Mediastinal Germ Cell Tumors in Childhood


YALÇIN B. , DEMIR H. A. , TANYEL F. C. , AKÇÖREN Z. , VARAN A. , AKYÜZ C. , ...Daha Fazla

PEDIATRIC HEMATOLOGY AND ONCOLOGY, cilt.29, sa.7, ss.633-642, 2012 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 29 Konu: 7
  • Basım Tarihi: 2012
  • Doi Numarası: 10.3109/08880018.2012.713084
  • Dergi Adı: PEDIATRIC HEMATOLOGY AND ONCOLOGY
  • Sayfa Sayıları: ss.633-642

Özet

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.