Robins Shannon M, Rosenblatt Mary, Bruce Jeffrey N, Canoll Peter, Zanazzi George
Geisel School of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH, United States.
Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, NY, United States.
J Neuropathol Exp Neurol. 2025 May 1;84(5):379-390. doi: 10.1093/jnen/nlaf008.
Solitary fibrous tumors (SFTs) are fibroblastic mesenchymal neoplasms defined by the presence of a NAB2::STAT6 fusion and exhibit a broad range of behaviors. SFTs in the pineal region are poorly understood due to the limited number of reported cases. Here, we report a 48-year-old woman with a pineal region SFT who subsequently developed metastatic left para-falcine parieto-occipital and right lung upper lobe SFTs over the next 12 years. This was the only pineal SFT identified in an institutional cohort of 34 resected pineal region lesions. Review of another, much larger institutional cohort of pineal region lesions revealed 4 additional patients with SFT but none with extracranial metastasis. We present descriptions of their clinical presentations, treatments, histopathologic findings, available genomic alterations, and longitudinal outcomes. Finally, we performed a comprehensive literature search and identified 19 individual patients with pineal region SFTs. None of these reported neoplasms had an extracranial metastasis. Taken together, this work contributes to the growing body of data characterizing this rare tumor with aggressive potential and reinforces SFTs as a possible differential diagnosis for pineal region tumors.
孤立性纤维性肿瘤（SFTs）是由NAB2::STAT6融合基因定义的成纤维细胞间叶性肿瘤，表现出广泛的行为特征。由于报道的病例数量有限，松果体区的SFTs了解甚少。在此，我们报告一名48岁患有松果体区SFT的女性，在接下来的12年中，其左旁矢状窦顶枕部和右肺上叶随后出现了转移性SFT。这是在一个包含34例切除的松果体区病变的机构队列中唯一确诊的松果体SFT。回顾另一个更大的机构性松果体区病变队列，发现另外4例SFT患者，但均无颅外转移。我们介绍了他们的临床表现、治疗方法、组织病理学发现、可用的基因组改变以及长期预后。最后，我们进行了全面的文献检索，确定了19例患有松果体区SFT的个体患者。这些报道的肿瘤均无颅外转移。综上所述，这项工作为越来越多描述这种具有侵袭潜能的罕见肿瘤的数据做出了贡献，并强化了SFTs作为松果体区肿瘤可能的鉴别诊断。
