Mediastinal germ cell tumors in childhood


YALÇIN B., Demir H. A. , Tanyel F. C. , AKÇÖREN Z., VARAN A., Akyüz C., ...More

Pediatric Hematology and Oncology, vol.29, no.7, pp.633-642, 2012 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 29 Issue: 7
  • Publication Date: 2012
  • Doi Number: 10.3109/08880018.2012.713084
  • Title of Journal : Pediatric Hematology and Oncology
  • Page Numbers: pp.633-642
  • Keywords: children, germ cell tumor, malignant, mediastinum, nonseminomatous, teratoma, PROGNOSTIC-FACTORS, CHILDREN, ADOLESCENTS, INTERGROUP, TERATOMAS, EXPERIENCE, PROTOCOLS

Abstract

Background: Mediastinal germ cell tumors (GCTs) are rare and usually located in anterior mediastinum. We aimed to review clinical and pathological characteristics of these tumors. Procedure: Between 1973 and 2011, 24 children with mediastinal GCTs were diagnosed. Hospital files were reviewed for presenting complaints, clinical, radiological and other laboratory data, surgical practices, treatments, and outcomes. Results: Median age was 4.5 years (0.2-16) (male/female: 10/14). Most common initial complaints were dyspnea, cough, anorexia/fatigue, fever, and chest pain. Primary tumors were located in anterior mediastinum (n = 22), posterior mediastinum (n = 1), and sternum (n = 1). Thirteen of 24 cases had mature teratomas (54.2%); four (16.7%) endodermal sinus tumor (EST); four (16.7%) immature teratomas; and one (4.2%) each of embryonal carcinoma, teratocarcinoma, and malignant teratoma. Mature teratomas underwent only surgical resection and were under follow-up without disease. Four cases with ESTs received chemotherapy and radiotherapy (n = 3), three underwent surgical resections: three died, one was followed for 284 months in remission. All but one immature teratomas were treated with surgery and all were under follow-up without disease. Two patients with embryonal carcinoma and malignant teratoma didn't undergo surgery; both received chemotherapy and radiotherapy but died with disease. The patient with teratocarcinoma was treated with surgery and chemotherapy but died with disease. Conclusions: No adjuvant therapy is needed for mature teratomas. Immature teratomas must be under close follow-up for recurrences. Prognosis for mediastinal malignant GCTs was poor. These cases need intensive chemotherapies and effective local control measures as surgery/radiotherapy to ensure long-term survival. © 2012 Informa Healthcare USA, Inc.