Department of Pathology, National University Health System, 5 Lower Kent Ridge Road, 119074, Singapore, Singapore.
Division of Neurosurgery, Department of Surgery, Ng Teng Fong General Hospital, Singapore, Singapore.
Brain Tumor Pathol. 2019 Jan;36(1):20-26. doi: 10.1007/s10014-018-0333-0. Epub 2019 Jan 2.
Solitary fibrous tumors/ hemangiopericytomas (SFT/HPC) are mesenchymal tumors that share a common genetic aberration and very rarely undergo dedifferentiation. We report a unique case of an intracranial anaplastic SFT/HPC with de-novo dedifferentiation, which pursued a rapidly fatal clinical course in a 41-year-old lady. The dedifferentiated component comprised a focal area of glandular formation with epithelial immunophenotype acquisition. The distinct biphasic pattern of the tumor imparted great diagnostic challenges to the pathologists. An increased awareness of SFT/HPCs with a diverse morphologic spectrum or even a biphasic histologic pattern is essential in working up such cases. We first attempted gamma knife radiosurgery in treating a recurrent dedifferentiated SFT/HPC; unfortunately it was to no avail. Although it is now known that SFT/HPC is characterized by NAB2-STAT6 gene fusion, the unavailability of targeted therapy against this molecular signature still results in a treatment dilemma.
孤立性纤维瘤/血管外皮细胞瘤（SFT/HPC）是一种间叶性肿瘤，具有共同的遗传异常，很少发生去分化。我们报告了一例颅内间变性 SFT/HPC 的去分化，这例 41 岁的女性患者疾病进展迅速，临床预后极差。去分化的部分包括局灶性腺体形成区域，上皮免疫表型获得。肿瘤的明显双相模式给病理学家带来了巨大的诊断挑战。在处理此类病例时，提高对具有不同形态谱甚至双相组织学模式的 SFT/HPC 的认识至关重要。我们首先尝试用伽玛刀放射外科手术治疗复发性去分化 SFT/HPC；但不幸的是，这并没有效果。尽管现在已经知道 SFT/HPC 的特征是 NAB2-STAT6 基因融合，但针对这种分子特征的靶向治疗仍无法获得，这导致了治疗困境。
