Extragonadal Primary Mixed Germ Cell Tumor of the Stomach

Germ cell tumors (GCT) are most commonly observed in the gonads. Extragonadal GCTs (EGCT) are uncommon and are considered metastatic or primary tumors. Histologically and immunophenotypically, EGCTs resemble gonadal GCT. EGCTs in the gastrointestinal tract are extremely rare and are seen more frequently as metastases than primary tumors. Here, we discuss a case of primary mixed GCT of the stomach in a 4-year-old boy to highlight the fact that GCT should be considered in the differential diagnosis of any poorly differentiated epithelioid neoplasm in the pediatric population. The site should not hinder one from thinking of it. Histomorphological appearances, supporting immunohistochemistry, and tumor markers can help in establishing the correct diagnosis and ensuring the appropriate management of patients.