Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, 36-1 Nishi-cho, Yonago, Tottori, 683-8504, Japan.
Department of Diagnostic Pathology, Kanazawa University Hospital, Kanazawa, Ishikawa, 920-8641, Japan.
Brain Tumor Pathol. 2020 Oct;37(4):165-170. doi: 10.1007/s10014-020-00374-y. Epub 2020 Aug 2.
Solitary fibrous tumor/hemangiopericytoma is a mesenchymal tumor that originates from a common NAB2-STAT6 fusion gene and is known to very rarely demonstrate dedifferentiation in the pattern of local recurrence or distant metastasis. Here we describe for the first time a rare case of intracranial dedifferentiated solitary fibrous tumor/hemangiopericytoma with osteosarcoma components that developed in an 84-year-old man after frequent gamma knife radiosurgery over a 14-year period. We performed tumor-debulking and gamma knife radiosurgery, but unfortunately the patient died shortly after the development of dedifferentiation. There is no established treatment for dedifferentiated cases due to the rare histology and limited published data, and therefore further accumulation of histological and genetic profiles is necessary to develop novel target gene therapies.
孤立性纤维瘤/血管外皮细胞瘤是一种间叶性肿瘤，源于常见的 NAB2-STAT6 融合基因，已知非常罕见地表现出局部复发或远处转移模式的去分化。在这里，我们首次描述了一例罕见的颅内去分化孤立性纤维瘤/血管外皮细胞瘤，伴骨肉瘤成分，发生于一名 84 岁男性，在 14 年期间频繁接受伽玛刀放射外科治疗后。我们进行了肿瘤切除术和伽玛刀放射外科治疗，但不幸的是，患者在发生去分化后不久死亡。由于罕见的组织学和有限的已发表数据，对于去分化病例没有既定的治疗方法，因此需要进一步积累组织学和遗传学特征，以开发新的靶向基因治疗方法。
