ABSTRACT 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%), fol- lowing 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 Journal of the Egyptian Nat. Cancer Inst., Vol. 20, No. 1, March: 70-79, 2008 The Role of Surgical Management in Pediatric Germ Cell Tumors (GCTs), NCI Case Series IBRAHIM M.Y. FAKHR, M.D., M.R.C.S.*; EL-SAYED ASHRAF KHALIL, M.D., F.R.C.S.I.*; TAREK S. EL-BARADIE, M.D.*; MOHAMED A. SHAALAN, M.D.*; LOBNA M. SHALABY, M.D.**; SHIMAA L.A. NASSIF, M.Sc.** and IMAN G. FARAHAT, M.D.*** The Departments of Surgical Oncology*, Pediatric Oncology** and Pathology***, National Cancer Institute (NCI), Cairo University. 70 with irresectable disease had comparable overall survival while none could be rendered disease free with chemo- therapy. 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. Key Words: Germ cell tumors – Childhood solid tumors – management – Surgery. INTRODUCTION Gonadal and extragonadal germ cell tumors are infrequent in children representing approx- imately 3% of cancers diagnosed in persons younger than 15 years [1,2]. Germ cell tumors are presumed to share a common cell of origin, the primordial germ cell, yet they remain a heterogeneous group of tumors. Variations re- garding age, sites of presentation, histopathology and malignant potential stem from the differ- ences in the stage of germ cell development at tumorigenesis, the differences in the tumor environment secondary to the gender of the patient and to the location of the clone and the occurrence of specific genetic aberrations [3]. Sacrococcygeal teratomas are the most common germ cell tumors of childhood, accounting for 40% of all and up to 78% of extragonadal germ cell tumors. They also are the most frequently recognized neoplasm of fetuses, with approxi- Correspondence: Ibrahim Fakhr, MD, MRCS ibrahimfakhr@hotmail.com