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A Case of Meningeal Solitary Fibrous Tumour/Haemangiopericytoma WHO Grade Ⅲ Metastasized to the Spleen Shortly After Tumor Resection Toshiyuki ENOMOTO 1,2 , Mikiko AOKI 1 , Hiromasa KOBAYASHI 2 , Hiroshi ABE 2 , Fuminori ISHII 3 , Yasushi YAMAUCHI 3 , Tooru INOUE 2 , Kazuki NABESHIMA 1 1Department of Pathology, Faculty of Medicine, Fukuoka University 2Department of Neurosurgery, Faculty of Medicine, Fukuoka University 3Department of Gastroenterological Surgery, Faculty of Medicine, Fukuoka University Keyword: solitary fibrous tumour , haemangiopericytoma , metastasis , spleen , NAB2-STAT6 pp.329-334
Published Date 2019/3/10
DOI https://doi.org/10.11477/mf.1436203938
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 Revision of WHO guidelines in 2016 led to the classification of solitary fibrous tumours(SFTs)and haemangiopericytomas(HPCs)as a single tumor entity characterized by NAB2-STAT6 fusion. Standard-of-care treatment involves surgery, but local recurrence and distant metastasis sometimes occur. The average latency to metastasis after surgery is 99 months.

 A 38-year-old female patient presented with a complaint of headache. An 8×5×2cm lesion showing Gd-T1 enhancement was detected near the superior sagittal sinus. Pathological assessment following resection revealed proliferating, polymorphic, atypical tumor cells with distinct nucleoli in a “patternless pattern.” Cellularity was moderate to high, and mitotic figures were observed in 15/10 high power fields. Immunohistochemically, tumor cells tested positive for STAT6, and RT-PCR revealed a NAB2-STAT6 fusion gene(exons 6 and 17, respectively), supporting a diagnosis of SFT/HPC WHO grade Ⅲ. Despite postoperative radiotherapy, multiple metastases to the spleen were detected 8 months after surgery, and distal pancreatectomy with splenectomy was performed. The pathology of the splenic tumor was similar to that of the intracranial tumor. Recurrent disease in a lymph node was detected 1 month later, and local radiation therapy was administered. The patient died of cancerous peritonitis 5 months later.

 In this case, exceedingly rapid metastasis to the spleen occurred, despite the administration of vigorous treatment. Here, we review SFT/HPC incidence, treatment, and outcomes to better understand this rare malignancy.


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電子版ISSN 1882-1251 印刷版ISSN 0301-2603 医学書院

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