Diagnosis, treatment and prognosis of primary mediastinal yolk sac tumor in 18 patients_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

GAO Xuehan , CHEN Yeye , ZHANG Jiaqi ,  LI Shanqing

Department of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, P. R. China;

Corresponding author：

LI Shanqing, Email: lishanqing@pumch.cn

Keywords：

Primary mediastinal yolk sac tumor; endodermal sinus tumor; pathological characteristics; prognosis

DOI：

10.7507/1007-4848.202107089

Video：

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Objective To investigate the clinical characteristics and treatment of primary mediastinal yolk sac tumor (PMYST). Methods We collected the clinical data of PMYST patients who were admitted to the Department of Thoracic Surgery of Peking Union Medical College Hospital from September 2000 to September 2020. The clinical and pathological characteristics, treatment and prognosis of PMYST patients were retrospectively analyzed. Results Finally 18 patients were enrolled, including 17 males and 1 female with a median age of 22.0 (16.6, 26.2) years. Patients had increased level of alpha fetoprotein (AFP). The pathological type can be single yolk sac tumor or combined with other mediastinal germ cell tumors. Chemotherapy and radical surgery were the main treatment methods. Extensive resection was feasible for patients with tumor invasion of other organs. Seven patients developed lung or pleural metastasis after operation, and 3 of them had extrapleural metastasis. One patient recurred within 1 year after surgery. All patients were followed up by telephone or outpatient department. At the last follow-up, 5 patients survived, 9 died, and 4 were lost to follow up, with a median survival of 16.8 months. The median disease-free survival was 14.9 months. The survival rates at 1, 3 and 5 years were 73.3%, 28.6% and 11.8%, respectively. Conclusion PMYST often occurs in young-middle aged male patients. Preoperative puncture can provide an effective diagnostic basis. R0 resection, AFP returning to normal after treatment and no metastasis may be potential indicators of good prognosis. The overall prognosis of PMYST is poor, and some patients can achieve long-term survival after treatment.

Citation： GAO Xuehan, CHEN Yeye, ZHANG Jiaqi, LI Shanqing. Diagnosis, treatment and prognosis of primary mediastinal yolk sac tumor in 18 patients. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2023, 30(9): 1280-1289. doi: 10.7507/1007-4848.202107089 Copy

