Clinicopathological and genetic heterogeneity of the head and neck solitary fibrous tumours

A comparative histological, immunohistochemical and molecular study of 36 cases

Yu-Chien Kao, Po Chun Lin, Shao Lun Yen, Shih Chiang Huang, Jen Wei Tsai, Chien Feng Li, Hui Chun Tai, Jui Lan, I. Chieh Chuang, Shih Chen Yu, Hsuan Ying Huang

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Aims: Solitary fibrous tumour (SFT) harbours recurrent inv12(q13q13)-derived NAB2-STAT6 fusions, resulting in STAT6 nuclear expression. SFTs affecting the head and neck are rare, for which we reported their clinicopathological, immunohistochemical, and genetic features. Methods and results: With 19 cases assessable for NAB2-STAT6 fusions, 36 head and neck SFTs (18 males; 18 females) diagnosed between 13 and 79 years (median, 47 years) of age were analysed for clinicopathological features and STAT6 immunoexpression. These SFTs, ranging from 5 to 80 mm (median, 25 mm), affected the oral cavity/pharynx (12), orbit (11), sinonasal structures (seven), and somatic soft tissues or skull (six). Histologically, 20 SFTs were conventional, six were giant-cell angiofibroma-like, one was fat-forming, four were cellular/atypical, and five were malignant (two developing metastases). STAT6 distinctively decorated the tumoral nuclei in 35 (97.2%) SFTs, but not in 29 site-relevant histological mimics categorized into 12 entities. Sixteen (84.2%) SFTs showed NAB2-STAT6 fusions with highly heterogeneous exon compositions, including NAB2ex6-STAT6ex17 in four cases, NAB2ex4-STAT6ex2 in three cases, NAB2ex2-STAT6ex2, NAB2ex4-STAT6ex4, NAB2ex6-STAT6ex16 and NAB2ex6-STAT6ex18 in two cases each, and NAB2ex3-STAT6ex19 in one case. Conclusions: Nuclear STAT6 immunoexpression is sensitive and specific for distinguishing SFT from mimics. However, considerable heterogeneity exists in the head and neck SFTs regarding the locations, histological patterns, and NAB2-STAT6 fusion variants.

Original languageEnglish
Pages (from-to)492-501
Number of pages10
JournalHistopathology
Volume68
Issue number4
DOIs
Publication statusPublished - Mar 1 2016

Fingerprint

Solitary Fibrous Tumors
Genetic Heterogeneity
Neck
Head
Angiofibroma
Orbit
Giant Cells
Pharynx
Skull
Mouth
Exons
Fats
Neoplasm Metastasis

Keywords

  • Head and neck
  • NAB2
  • RT-PCR
  • Solitary fibrous tumour
  • STAT6

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

Cite this

Clinicopathological and genetic heterogeneity of the head and neck solitary fibrous tumours : A comparative histological, immunohistochemical and molecular study of 36 cases. / Kao, Yu-Chien; Lin, Po Chun; Yen, Shao Lun; Huang, Shih Chiang; Tsai, Jen Wei; Li, Chien Feng; Tai, Hui Chun; Lan, Jui; Chuang, I. Chieh; Yu, Shih Chen; Huang, Hsuan Ying.

In: Histopathology, Vol. 68, No. 4, 01.03.2016, p. 492-501.

Research output: Contribution to journalArticle

Kao, Yu-Chien ; Lin, Po Chun ; Yen, Shao Lun ; Huang, Shih Chiang ; Tsai, Jen Wei ; Li, Chien Feng ; Tai, Hui Chun ; Lan, Jui ; Chuang, I. Chieh ; Yu, Shih Chen ; Huang, Hsuan Ying. / Clinicopathological and genetic heterogeneity of the head and neck solitary fibrous tumours : A comparative histological, immunohistochemical and molecular study of 36 cases. In: Histopathology. 2016 ; Vol. 68, No. 4. pp. 492-501.
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abstract = "Aims: Solitary fibrous tumour (SFT) harbours recurrent inv12(q13q13)-derived NAB2-STAT6 fusions, resulting in STAT6 nuclear expression. SFTs affecting the head and neck are rare, for which we reported their clinicopathological, immunohistochemical, and genetic features. Methods and results: With 19 cases assessable for NAB2-STAT6 fusions, 36 head and neck SFTs (18 males; 18 females) diagnosed between 13 and 79 years (median, 47 years) of age were analysed for clinicopathological features and STAT6 immunoexpression. These SFTs, ranging from 5 to 80 mm (median, 25 mm), affected the oral cavity/pharynx (12), orbit (11), sinonasal structures (seven), and somatic soft tissues or skull (six). Histologically, 20 SFTs were conventional, six were giant-cell angiofibroma-like, one was fat-forming, four were cellular/atypical, and five were malignant (two developing metastases). STAT6 distinctively decorated the tumoral nuclei in 35 (97.2{\%}) SFTs, but not in 29 site-relevant histological mimics categorized into 12 entities. Sixteen (84.2{\%}) SFTs showed NAB2-STAT6 fusions with highly heterogeneous exon compositions, including NAB2ex6-STAT6ex17 in four cases, NAB2ex4-STAT6ex2 in three cases, NAB2ex2-STAT6ex2, NAB2ex4-STAT6ex4, NAB2ex6-STAT6ex16 and NAB2ex6-STAT6ex18 in two cases each, and NAB2ex3-STAT6ex19 in one case. Conclusions: Nuclear STAT6 immunoexpression is sensitive and specific for distinguishing SFT from mimics. However, considerable heterogeneity exists in the head and neck SFTs regarding the locations, histological patterns, and NAB2-STAT6 fusion variants.",
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T1 - Clinicopathological and genetic heterogeneity of the head and neck solitary fibrous tumours

