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Presented by Pedram Argani, M.D. and prepared by Zarir E. Karanjawala, M.D., Ph.D.
Case 3: An 11 year old male with a periventricular brain tumor.
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1. Question
Week 316: Case 3
An 11 year old male with a periventricular brain tumor/images/argani070907_3a.jpg
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Answer: Dysgerminoma & Yolk sac tumor
Histology: This lesion has biphasic morphology. One component of the lesion is composed of epithelioid cells with round nuclear contours and prominent nucleoli, associated with a dense lymphoid infiltrate. These are the typical features of a dysgerminoma. The second component of this lesion has higher cytologic grade, in that the nuclei are more irregular and hyperchromatic. This component has variable architectural patterns, including microcystic and pseudopapillary. The former component of this biphasic neoplasm labeled for OCT4 and C-kit, whereas the latter labeled for cytokeratins and alpha-feta protein. These results support the diagnosis of mixed germ cell tumor.
Discussion: The absence of OCT4 labeling in the more pleomorphic component of this tumor, along with the alpha-fetoprotein immunoreactivity, argue against embryonal carcinoma. The biphasic nature of the proliferation argues for a mixed cell germ tumor. While dysgerminomas of the central nervous system are highly responsive to radiation therapy, the presence of a non-dysgerminomatous component diminishes the prognosis significantly.
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Answer: Dysgerminoma & Yolk sac tumor
Histology: This lesion has biphasic morphology. One component of the lesion is composed of epithelioid cells with round nuclear contours and prominent nucleoli, associated with a dense lymphoid infiltrate. These are the typical features of a dysgerminoma. The second component of this lesion has higher cytologic grade, in that the nuclei are more irregular and hyperchromatic. This component has variable architectural patterns, including microcystic and pseudopapillary. The former component of this biphasic neoplasm labeled for OCT4 and C-kit, whereas the latter labeled for cytokeratins and alpha-feta protein. These results support the diagnosis of mixed germ cell tumor.
Discussion: The absence of OCT4 labeling in the more pleomorphic component of this tumor, along with the alpha-fetoprotein immunoreactivity, argue against embryonal carcinoma. The biphasic nature of the proliferation argues for a mixed cell germ tumor. While dysgerminomas of the central nervous system are highly responsive to radiation therapy, the presence of a non-dysgerminomatous component diminishes the prognosis significantly.
