Pediatric mediastinal germ cell tumors

Abstract

Mediastinal germ cell tumors (GCTs) are a rare and heterogeneous group of neoplasms. Although histologically resembling their gonadal counterparts, they differ considerably in their clinical characteristics, biological behavior and prognostic outcome. The rarity of mediastinal GCTs has hindered their meaningful analysis, with most studies and clinical trials including them along with other extragonadal GCTs, which has led to a lack of consensus on optimal treatment strategies, and a lull in improvement in patient outcomes. Diagnosis of mediastinal GCT requires a multipronged approach, and encompasses multidisciplinary treatment including chemotherapy followed by surgery, with or without radiotherapy. In view of sustained response rates to current management protocols, the focus needs to be shifted to identifying patients in whom treatment regimens can be downscaled with the aim of decreasing long term morbidity and improving quality of life in low risk patient groups, while improving survival rates in poor risk patient subsets. In this scenario, better understanding of the molecular pathogenesis of these tumors may lead to identification of novel biomarkers and therapeutic targets, as well as improved disease segmentation and risk stratification, thus helping to avoid the toxicity and morbidity associated with current one-fits-all treatment strategies. Multi-institutional collaborations across continents are necessary to generate meaningful data, and are the face of future developments in this arena.

Keywords: Mediastinum; adolescent; childhood; germ cell tumor (GCT); nonseminomatous; seminoma.