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Germ cell tumors (GCTs), as their name implies, originate from germ cells. Such tumors can arise from gonads, as well as in extragonadal sites. Extragonadal GCTs are most often located in the midline and are typically found in the retroperi-toneum, sacrococcygeal region, anterior mediastinum, or intracranial areas (usually in the pituitary region). Less commonly, GCTs are found in other regions, such as the neck, extremities, or internal organs (Table 4-1).

Germ cell tumors of the gonads have essentially the same microscopic features as those found in other locations. With some notable exceptions, all of these tumors can be classified according to the same principles, suggesting that all of them share the same biology and are most likely derived from germ cells in an identical manner. Unfortunately, despite considerable progress, our understanding of the basic aspects of the pathogenesis of GCT remains incomplete, and the crucial events leading to tumor formation remain obscure. This chapter will briefly reviews the current views of the histogenesis of GCTs in general, including the experimental data supporting various theories of carcinogenesis. The pathology of the most common forms of germ cell neoplasia in humans is then reviewed.

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