Immature neuroectodermal tissue can be found in the ovary as part of an immature teratoma or as part of a teratoma with malignant neuroectodermal transformation. Such lesions may closely resemble central nervous system tumors, but their biologic similarity is unclear. We describe an 18-yr-old female who presented with abdominal pain caused by an ovarian mass with widespread metastases. Histology showed a primitive, high-grade tumor arising in the background of a mature teratoma. The tumor was SOX10 positive, with focal expression of GFAP, S100, NSE, and synaptophysin. Molecular analysis demonstrated co-amplification of PDGFRA and KIT, alterations common in high-grade gliomas. By whole-genome methylation profiling, it clustered into the "diffuse pediatric-type high-grade glioma, RTK1 subtype, subclass c" group. Despite progressing through 2 lines of chemotherapy with widespread metastatic disease, she achieved an excellent response to chemotherapy directed toward aggressive germ cell tumors. This case emphasizes the importance of immunohistochemical, genomic, and epigenetic analyses to accurately classify these exceedingly rare tumors and determine the optimal therapy.

中文翻译：

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起源于卵巢成熟囊性畸胎瘤的弥漫性儿科型高级胶质瘤。

未成熟的神经外胚层组织可以作为未成熟畸胎瘤的一部分或作为具有恶性神经外胚层转化的畸胎瘤的一部分存在于卵巢中。此类病变可能与中枢神经系统肿瘤非常相似，但其生物学相似性尚不清楚。我们描述了一名 18 岁的女性，她因广泛转移的卵巢肿块引起腹痛。组织学显示在成熟畸胎瘤的背景下出现原始的、高级别肿瘤。肿瘤为 SOX10 阳性，具有 GFAP、S100、NSE 和突触素的局部表达。分子分析证明了 PDGFRA 和 KIT 的共同扩增，这是高级别神经胶质瘤中常见的改变。通过全基因组甲基化分析，它被聚类为“弥漫性儿科型高级别胶质瘤，RTK1 亚型，c 亚类”组。尽管通过 2 线化疗取得了广泛转移性疾病的进展，但她对针对侵袭性生殖细胞肿瘤的化疗取得了极好的反应。该病例强调了免疫组织化学、基因组和表观遗传分析对准确分类这些极为罕见的肿瘤并确定最佳治疗方法的重要性。