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- Title
Clinicopathological review of solitary fibrous tumors: dedifferentiation is a major cause of patient death.
- Authors
Yamada, Yuichi; Kohashi, Kenichi; Kinoshita, Izumi; Yamamoto, Hidetaka; Iwasaki, Takeshi; Yoshimoto, Masato; Ishihara, Shin; Toda, Yu; Itou, Yoshihiro; Koga, Yutaka; Hashisako, Mikiko; Nozaki, Yui; Kiyozawa, Daisuke; Kitahara, Daichi; Inoue, Takeshi; Mukai, Munenori; Honda, Yumi; Toyokawa, Gouji; Tsuchihashi, Kenji; Matsushita, Yoshifumi
- Abstract
Solitary fibrous tumor (SFT) is a soft-tissue neoplasm of intermediate malignant potential, presenting a wide histopathological spectrum. Poorer prognosis of hemangiopericytoma of the central nervous system (CNS), hypoglycemic SFT, and dedifferentiation are well-known characters of SFT, but their clinical significance were not demonstrated enough by large-sized study. Here, the clinicopathological features of SFTs are reviewed and the relationship between genetics and clinicopathological features is examined using 145 SFT cases. All cases were STAT6 IHC-positive and/or NAB2-STAT6 fusion gene-positive. Tumor location was classified into three categories: 30 pleuropulmonary, 96 non-pleuropulmonary/non-central nervous system (CNS), and 18 CNS tumors. The tumor developed recurrence in 21 of 93 available cases (22.5%), metastasis in 11 of 93 (11.8%), and tumor death in 9 of 93 (9.6%). Hypoglycemia occurred in 2 primary tumors and 1 metastatic tumor among 63 reviewable cases, and dedifferentiation occurred in 10 cases (6.8%) including 6 primary tumors, 2 recurrent tumors, and 2 metastatic tumors. Recurrence was positively associated with CNS location (p = 0.0109) and hypoglycemia (p = 0.001); metastasis was positively associated with CNS location (p = 0.0231), hypoglycemia (p < 0.0001), and dedifferentiation (p < 0.0001), while metastasis was negatively correlated with pleural location (p = 0.0471). Tumor death was positively associated with male sex (p = 0.0154), larger size (p = 0.0455), hypoglycemia (p < 0.0001), and dedifferentiation (p < 0.0001). Multivariate analysis revealed independent statistical significance of dedifferentiation for overall survival (p = 0.0467). Exon variant of the fusion gene had no statistical correlation with clinical outcome. In conclusion, dedifferentiation is a major prognostic factor of SFT, and specific location such as cerebromeningeal and intra-abdominal site and hypoglycemia also had a high risk for unfavorable prognosis.
- Subjects
CAUSES of death; GENE fusion; CENTRAL nervous system; TUMORS; ARTIFICIAL pancreases
- Publication
Virchows Archiv: European Journal of Pathology, 2019, Vol 475, Issue 4, p467
- ISSN
0945-6317
- Publication type
journal article
- DOI
10.1007/s00428-019-02622-9