A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course
| العنوان: | A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course |
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| المؤلفون: | Masamichi Kurosaki, Tetsuji Uno, Kohei Shomori, Atsushi Kambe, Shinji Kondo, Yuichiro Nagao, Satoko Nakada, Makoto Sakamoto, Michiharu Tanabe |
| المصدر: | Brain Tumor Pathology. 37:165-170 |
| بيانات النشر: | Springer Science and Business Media LLC, 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Male, Cancer Research, Pathology, medicine.medical_specialty, Solitary fibrous tumor, Radiosurgery, Neurosurgical Procedures, 03 medical and health sciences, Fatal Outcome, Rare Diseases, 0302 clinical medicine, medicine, Humans, Dedifferentiated Solitary Fibrous Tumor, Aged, Aged, 80 and over, Hemangiopericytoma, Osteosarcoma, Brain Neoplasms, business.industry, Clinical course, Neoplasms, Second Primary, Histology, Cytoreduction Surgical Procedures, General Medicine, Cell Dedifferentiation, medicine.disease, Repressor Proteins, Oncology, Tumor progression, Solitary Fibrous Tumors, 030220 oncology & carcinogenesis, Disease Progression, Neurology (clinical), Neurosurgery, Gene Fusion, STAT6 Transcription Factor, business, 030217 neurology & neurosurgery |
| الوصف: | 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. |
| تدمد: | 1861-387X 1433-7398 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e17945682e9d5276938da8f075e1b5bdTest https://doi.org/10.1007/s10014-020-00374-yTest |
| حقوق: | CLOSED |
| رقم الانضمام: | edsair.doi.dedup.....e17945682e9d5276938da8f075e1b5bd |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 1861387X 14337398 |
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