A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course
A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course
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DOI：
10.1007/s10014-020-00374-y
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发表时间：
2020-08
影响因子：
3.3
通讯作者：
A. Kambe;S. Nakada;Y. Nagao;T. Uno;M. Sakamoto;K. Shomori;M. Tanabe;S. Kondo;M. Kurosaki
中科院分区：
文献类型：
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作者：
A. Kambe;S. Nakada;Y. Nagao;T. Uno;M. Sakamoto;K. Shomori;M. Tanabe;S. Kondo;M. Kurosaki
孤立性纤维瘤/血管外皮细胞瘤是一种起源于常见的NAB2-STAT6融合基因的间叶性肿瘤，在局部复发或远处转移的模式中很少表现出去分化。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，伴有骨肉瘤成分，患者为84岁男性，在14年间频繁地接受伽玛刀放射外科治疗后发生。我们进行了肿瘤剥离和伽玛刀放射外科手术，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公开数据，目前还没有针对去分化病例的既定治疗方法，因此进一步积累组织学和遗传学特征对于开发新的靶基因疗法是必要的。
Solitary fibrous tumor/hemangiopericytoma is a mesenchymal tumor that originates from a commonNAB2–STAT6fusion 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.