1.	Geng R, Zheng Z, Lin Y, et al. Clinical characteristics and prognostic factors of male yolk sac tumor: A surveillance, epidemiology, and end results program study. World J Urol, 2021, 39(4): 1211-1217.
2.	Detterbeck FC, Nicholson AG, Kondo K, et al. The Masaoka-Koga stage classification for thymic malignancies clarification and definition of terms. Zhongguo Fei Ai Za Zhi, 2014, 17(2): 75-81.
3.	Teilum G. Endodermal sinus tumors of the ovary and testis. Comparative morphogenesis of the so-called mesoephroma ovarii (Schiller) and extraembryonic (yolk sac-allantoic) structures of the rat's placenta. Cancer, 1959, 12: 1092-1105.
4.	Talerman A. Endodermal sinus (yolk sac) tumor elements in testicular germ-cell tumors in adults: Comparison of prospective and retrospective studies. Cancer, 1980, 46(5): 1213-1217.
5.	Albany C, Einhorn LH. Extragonadal germ cell tumors: Clinical presentation and management. Curr Opin Oncol, 2013, 25(3): 261-265.
6.	Yamada Y, Tomita K, Fujimura T, et al. Metachronous testicular tumor developing eight years after retroperitoneal extragonadal germ cell tumor. Int J Urol, 2008, 15(3): 267-269.
7.	Glenn OA, Barkovich AJ. Intracranial germ cell tumors: A comprehensive review of proposed embryologic derivation. Pediatr Neurosurg, 1996, 24(5): 242-251.
8.	Takeda S, Miyoshi S, Ohta M, et al. Primary germ cell tumors in the mediastinum: A 50-year experience at a single Japanese institution. Cancer, 2003, 97(2): 367-376.
9.	Motzer RJ, Amsterdam A, Prieto V, et al. Teratoma with malignant transformation: Diverse malignant histologies arising in men with germ cell tumors. J Urol, 1998, 159(1): 133-138.
10.	Schneider DT, Schuster AE, Fritsch MK, et al. Genetic analysis of mediastinal nonseminomatous germ cell tumors in children and adolescents. Genes Chromosomes Cancer, 2002, 34(1): 115-125.
11.	Marina N, London WB, Frazier AL, et al. Prognostic factors in children with extragonadal malignant germ cell tumors: A pediatric intergroup study. J Clin Oncol, 2006, 24(16): 2544-2548.
12.	Truong LD, Harris L, Mattioli C, et al. Endodermal sinus tumor of the mediastinum. A report of seven cases and review of the literature. Cancer, 1986, 58(3): 730-739.
13.	Imaniar R, Syahruddin E, Soepandi PZ, et al. Mediastinal yolk sac tumor infiltrating the heart. Exp Oncol, 2018, 40(1): 82-84.
14.	马义祥, 刘敬伟. 原发性纵隔卵黄囊瘤7例. 北京大学学报(医学版), 2019, 51(6): 1091-1095.
15.	Tsuchida Y, Kaneko M, Yokomori K, et al. Alpha-fetoprotein, prealbumin, albumin, alpha-1-antitrypsin and transferrin as diagnostic and therapeutic markers for endodermal sinus tumors. J Pediatr Surg, 1978, 13(1): 25-29.
16.	Andrade RS, Kesler KA, Wilson JL, et al. Short- and long-term outcomes after large pulmonary resection for germ cell tumors after bleomycin-combination chemotherapy. Ann Thorac Surg, 2004, 78(4): 1224-1228.
17.	Loehrer PJ, Johnson D, Elson P, et al. Importance of bleomycin in favorable-prognosis disseminated germ cell tumors: An Eastern Cooperative Oncology Group trial. J Clin Oncol, 1995, 13(2): 470-476.
18.	Sleijfer S. Bleomycin-induced pneumonitis. Chest, 2001, 120(2): 617-624.
19.	Simpson AB, Paul J, Graham J, et al. Fatal bleomycin pulmonary toxicity in the west of Scotland 1991-95: A review of patients with germ cell tumours. Br J Cancer, 1998, 78(8): 1061-1066.
20.	Kesler KA, Rieger KM, Hammoud ZT, et al. A 25-year single institution experience with surgery for primary mediastinal nonseminomatous germ cell tumors. Ann Thorac Surg, 2008, 85(2): 371-378.
21.	Vuky J, Bains M, Bacik J, et al. Role of postchemotherapy adjunctive surgery in the management of patients with nonseminoma arising from the mediastinum. J Clin Oncol, 2001, 19(3): 682-688.
22.	Radaideh SM, Cook VC, Kesler KA, et al. Outcome following resection for patients with primary mediastinal nonseminomatous germ-cell tumors and rising serum tumor markers post-chemotherapy. Ann Oncol, 2010, 21(4): 804-807.
23.	Ganjoo KN, Rieger KM, Kesler KA, et al. Results of modern therapy for patients with mediastinal nonseminomatous germ cell tumors. Cancer, 2000, 88(5): 1051-1056.
24.	Bokemeyer C, Nichols CR, Droz JP, et al. Extragonadal germ cell tumors of the mediastinum and retroperitoneum: Results from an international analysis. J Clin Oncol, 2002, 20(7): 1864-1873.
25.	Fujita M, Inoue M, Tanizawa O, et al. Retrospective review of 41 patients with endodermal sinus tumor of the ovary. Int J Gynecol Cancer, 1993, 3(5): 329-335.
26.	Wang P, Hou G, Li F, et al. A case of primary sinonasal yolk sac tumor. Clin Nucl Med, 2020, 45(11): 908-909.
27.	Choi JE, Choe AR, Yoon SE, et al. Germ cell tumor targeting chemotherapy in gastric adenocarcinoma with an endodermal sinus tumor component: A case report. Chemotherapy, 2017, 62(1): 54-57.
28.	Shojaei H, Hong H, Redline RW. High-level expression of divergent endodermal lineage markers in gonadal and extra-gonadal yolk sac tumors. Mod Pathol, 2016, 29(10): 1278-1288.
29.	International germ cell consensus classification: A prognostic factor-based staging system for metastatic germ cell cancers. International Germ Cell Cancer Collaborative Group. J Clin Oncol, 1997, 15(2): 594-603.
30.	Hartmann JT, Nichols CR, Droz JP, et al. Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors. J Natl Cancer Inst, 2000, 92(1): 54-61.
31.	Bokemeyer C, Droz JP, Horwich A, et al. Extragonadal seminoma: an international multicenter analysis of prognostic factors and long term treatment outcome. Cancer, 2001, 91(7): 1394-1401.
32.	Sakaguchi M, Maebayashi T, Aizawa T, et al. Successful radiotherapy in postoperative recurrence of a primary mediastinal yolk sac tumor: A case report. Thorac Cancer, 2016, 7(3): 358-362.

