2019
DOI: 10.1097/pas.0000000000001437
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Uterine Cervical Sarcoma With a Novel RET-SPECC1L Fusion in an Adult

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Cited by 24 publications
(15 citation statements)
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“…A subset of NTRK -rearranged soft tissue tumors show some overlapping histologic characteristics with NTRK sarcomas of the uterus (fascicular spindle cell proliferations with stromal and perivascular hyalinization and S100/CD34 expression),27 however, the relationship between the 2 remains unclear. Similarly, some spindle cell uterine tumors with fibrosarcoma-like morphology harbor targetable non- NTRK fusions, including COL1A1::PDGFB , FGFR1::TACC1 , and SPECC1L::RET 3,4,38. The relationship between these tumors and those that are driven by NTRK fusions remains to be fully elucidated, but all of these tumor types may ultimately fall under the same broad diagnostic category of “fibrosarcoma-like uterine sarcomas.” Additional studies examining their morphology, clinical behavior, and molecular characteristics (eg, methylation profiles) will be helpful to classify them and better understand how they are related.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…A subset of NTRK -rearranged soft tissue tumors show some overlapping histologic characteristics with NTRK sarcomas of the uterus (fascicular spindle cell proliferations with stromal and perivascular hyalinization and S100/CD34 expression),27 however, the relationship between the 2 remains unclear. Similarly, some spindle cell uterine tumors with fibrosarcoma-like morphology harbor targetable non- NTRK fusions, including COL1A1::PDGFB , FGFR1::TACC1 , and SPECC1L::RET 3,4,38. The relationship between these tumors and those that are driven by NTRK fusions remains to be fully elucidated, but all of these tumor types may ultimately fall under the same broad diagnostic category of “fibrosarcoma-like uterine sarcomas.” Additional studies examining their morphology, clinical behavior, and molecular characteristics (eg, methylation profiles) will be helpful to classify them and better understand how they are related.…”
Section: Discussionmentioning
confidence: 99%
“…Similarly, some spindle cell uterine tumors with fibrosarcoma-like morphology harbor targetable non-NTRK fusions, including COL1A1::PDGFB, FGFR1:: TACC1, and SPECC1L::RET. 3,4,38 The relationship between these tumors and those that are driven by NTRK fusions remains to be fully elucidated, but all of these tumor types may ultimately fall under the same broad diagnostic category of "fibrosarcoma-like uterine sarcomas." Additional studies examining their morphology, clinical behavior, and molecular characteristics (eg, methylation profiles) will be helpful to classify them and better understand how they are related.…”
Section: Ntrk Fusions and Related Tumorsmentioning
confidence: 99%
“…Although mesenchymal tumors with kinase fusion were first described in superficial soft tissues,10 a wide range of anatomic sites, including deep soft tissue, thoracic cavity, gynecologic tract, and gastrointestinal tract, can be involved by these tumors 3,19,20,25–29. The HN region can occasionally be affected, however, to date, merely 9 HN cases have been reported as single case reports or small series, including 7 with NTRK1 fusion, and 1 each with NTRK3 fusion, and RET fusion 3,8,10,11,26,30.…”
mentioning
confidence: 99%
“…[21][22][23][24] Although mesenchymal tumors with kinase fusion were first described in superficial soft tissues, 10 a wide range of anatomic sites, including deep soft tissue, thoracic cavity, gynecologic tract, and gastrointestinal tract, can be involved by these tumors. 3,19,20,[25][26][27][28][29] The HN region can occasionally be affected, however, to date, merely 9 HN cases have been reported as single case reports or small series, including 7 with NTRK1 fusion, and 1 each with NTRK3 fusion, and RET fusion. 3,8,10,11,26,30 We herein describe the clinicopathologic features of a large cohort of 15 cases with HN mesenchymal tumors harboring kinase fusions, including 3 cases of IFS with canonical ETV6:: NTRK3 fusion, 1 IFS-like tumor with RBPMS::MET fusions, and 12 other mesenchymal tumors with NTRK1, NTRK3, BRAF, or RET gene rearrangements.…”
mentioning
confidence: 99%
“…RET fusion positive neoplasms may also exhibit fibroblastic or neural-like differentiation and have phenotypic overlap with NTRK-related neoplasms (99). Recently a cervical sarcoma with a novel RET-SPECC1L fusion has been described (100). Rare spindle cell sarcomas with recurrent MEIS1-NCOA2 fusions have also been recently described (101).…”
Section: S51 Iccr Reporting Dataset:uterine Mesenchymal Tumorsmentioning
confidence: 99%