Hélène Sudour-Bonnange1, Cécile Faure-Conter2, Hélène Martelli3, Frederic Hameury4, Brice Fresneau5, Daniel Orbach6, Cécile Vérité7. 1. Pediatric Oncology Unit, Anti Cancer Center Oscar Lambret, Lille Cedex, France. 2. Pediatric Oncology Unit, Pediatric Hematology-Oncology Institute, Lyon Cedex, France. 3. Pediatric Surgery Department, Bicêtre Hospital, Le Kremlin-Bicêtre, France. 4. Pediatric Surgery Department, Pediatric Hematology-Oncology Institute, Lyon Cedex, France. 5. Pediatric Oncology Unit, Anti Cancer Center Gustave Roussy, Villejuif Cedex, France. 6. Pediatric Adolescent Young Adult Department, Institut Curie, Paris, France. 7. Pediatric Hematology-Oncology Unit, Pellegrin Hospital, CHU Bordeaux, Bordeaux, France.
Abstract
PURPOSE: To examine the clinical presentation, treatment and results in children and adolescents with primary mediastinal (PM) and retroperitoneal (RP) germ cell tumors (GCTs). METHODS: The TGM95 trial for malignant GCTs was conducted in France between 1995 and 2005 to evaluate a strategy adapted to prognostic factors with cisplatin-based chemotherapy and surgical management. We reviewed patients with TGCTs at PM and RP sites. RESULTS: Among 239 patients, there were 16 patients with PM and 5 with RP tumors, which represent 9% of all patients, highlighting the rarity of these extragonadal locations. A bimodal demographic distribution was observed (11/21 patients <5 years old and 7/21 patients >12 years old). A majority of patients presented with bulky tumors that required urgent care with neoadjuvant chemotherapy. In all patients, elevation of alpha-fetoprotein indicated a yolk sac tumor component. Human chorionic gonadotrophin was elevated in five patients (four adolescents), suggesting a choriocarcinoma or seminoma component. The diagnosis was based on elevation of these tumor markers in addition to imaging. Chemosensitivity was observed for a majority of patients. An aggressive surgical approach allowed a microscopic complete resection in 12/15 patients with PM tumors and 4/5 with RP tumors. Overall, 14/16 and 4/5 patients survived, respectively. Three adolescents died of tumor progression. CONCLUSION: In children with mediastinal or RP GCTs, the prognosis is favorable when a strategy of delayed aggressive surgery is performed after cisplatin-based chemotherapy. Younger patients have a better prognosis. Relapses were observed only in adolescents and could not be cured.
PURPOSE: To examine the clinical presentation, treatment and results in children and adolescents with primary mediastinal (PM) and retroperitoneal (RP) germ cell tumors (GCTs). METHODS: The TGM95 trial for malignant GCTs was conducted in France between 1995 and 2005 to evaluate a strategy adapted to prognostic factors with cisplatin-based chemotherapy and surgical management. We reviewed patients with TGCTs at PM and RP sites. RESULTS: Among 239 patients, there were 16 patients with PM and 5 with RP tumors, which represent 9% of all patients, highlighting the rarity of these extragonadal locations. A bimodal demographic distribution was observed (11/21 patients <5 years old and 7/21 patients >12 years old). A majority of patients presented with bulky tumors that required urgent care with neoadjuvant chemotherapy. In all patients, elevation of alpha-fetoprotein indicated a yolk sac tumor component. Human chorionic gonadotrophin was elevated in five patients (four adolescents), suggesting a choriocarcinoma or seminoma component. The diagnosis was based on elevation of these tumor markers in addition to imaging. Chemosensitivity was observed for a majority of patients. An aggressive surgical approach allowed a microscopic complete resection in 12/15 patients with PM tumors and 4/5 with RP tumors. Overall, 14/16 and 4/5 patients survived, respectively. Three adolescents died of tumor progression. CONCLUSION: In children with mediastinal or RP GCTs, the prognosis is favorable when a strategy of delayed aggressive surgery is performed after cisplatin-based chemotherapy. Younger patients have a better prognosis. Relapses were observed only in adolescents and could not be cured.