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Pediatr Hematol Oncol. 2012 Oct;29(7):633-42. Epub 2012 Aug 9.

Mediastinal germ cell tumors in childhood.

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1
Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey.

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.

PMID:
22877235
DOI:
10.3109/08880018.2012.713084
[Indexed for MEDLINE]
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