T2 - A comparative histological, immunohistochemical and molecular study of 36 cases

AU - Kao, Yu-Chien

AU - Lin, Po Chun

AU - Yen, Shao Lun

AU - Huang, Shih Chiang

AU - Tsai, Jen Wei

AU - Li, Chien Feng

AU - Tai, Hui Chun

AU - Lan, Jui

AU - Chuang, I. Chieh

AU - Yu, Shih Chen

AU - Huang, Hsuan Ying

PY - 2016/3/1

Y1 - 2016/3/1

N2 - Aims: Solitary fibrous tumour (SFT) harbours recurrent inv12(q13q13)-derived NAB2-STAT6 fusions, resulting in STAT6 nuclear expression. SFTs affecting the head and neck are rare, for which we reported their clinicopathological, immunohistochemical, and genetic features. Methods and results: With 19 cases assessable for NAB2-STAT6 fusions, 36 head and neck SFTs (18 males; 18 females) diagnosed between 13 and 79 years (median, 47 years) of age were analysed for clinicopathological features and STAT6 immunoexpression. These SFTs, ranging from 5 to 80 mm (median, 25 mm), affected the oral cavity/pharynx (12), orbit (11), sinonasal structures (seven), and somatic soft tissues or skull (six). Histologically, 20 SFTs were conventional, six were giant-cell angiofibroma-like, one was fat-forming, four were cellular/atypical, and five were malignant (two developing metastases). STAT6 distinctively decorated the tumoral nuclei in 35 (97.2%) SFTs, but not in 29 site-relevant histological mimics categorized into 12 entities. Sixteen (84.2%) SFTs showed NAB2-STAT6 fusions with highly heterogeneous exon compositions, including NAB2ex6-STAT6ex17 in four cases, NAB2ex4-STAT6ex2 in three cases, NAB2ex2-STAT6ex2, NAB2ex4-STAT6ex4, NAB2ex6-STAT6ex16 and NAB2ex6-STAT6ex18 in two cases each, and NAB2ex3-STAT6ex19 in one case. Conclusions: Nuclear STAT6 immunoexpression is sensitive and specific for distinguishing SFT from mimics. However, considerable heterogeneity exists in the head and neck SFTs regarding the locations, histological patterns, and NAB2-STAT6 fusion variants.

AB - Aims: Solitary fibrous tumour (SFT) harbours recurrent inv12(q13q13)-derived NAB2-STAT6 fusions, resulting in STAT6 nuclear expression. SFTs affecting the head and neck are rare, for which we reported their clinicopathological, immunohistochemical, and genetic features. Methods and results: With 19 cases assessable for NAB2-STAT6 fusions, 36 head and neck SFTs (18 males; 18 females) diagnosed between 13 and 79 years (median, 47 years) of age were analysed for clinicopathological features and STAT6 immunoexpression. These SFTs, ranging from 5 to 80 mm (median, 25 mm), affected the oral cavity/pharynx (12), orbit (11), sinonasal structures (seven), and somatic soft tissues or skull (six). Histologically, 20 SFTs were conventional, six were giant-cell angiofibroma-like, one was fat-forming, four were cellular/atypical, and five were malignant (two developing metastases). STAT6 distinctively decorated the tumoral nuclei in 35 (97.2%) SFTs, but not in 29 site-relevant histological mimics categorized into 12 entities. Sixteen (84.2%) SFTs showed NAB2-STAT6 fusions with highly heterogeneous exon compositions, including NAB2ex6-STAT6ex17 in four cases, NAB2ex4-STAT6ex2 in three cases, NAB2ex2-STAT6ex2, NAB2ex4-STAT6ex4, NAB2ex6-STAT6ex16 and NAB2ex6-STAT6ex18 in two cases each, and NAB2ex3-STAT6ex19 in one case. Conclusions: Nuclear STAT6 immunoexpression is sensitive and specific for distinguishing SFT from mimics. However, considerable heterogeneity exists in the head and neck SFTs regarding the locations, histological patterns, and NAB2-STAT6 fusion variants.

KW - Head and neck

KW - NAB2

KW - RT-PCR

KW - Solitary fibrous tumour

KW - STAT6

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