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 was to describe the clinical features of nonteratomatous germ cell tumors (NTGCT) by reviewing a large series of patients.

Methods: Between 1945 and 1997, there were 78 cases of nonteratomatous germ cell tumors (NTGCT's) in children at The Children's Hospital, Columbus. Their records were reviewed retrospectively. There were 35 boys and 43 girls (M:F ratio 0.8). Mean follow-up was 87 months.

Results: Histological subtypes included germinoma (33 cases, 42%), endodermal sinus tumor (24 cases, 31%), embryonal carcinoma (12 cases, 15%), gonadoblastoma (4 cases, 5%), mixed histology (4 cases, 5%), and choriocarcinoma (1 case, 2%). Forty-two tumors were in gonadal sites, but a significant percentage were extragonadal (36 cases, 46%). Forty-six patients (59%) had localized disease, 18 (23%) had regional disease, and 14 (18%) had metastases. Treatment consisted of surgery and selective chemotherapy and radiation. Complete tumor resection was more likely for gonadal (29 of 42, 69%) than extragonadal primaries (15 of 36, 41%; P < or = .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 < or = .01). Survival in patients with localized disease (34 of 46, 74%) exceeded that in patients with regional extension or metastases (15 of 32, 47%; P < .05).

Conclusions: This study highlights the fact that tumor location, gonadal versus extragonadal, was important in determining prognosis, whereas tumor histology was not. This may be the result of a higher rate of complete tumor resection for gonadal primaries and underscores the important role of surgery in the optimal treatment of these unusual tumors.