Rare Tumors In Children and Adolescents

Part of the series Pediatric Oncology pp 205-211


Mediastinal Germ Cell Tumors

  • Dominik T. SchneiderAffiliated withKlinik für Kinder- und Jugendmedizin, Clinic of Pediatrics Email author 
  • , Giovanni CecchettoAffiliated withDepartment of Pediatrics, Pediatric Surgery, University of Padua
  • , Thomas A. OlsonAffiliated withDivision Pediatric Hematology/Oncology, Children’s Healthcare of Atlanta at Egleston

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The general aspects of germ cell tumors common to all germ cell tumors occurring at different sites are discussed in the chapters of gonadal germ cell tumors (see Chap. 39). In the thorax, germ cell tumors almost exclusively develop in midline locations, mostly in the anterior mediastinum. Therefore, it has been debated whether such extragonadal germ cell tumors, in particular teratomas may originate from midline somatic stem cells. On the other hand, there is molecular evidence that both gonadal and extragonadal germ cell tumors indeed originate from primordial germ cells at different stages of development. Thus, the examination of the epigenetic control of genomic imprinting reveals a methylation pattern that is characteristic of primordial germ cells during and shortly after their migration during early embryonal development (Schneider et al. 2001b; Bussey et al. 2001). In addition, this methylation pattern distinguishes germ cell tumors from other embryonal tumors with presumed stem cell origin such as nephroblastoma (Sievers et al. 2005), thus substantiating the hypothesis of their specific germ cell origin.