NAB2-STAT6 gene fusion and STAT6 immunoexpression in extrathoracic solitary fibrous tumors: The association between fusion variants and locations

I. Chieh Chuang, Kuan Cho Liao, Hsuan Ying Huang, Yu-Chien Kao, Chien Feng Li, Shih Chiang Huang, Jen Wei Tsai, Ko Chin Chen, Jui Lan, Po Chun Lin

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm harboring NAB2-STAT6 fusion, which drives STAT6 nuclear relocation. For extrathoracic SFTs, the clinical relevance of this molecular hallmark remains obscure. We assessed STAT6 immunoexpression for 61 extrathoracic SFTs exclusive of the meninges and head and neck, and 25 had analyzable RNAs to distinguish fusion variants by RT-PCR. The immunohistochemical and molecular findings were correlated with clincopathological features and disease-free survival (DFS). Twenty-eight males and 33 females had SFTs in the body cavities (n=31), extremities (n=17), and trunk (n=13), categorized into 53 non-malignant and 8 malignant tumors. The vast majority (n=57, 93%) exhibited distinctive STAT6 nuclear expression, including malignant ones. The common fusion variants were NAB2ex6-STAT6ex16/17 in 13 SFTs and NAB2ex4-STAT6ex2 in 8, while miscellaneous variants were detected only in 4 SFTs in the limbs and trunk but not in any body cavity-based cases (P=0.026). The worse DFS was univariately associated with malignant histology (P=0.04) but unrelated to tumor size, location, or fusion variant. Conclusively, extrathoracic SFTs mostly harbor NAB2ex6-STAT6ex16/17, followed by NAB2ex4-STAT6ex2. Miscellaneous variants are significantly rare in SFTs within the body cavities. The clinical aggressiveness of extrathoraic SFTs is associated with malignant histology but unrelated to the NAB2-STAT6 fusion variants.

Original languageEnglish
Pages (from-to)288-296
Number of pages9
JournalPathology International
Volume66
Issue number5
DOIs
Publication statusPublished - May 1 2016

Fingerprint

Solitary Fibrous Tumors
Gene Fusion
Disease-Free Survival
Histology
Extremities
Meninges
Neoplasms
Neck
Head
RNA
Polymerase Chain Reaction

Keywords

  • Extrathoracic
  • NAB2-STAT6
  • Solitary fibrous tumor

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

NAB2-STAT6 gene fusion and STAT6 immunoexpression in extrathoracic solitary fibrous tumors : The association between fusion variants and locations. / Chuang, I. Chieh; Liao, Kuan Cho; Huang, Hsuan Ying; Kao, Yu-Chien; Li, Chien Feng; Huang, Shih Chiang; Tsai, Jen Wei; Chen, Ko Chin; Lan, Jui; Lin, Po Chun.

In: Pathology International, Vol. 66, No. 5, 01.05.2016, p. 288-296.

Research output: Contribution to journalArticle

Chuang, I. Chieh ; Liao, Kuan Cho ; Huang, Hsuan Ying ; Kao, Yu-Chien ; Li, Chien Feng ; Huang, Shih Chiang ; Tsai, Jen Wei ; Chen, Ko Chin ; Lan, Jui ; Lin, Po Chun. / NAB2-STAT6 gene fusion and STAT6 immunoexpression in extrathoracic solitary fibrous tumors : The association between fusion variants and locations. In: Pathology International. 2016 ; Vol. 66, No. 5. pp. 288-296.
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abstract = "Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm harboring NAB2-STAT6 fusion, which drives STAT6 nuclear relocation. For extrathoracic SFTs, the clinical relevance of this molecular hallmark remains obscure. We assessed STAT6 immunoexpression for 61 extrathoracic SFTs exclusive of the meninges and head and neck, and 25 had analyzable RNAs to distinguish fusion variants by RT-PCR. The immunohistochemical and molecular findings were correlated with clincopathological features and disease-free survival (DFS). Twenty-eight males and 33 females had SFTs in the body cavities (n=31), extremities (n=17), and trunk (n=13), categorized into 53 non-malignant and 8 malignant tumors. The vast majority (n=57, 93{\%}) exhibited distinctive STAT6 nuclear expression, including malignant ones. The common fusion variants were NAB2ex6-STAT6ex16/17 in 13 SFTs and NAB2ex4-STAT6ex2 in 8, while miscellaneous variants were detected only in 4 SFTs in the limbs and trunk but not in any body cavity-based cases (P=0.026). The worse DFS was univariately associated with malignant histology (P=0.04) but unrelated to tumor size, location, or fusion variant. Conclusively, extrathoracic SFTs mostly harbor NAB2ex6-STAT6ex16/17, followed by NAB2ex4-STAT6ex2. Miscellaneous variants are significantly rare in SFTs within the body cavities. The clinical aggressiveness of extrathoraic SFTs is associated with malignant histology but unrelated to the NAB2-STAT6 fusion variants.",
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AU - Huang, Hsuan Ying

AU - Kao, Yu-Chien

AU - Li, Chien Feng

AU - Huang, Shih Chiang

AU - Tsai, Jen Wei

AU - Chen, Ko Chin

AU - Lan, Jui

AU - Lin, Po Chun

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