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- W2783107411 abstract "Extragonadal germ cell tumors are tumors that account for 20% of mediastinal masses. Their histogenesis is under dispute, but their cellular origin is favored to be from an extragonadal germ cell. In this case, we report a rare extragonadal germ cell neoplasm with mature and immature teratomatous elements in association with malignant epithelial and mesenchymal somatic components. A 22-year-old man presented with a rapidly growing mediastinal mass that was unresponsive to chemotherapy. The mass was pushing on the heart and airway with complete collapse of the left lung. Serum alpha-fetoprotein was 260 ng/mL. He underwent surgical resection of the tumor. Histologically, the tumor demonstrated an extensively viable extra-gonadal germ cell neoplasm composed of mature and immature teratomatous elements in association with sarcomatous and carcinomatous components. The malignant somatic elements were intermixed with the teratomatous elements. Immunohistochemistry for cytokeratin and desmin was positive in addition to P53 over expression. SOX-2 immunohistochemistry was useful by highlighting the immature nonseminomatous components. Cytogenetics revealed 48, XY, +1, +21 and 49, XY, +1, +21 + 22 karyotypes. Nonseminomatous extra-gonadal germ cell tumors (NSEGCTs) of the mediastinum carry a significantly worse prognosis than teratomas with a five-year overall survival of 40%-45%. When teratomas present with overtly malignant germ cell components, they should be classified as malignant mixed germ cell tumors. Due to the pluripotency associated with SOX-2 expression, it may explain the malignant sarcomatous and carcinomatous somatic components in our case. Teratomas with malignant transformation require complete surgical resection for disease cure, whereas NSEGCTs require surgical resection and may be candidates for chemotherapy. The distinction between teratoma with immature and malignant germ cell layers from NSEGCTs with malignant teratomatous components can be diagnostically challenging but may have an impact on prognosis as well as patient management." @default.
- W2783107411 created "2018-01-26" @default.
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- W2783107411 date "2018-01-01" @default.
- W2783107411 modified "2023-09-26" @default.
- W2783107411 title "248 Nonseminomatous Extragonadal Germ Cell Tumor With Teratomatous and Multiple Malignant Somatic Components: A Rare Case Report" @default.
- W2783107411 doi "https://doi.org/10.1093/ajcp/aqx123.247" @default.
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