The role of surgical management in pediatric germ cell tumors (GCTs), NCI case series.

PURPOSE To review the experience of a tertiary referral center in pediatric germ cell tumors (GCTs) in the last 8 years and to investigate the impact of surgery and site of disease on prognosis. PATIENTS AND METHODS We retrospectively analyzed the cases of pediatric germ cell tumors at National Cancer Institute over an 8 years period. Data concerning diagnosis, surgery and medical decisions were reviewed and analyzed for all patients. A total of 34 children with (GCTS) were found, with a mean age, at presentation, of 6.7 years and a follow-up period ranging from 3-52 months. One patient with benign GCT was excluded during analysis of the results. RESULTS Among the 34 patients, there were 14 males and 20 females with mean age of 6.7 years (range: 9 months-15 years), with male to female ratio 1:1.4. All patients were symptomatic at presentation, most commonly with abdominal swelling (18 patients; 52.9%). Anatomic distribution of GCTs according to sex organ involvement was either gonadal in 21 patients (61.8%) or extragonadal in 13 patients (38.2%). All patients had surgery either in the form of curative resection or biopsy after formal exploration and evidence of irresectability. No significant surgical morbidity or mortality were encountered in our patients. Yolk sac tumor and malignant teratoma were the commonest histologic subtypes in our series. Metastatic disease was encountered in nine out of 33 patients (27.2%). Adjuvant chemotherapy was administered in 28 out of 33 patients (84.8%), following surgery, including all patients with extragonadal disease. Our patients were followed-up to 52 months. Twenty-two patients (66.7%) had no recurrence while two patients (6.1%) died from disease. Pelvic extragonadal site was the worst site regarding resectability. Complete surgical resection showed better disease free survival, while those with irresectable disease had comparable overall survival while none could be rendered disease free with chemotherapy. CONCLUSION The initial surgical approach to malignant GCTs at all sites should be complete resection when possible; the morbidity of extensive surgical resection should be weighed carefully against the good tumor control with chemotherapy. Surgical staging does not preclude preservation of fertility, which should always be considered in this young age. The site of primary disease plays a role in the prognosis of pediatric germ cell tumors with the extragonadal pelvic tumors being the worst regarding resectability. Good tumor response can be achieved with surgery and chemotherapy even for advanced stage and metastatic disease.