Cell Tumors. Int J Gynecol Cancer. 2016; 26(5): 859–64. doi: 10.1097/IGC.0000000000000702.

20. Kojimahara T., Nakahara K., Takano T., Yaegashi N., Nishiyama H., Fujimori K., Sato N., Terada Y., Tase T., Yokoyama Y., Mizunuma H., Shoji T., Sugiyama T., Kurachi H. Yolk sac tumor of the ovary: a retrospective multicenter study of 33 Japanese women by Tohoku Gynecologic Cancer Unit (TGCU). Tohoku J Exp Med. 2013; 230(4): 211–7. doi: 10.1620/tjem.230.211.

21. Park J.Y., Kim D.Y., Suh D.S., Kim J.H., Kim Y.M., Kim Y.T., Nam J.H. Analysis of outcomes and prognostic factors after fertility-sparing surgery in malignant ovarian germ cell tumors. Gynecol Oncol. 2017; 145(3): 513–8. doi: 10.1016/j.ygyno.2017.03.023.

22. Nasioudis D., Chapman-Davis E., Frey M.K., Caputo T.A., Holcomb K. Management and prognosis of ovarian yolk sac tumors; an analysis of the National Cancer Data Base. Gynecol Oncol. 2017; 147(2): 296–301. doi: 10.1016/j.ygyno.2017.08.013.

23. Nasioudis D., Frey M.K., Chapman-Davis E., Caputo T.A., Holcomb K. Fertility-preserving surgery for advanced stage ovarian germ cell tumors. Gynecol Oncol. 2017; 147(3): 493–6. doi: 10.1016/j.ygyno.2017.10.010.

24. Di Tucci C., Casorelli A., Morrocchi E., Palaia I., Muzii L., Panici P.B. Fertility management for malignant ovarian germ cell tumors patients. Crit Rev Oncol Hematol. 2017; 120: 34–42. doi: 10.1016/j.critrevonc.2017.10.005.