1. Geng R, Zheng Z, Lin Y, et al. Clinical characteristics and prognostic factors of male yolk sac tumor: A surveillance, epidemiology, and end results program study. World J Urol, 2021, 39(4): 1211-1217.
2. Detterbeck FC, Nicholson AG, Kondo K, et al. The Masaoka-Koga stage classification for thymic malignancies clarification and definition of terms. Zhongguo Fei Ai Za Zhi, 2014, 17(2): 75-81.
3. Teilum G. Endodermal sinus tumors of the ovary and testis. Comparative morphogenesis of the so-called mesoephroma ovarii (Schiller) and extraembryonic (yolk sac-allantoic) structures of the rat's placenta. Cancer, 1959, 12: 1092-1105.
4. Talerman A. Endodermal sinus (yolk sac) tumor elements in testicular germ-cell tumors in adults: Comparison of prospective and retrospective studies. Cancer, 1980, 46(5): 1213-1217.
5. Albany C, Einhorn LH. Extragonadal germ cell tumors: Clinical presentation and management. Curr Opin Oncol, 2013, 25(3): 261-265.
6. Yamada Y, Tomita K, Fujimura T, et al. Metachronous testicular tumor developing eight years after retroperitoneal extragonadal germ cell tumor. Int J Urol, 2008, 15(3): 267-269.
7. Glenn OA, Barkovich AJ. Intracranial germ cell tumors: A comprehensive review of proposed embryologic derivation. Pediatr Neurosurg, 1996, 24(5): 242-251.
8. Takeda S, Miyoshi S, Ohta M, et al. Primary germ cell tumors in the mediastinum: A 50-year experience at a single Japanese institution. Cancer, 2003, 97(2): 367-376.
9. Motzer RJ, Amsterdam A, Prieto V, et al. Teratoma with malignant transformation: Diverse malignant histologies arising in men with germ cell tumors. J Urol, 1998, 159(1): 133-138.
10. Schneider DT, Schuster AE, Fritsch MK, et al. Genetic analysis of mediastinal nonseminomatous germ cell tumors in children and adolescents. Genes Chromosomes Cancer, 2002, 34(1): 115-125.
11. Marina N, London WB, Frazier AL, et al. Prognostic factors in children with extragonadal malignant germ cell tumors: A pediatric intergroup study. J Clin Oncol, 2006, 24(16): 2544-2548.
12. Truong LD, Harris L, Mattioli C, et al. Endodermal sinus tumor of the mediastinum. A report of seven cases and review of the literature. Cancer, 1986, 58(3): 730-739.
13. Imaniar R, Syahruddin E, Soepandi PZ, et al. Mediastinal yolk sac tumor infiltrating the heart. Exp Oncol, 2018, 40(1): 82-84.
14. 马义祥, 刘敬伟. 原发性纵隔卵黄囊瘤7例. 北京大学学报(医学版), 2019, 51(6): 1091-1095.
15. Tsuchida Y, Kaneko M, Yokomori K, et al. Alpha-fetoprotein, prealbumin, albumin, alpha-1-antitrypsin and transferrin as diagnostic and therapeutic markers for endodermal sinus tumors. J Pediatr Surg, 1978, 13(1): 25-29.
16. Andrade RS, Kesler KA, Wilson JL, et al. Short- and long-term outcomes after large pulmonary resection for germ cell tumors after bleomycin-combination chemotherapy. Ann Thorac Surg, 2004, 78(4): 1224-1228.
17. Loehrer PJ, Johnson D, Elson P, et al. Importance of bleomycin in favorable-prognosis disseminated germ cell tumors: An Eastern Cooperative Oncology Group trial. J Clin Oncol, 1995, 13(2): 470-476.
