TY - JOUR
T1 - Clinicopathological review of solitary fibrous tumors
T2 - dedifferentiation is a major cause of patient death
AU - Yamada, Yuichi
AU - Kohashi, Kenichi
AU - Kinoshita, Izumi
AU - Yamamoto, Hidetaka
AU - Iwasaki, Takeshi
AU - Yoshimoto, Masato
AU - Ishihara, Shin
AU - Toda, Yu
AU - Itou, Yoshihiro
AU - Koga, Yutaka
AU - Hashisako, Mikiko
AU - Nozaki, Yui
AU - Kiyozawa, Daisuke
AU - Kitahara, Daichi
AU - Inoue, Takeshi
AU - Mukai, Munenori
AU - Honda, Yumi
AU - Toyokawa, Gouji
AU - Tsuchihashi, Kenji
AU - Matsushita, Yoshifumi
AU - Fushimi, Fumiyoshi
AU - Taguchi, Kenichi
AU - Tamiya, Sadafumi
AU - Oshiro, Yumi
AU - Furue, Masutaka
AU - Nakashima, Yasuharu
AU - Suzuki, Satoshi
AU - Iwaki, Toru
AU - Oda, Yoshinao
N1 - Funding Information:
This study was supported by a JSPS KAKEN Grant (No. 17K15645) and by funds from the Scholarship Program of the Takeda Science Foundation.
Funding Information:
Technical support for the experimental trials was provided by the following laboratory assistants: Motoko Tomita, Mami Nakamizo, Juri Godo, Miwako Ishii, Hisami Matsumoto, and Noriko Aoki. We also appreciate the technical assistance from staff at The Research Support Center, Kyushu University Graduate School of Medical Sciences.
Publisher Copyright:
© 2019, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2019/10/1
Y1 - 2019/10/1
N2 - 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.
AB - 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.
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U2 - 10.1007/s00428-019-02622-9
DO - 10.1007/s00428-019-02622-9
M3 - Article
C2 - 31392468
AN - SCOPUS:85070213177
VL - 475
SP - 467
EP - 477
JO - Virchows Archiv
JF - Virchows Archiv
SN - 0945-6317
IS - 4
ER -