PMID- 31112350
OWN - NLM
STAT- MEDLINE
DCOM- 20200214
LR  - 20221207
IS  - 1098-2264 (Electronic)
IS  - 1045-2257 (Print)
IS  - 1045-2257 (Linking)
VI  - 58
IP  - 11
DP  - 2019 Nov
TI  - The histologic spectrum of soft tissue spindle cell tumors with NTRK3 gene 
      rearrangements.
PG  - 739-746
LID - 10.1002/gcc.22767 [doi]
AB  - NTRK3-rearranged tumors other than infantile fibrosarcomas (IFSs) harboring the 
      canonical ETV6-NTRK3 fusions are uncommon, and include mainly inflammatory 
      myofibroblastic tumors and gastrointestinal stromal tumors. Herein, we describe 
      an additional subset of seven tumors sharing NTRK3 gene rearrangements. The 
      cohort included five females and two males (age range 1-67 years). Tumors were 
      located in extremities, trunk, retroperitoneum, or intra-abdominal. In all 
      tumors, fluorescence in situ hybridization (FISH) revealed rearrangements in 
      NTRK3 accompanied by NTRK3 amplification in two cases. In three cases, RNA 
      sequencing identified a fusion transcript composed of NTRK3 exon 14 fused to 
      ETV6, TFG, and TPM4, respectively, retaining the NTRK3 kinase domain. All tumors 
      were positive for pan-TRK by immunohistochemistry (IHC). Two cases showed low- to 
      intermediate-grade histology composed of monomorphic spindle cells arranged in a 
      patternless architecture, stromal bands, and perivascular rings of hyalinized 
      collagen and coexpression of S100 and CD34. The remaining five cases were 
      high-grade fascicular monomorphic spindle cell sarcomas, morphologically somewhat 
      reminiscent of either malignant peripheral nerve sheath tumors (MPNSTs) or 
      fibrosarcomas (FSs). Two high-grade NTRK3 sarcomas showed aggressive clinical 
      behavior with development of lung metastases. Identification of high-grade 
      NTRK3-rearranged sarcomas is clinically important, since the development of 
      selective NTRK inhibitors has opened new avenues for targeted therapy. Although 
      IHC for pan-TRK can be applied as a screening tool, molecular studies are 
      recommended for a conclusive diagnosis of NTRK-rearranged neoplasms.
CI  - (c) 2019 Wiley Periodicals, Inc.
FAU - Suurmeijer, Albert J
AU  - Suurmeijer AJ
AD  - Department of Pathology, University Medical Center Groningen, University of 
      Groningen, Groningen, The Netherlands.
FAU - Dickson, Brendan C
AU  - Dickson BC
AUID- ORCID: 0000-0003-2269-6216
AD  - Department of Pathology & Laboratory Medicine, Mount Sinai Hospital, Toronto, 
      Ontario, Canada.
FAU - Swanson, David
AU  - Swanson D
AD  - Department of Pathology & Laboratory Medicine, Mount Sinai Hospital, Toronto, 
      Ontario, Canada.
FAU - Zhang, Lei
AU  - Zhang L
AD  - Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New 
      York.
FAU - Sung, Yun-Shao
AU  - Sung YS
AD  - Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New 
      York.
FAU - Huang, Hsuan-Ying
AU  - Huang HY
AD  - Department of Anatomical Pathology, Kaohsiung Chang Gung Memorial Hospital and 
      Chang Gung University, Kaohsiung City, Taiwan.
FAU - Fletcher, Christopher D
AU  - Fletcher CD
AD  - Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 
      Boston, Massachusetts.
FAU - Antonescu, Cristina R
AU  - Antonescu CR
AUID- ORCID: 0000-0002-9717-8205
AD  - Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New 
      York.
LA  - eng
GR  - P30 CA008748/CA/NCI NIH HHS/United States
GR  - P50 CA140146/CA/NCI NIH HHS/United States
GR  - P50 CA217694/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20190604
PL  - United States
TA  - Genes Chromosomes Cancer
JT  - Genes, chromosomes & cancer
JID - 9007329
RN  - 0 (Biomarkers, Tumor)
RN  - EC 2.7.10.1 (Receptor, trkC)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Biomarkers, Tumor/genetics
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Gene Fusion/genetics
MH  - Gene Rearrangement/genetics
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence/methods
MH  - Infant
MH  - Male
MH  - Middle Aged
MH  - Receptor, trkC/*genetics/metabolism
MH  - Sarcoma/*genetics/*pathology
MH  - Soft Tissue Neoplasms/genetics
MH  - Exome Sequencing
PMC - PMC6733642
MID - NIHMS1040149
OTO - NOTNLM
OT  - NTRK3
OT  - fibrosarcoma
OT  - fusion
OT  - malignant peripheral nerve sheath tumor
OT  - sarcoma
EDAT- 2019/05/22 06:00
MHDA- 2020/02/15 06:00
PMCR- 2020/11/01
CRDT- 2019/05/22 06:00
PHST- 2019/04/16 00:00 [received]
PHST- 2019/05/18 00:00 [revised]
PHST- 2019/05/20 00:00 [accepted]
PHST- 2019/05/22 06:00 [pubmed]
PHST- 2020/02/15 06:00 [medline]
PHST- 2019/05/22 06:00 [entrez]
PHST- 2020/11/01 00:00 [pmc-release]
AID - 10.1002/gcc.22767 [doi]
PST - ppublish
SO  - Genes Chromosomes Cancer. 2019 Nov;58(11):739-746. doi: 10.1002/gcc.22767. Epub 
      2019 Jun 4.