Primary mediastinal and retroperitoneal malignant germ cell tumors in children and adolescents: Results of the TGM95 trial, a study of the French Society of Pediatric Oncology (Société Française des Cancers de l'Enfant). Issue 9 (17th March 2017)

• Record Type: Journal Article
• Authors: Sudour‐Bonnange, Hélène; Faure‐Conter, Cécile; Martelli, Hélène; Hameury, Frederic; Fresneau, Brice; Orbach, Daniel; Vérité, Cécile
• Is Part Of: Pediatric blood & cancer. Volume 64:Issue 9(2017)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2017-03-17
• Subjects: adolescents -- children -- malignant germ cell tumor -- mediastinal tumor -- retroperitoneal tumor; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26494
• Languages: English
• ISSNs: 1545-5009

Abstract

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.