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Special Feature
April 2002

Pathological Case of the Month

Arch Pediatr Adolesc Med. 2002;156(4):406. doi:

Figure 1. Axial computed tomographic scan through the pelvis (with contrast enhancement) shows a large, multilobulated, heterogenously enhancing mass filling the lower pelvis and displacing bowel to the left (arrow) and indenting the posterior bladder (B) wall.

Figure 2. A, The characteristic Schiller-Duval bodies seen in yolk sac tumors, consisting of a central fibrovascular core surrounded by a rim of primitive epithelium (hematoxylin-eosin, original magnification ×200). B, Immunohistochemistry, using an anti–α-fetoprotein antibody reveals positive dark staining in many epithelial cells (immunoperoxidase with hematoxylin counterstain, original magnification ×200).

Sacrococcygeal teratomas account for 40% of all germ cell tumors and up to 78% of extragonadal germ cell tumors.1 The incidence of germ cell tumors in children in the United States is 2.4 cases per 1 million children, with a biphasic age distribution. The first peak occurs at 2 years of age and a second peak at 15 to 20 years of age.2 There are 3 distinct clinical presentations of germ cell tumors: (1) tumors of the adolescent testis and ovary; (2) extragonadal germ cell tumors of older children; and (3) tumors of infants and young children. The origin of extragonadal germ cell tumors is presumed to be either aberrantly migrated germ cells, or alternatively, totipotential embryonic cells. The most common locations for extragonadal germ cell tumors are sacrococcygeal, mediastinal (including the pericardium, heart, and lung), intracranial, and retroperitoneal, respectively.