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01-10-2020 | osteosarcoma | Case Report

A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course

Authors: Atsushi Kambe, Satoko Nakada, Yuichiro Nagao, Tetsuji Uno, Makoto Sakamoto, Kohei Shomori, Michiharu Tanabe, Shinji Kondo, Masamichi Kurosaki

Published in: Brain Tumor Pathology | Issue 4/2020

Abstract

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.

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Title: A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course
Authors: Atsushi Kambe
Satoko Nakada
Yuichiro Nagao
Tetsuji Uno
Makoto Sakamoto
Kohei Shomori
Michiharu Tanabe
Shinji Kondo
Masamichi Kurosaki
Publication date: 01-10-2020
Publisher: Springer Singapore
Keywords: osteosarcoma
Osteosarcoma
Published in: Brain Tumor Pathology / Issue 4/2020
Print ISSN: 1433-7398
Electronic ISSN: 1861-387X
DOI: https://doi.org/10.1007/s10014-020-00374-y

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A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course

Abstract

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