A rare case of testicular tumor in a 20-year-old male with Primitive Neuroectodermal Tumor (PNET) was reported. Imaging studies showed a large heterogenous mass in the right scrotal sac and a large retroperitoneal mass with metastasis in the lung and liver. Serum alpha fetoprotein (AFP) was markedly elevated with moderate increase in serum β-human chorionic gonadotropin (hCG) levels. After orchidectomy, a histological diagnosis of mixed germ cell tumor-teratoma with primitive neuroectodermal, embryonal, and yolk sac components was made. Some scattered embryoid bodies representative of primitive germ cell tumor were also present. Morphological diversity including PNET prompted the authors to report this case as PNET points toward a poor prognosis.
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