A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal
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CASE REPORT
A dedifferentiated intracranial solitary fibrous tumor with osteosarcoma components: rapid tumor progression and lethal clinical course Atsushi Kambe1 · Satoko Nakada2 · Yuichiro Nagao1 · Tetsuji Uno1 · Makoto Sakamoto1 · Kohei Shomori3 · Michiharu Tanabe4 · Shinji Kondo4 · Masamichi Kurosaki1 Received: 3 May 2020 / Accepted: 27 July 2020 © The Japan Society of Brain Tumor Pathology 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. Keywords Dedifferentiation · Solitary fibrous tumor · Hemangiopericytoma · Intracranial · Osteosarcoma
Introduction Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that primarily originates in the visceral pleura or at any site in the soft tissue of the head and neck, including the orbital walls, nasal cavities, and thyroid and parotid glands [1]. In the central nervous system, SFTs usually originate from the cranial and spinal meninges [2]. Recently, the NAB2–STAT6 fusion gene was identified as a driver mutation of SFTs [3, 4], and the nuclear expression of STAT6 protein, detected by immunohistochemistry, was established as an * Atsushi Kambe kanimo@tottori‑u.ac.jp 1
Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, 36‑1 Nishi‑cho, Yonago, Tottori 683‑8504, Japan
2
Department of Diagnostic Pathology, Kanazawa University Hospital, Kanazawa, Ishikawa 920‑8641, Japan
3
Department of Diagnostic Pathology, San-in Rosai Hospital, Yonago, Tottori 683‑8605, Japan
4
Department of Neurosurgery, San-in Rosai Hospital, Yonago, Tottori 683‑8605, Japan
alternative marker for this gene [5]. Based on the identification of the novel NAB2–STAT6 fusion gene, SFT and hemangiopericytoma (HPC) were reclassified as a new combined entity for which a soft-tissue–type grading system from 1 to 3 was introduced in the 2016 World Health Organization (WHO) classification of the tumors of central nervous system [6]. The majority of SFT/HPC cases is pathologically benign and demonstrate a favorable clinical course; however, approximately 5% of cases have a dismal clinical course with local recurrence or distant metastasis [7]. Malignant morphological changes such as increased mitosis, nuclear atypia
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