Germ cell tumors: Pathology and genetics

Germ cell tumors (GCT) are a heterogeneous family of gonadal and extragonadal tumors derived from germ cells, and their precursors: primordial germ cells (PGC) and gonocytes. The wide range of clinical manifestations of GCT is due to the different developmental potential and anatomical sites of the originating cells. Consistent with most GCT being caused by disturbed development, genetic changes are usually not the initiators of neoplastic transformation, but rather progression-related. Familial occurrence and multiplicity of GCT is likely explained by predisposing genetic factors interacting with environmental cues. Germ cell- and embryonic characteristics of DNA-repair of the tumor stem cells explain the exquisite sensitivity of GCT for DNA-damaging agents.