NONTERATOMATOUS GERM-CELL TUMORS IN CHILDREN

Background/Purpose: Totipotential germ cells may give rise to a broad range of tumors. The teratomatous variety of germ cell tumors has been the subject of several large studies. The goal of the current study w as to describe the clinical features of nonteratomatous germ cell tumo rs (NTGCT) by reviewing a large series of patients. Methods: Between 1 945 and 1997, there were 78 cases of nonteratomatous germ cell tumors (NTGCT's) in children at The Children's Hospital, Columbus. Their reco rds were reviewed retrospectively. There were 35 boys and 43 girls (M: F ratio 0.8). Mean follow-up was 87 months. Results: Histological subt ypes included germinoma (33 cases, 42%), endodermal sinus tumor (24 ca ses, 31%), embryonal carcinoma (12 cases, 15%), gonadoblastoma (4 case s, 5%), mixed histology (4 cases, 5%), and choriocarcinoma (1 case, 2% ). Forty-two tumors were in gonadal sites, but a significant percentag e were extragonadal (36 cases, 46%). Forty-six patients (59%) had loca lized disease, 18 (23%) had regional disease, and 14 (18%) had metasta ses. Treatment consisted of surgery and selective chemotherapy and rad iation. Complete tumor resection was more likely for gonadal (29 of 42 , 69%) than extragonadal primaries (15 of 36, 41%; P less than or equa l to.05). Forty-nine (63%) of all patients survived, whereas 29 (37%) died of their disease. Survival in patients with gonadal primaries (32 of 42, 76%) exceeded that in patients with extragonadal primaries (17 of 36, 47%; P less than or equal to.01). Survival in patients with lo calized disease (34 of 46, 74%) exceeded that in patients with regiona l extension or metastases (15 of 32, 47%; P<.05). Conclusions: This st udy highlights the fact that tumor location, gonadal versus extragonad al, was important in determining prognosis, whereas tumor histology wa s not. This may be the result of a higher rate of complete tumor resec tion for gonadal primaries and underscores the important role of surge ry in the optimal treatment of these unusual tumors. J Pediatr Surg 33 :1122-1127. Copyright (C) 1998 by W.B. Saunders Company.