18. Sleijfer S. Bleomycin-induced pneumonitis. Chest, 2001, 120(2): 617-624.
19. Simpson AB, Paul J, Graham J, et al. Fatal bleomycin pulmonary toxicity in the west of Scotland 1991-95: A review of patients with germ cell tumours. Br J Cancer, 1998, 78(8): 1061-1066.
20. Kesler KA, Rieger KM, Hammoud ZT, et al. A 25-year single institution experience with surgery for primary mediastinal nonseminomatous germ cell tumors. Ann Thorac Surg, 2008, 85(2): 371-378.
21. Vuky J, Bains M, Bacik J, et al. Role of postchemotherapy adjunctive surgery in the management of patients with nonseminoma arising from the mediastinum. J Clin Oncol, 2001, 19(3): 682-688.
22. Radaideh SM, Cook VC, Kesler KA, et al. Outcome following resection for patients with primary mediastinal nonseminomatous germ-cell tumors and rising serum tumor markers post-chemotherapy. Ann Oncol, 2010, 21(4): 804-807.
23. Ganjoo KN, Rieger KM, Kesler KA, et al. Results of modern therapy for patients with mediastinal nonseminomatous germ cell tumors. Cancer, 2000, 88(5): 1051-1056.
24. Bokemeyer C, Nichols CR, Droz JP, et al. Extragonadal germ cell tumors of the mediastinum and retroperitoneum: Results from an international analysis. J Clin Oncol, 2002, 20(7): 1864-1873.
25. Fujita M, Inoue M, Tanizawa O, et al. Retrospective review of 41 patients with endodermal sinus tumor of the ovary. Int J Gynecol Cancer, 1993, 3(5): 329-335.
26. Wang P, Hou G, Li F, et al. A case of primary sinonasal yolk sac tumor. Clin Nucl Med, 2020, 45(11): 908-909.
27. Choi JE, Choe AR, Yoon SE, et al. Germ cell tumor targeting chemotherapy in gastric adenocarcinoma with an endodermal sinus tumor component: A case report. Chemotherapy, 2017, 62(1): 54-57.
28. Shojaei H, Hong H, Redline RW. High-level expression of divergent endodermal lineage markers in gonadal and extra-gonadal yolk sac tumors. Mod Pathol, 2016, 29(10): 1278-1288.
29. International germ cell consensus classification: A prognostic factor-based staging system for metastatic germ cell cancers. International Germ Cell Cancer Collaborative Group. J Clin Oncol, 1997, 15(2): 594-603.
30. Hartmann JT, Nichols CR, Droz JP, et al. Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors. J Natl Cancer Inst, 2000, 92(1): 54-61.
31. Bokemeyer C, Droz JP, Horwich A, et al. Extragonadal seminoma: an international multicenter analysis of prognostic factors and long term treatment outcome. Cancer, 2001, 91(7): 1394-1401.
32. Sakaguchi M, Maebayashi T, Aizawa T, et al. Successful radiotherapy in postoperative recurrence of a primary mediastinal yolk sac tumor: A case report. Thorac Cancer, 2016, 7(3): 358-362.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Diagnosis, treatment and prognosis of primary mediastinal yolk sac tumor in 18 patients

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