Mediastinal Germ Cell Tumors in Childhood

• Published in: Pediatric hematology and oncology 2012-10, Vol.29 (7), p.633-642
• Main Authors: Yalç n, Bilgehan, Demir, Hac Ahmet, Tanyel, Feridun Cahit, Akçören, Zühal, Varan, Ali, Akyüz, Canan, Kutluk, Tezer, Büyükpamukçu, Münevver
• Format: Article
• Language: English
• Subjects: Adolescent; Child; Child, Preschool; children; Female; Follow-Up Studies; germ cell tumor; Humans; Infant; Male; malignant; Mediastinal Neoplasms - diagnosis; Mediastinal Neoplasms - therapy; mediastinum; Neoplasms, Germ Cell and Embryonal - diagnosis; Neoplasms, Germ Cell and Embryonal - therapy; nonseminomatous; Prognosis; Remission Induction; Retrospective Studies; teratoma; Treatment Outcome
• ISSN: 0888-0018; 1521-0669
• DOI: 10.3109/08880018.2012.713084

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.