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TGFBR3 Gene Rearrangement

MedGen UID:
1791417
•Concept ID:
C5554976
•
Cell or Molecular Dysfunction
Synonyms: Betaglycan Gene Rearrangement; BGCAN Gene Rearrangement; Transforming Growth Factor Beta Receptor 3 Gene Rearrangement

Definition

A molecular abnormality indicating rearrangement of the TGFBR3 gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTGFBR3 Gene Rearrangement

Recent clinical studies

Etiology

Recurrent BRAF Gene Rearrangements in Myxoinflammatory Fibroblastic Sarcomas, but Not Hemosiderotic Fibrolipomatous Tumors.
Kao YC, Ranucci V, Zhang L, Sung YS, Athanasian EA, Swanson D, Dickson BC, Antonescu CR
Am J Surg Pathol 2017 Nov;41(11):1456-1465. doi: 10.1097/PAS.0000000000000899. PMID: 28692601Free PMC Article
TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms.
Carter JM, Sukov WR, Montgomery E, Goldblum JR, Billings SD, Fritchie KJ, Folpe AL
Am J Surg Pathol 2014 Sep;38(9):1182-992. doi: 10.1097/PAS.0000000000000212. PMID: 24705316

Diagnosis

Myxoinflammatory fibroblastic sarcoma: an immunohistochemical and molecular genetic study of 73 cases.
Suster D, Michal M, Huang H, Ronen S, Springborn S, Debiec-Rychter M, Billings SD, Goldblum JR, Rubin BP, Michal M, Suster S, Mackinnon AC
Mod Pathol 2020 Dec;33(12):2520-2533. Epub 2020 Jun 8 doi: 10.1038/s41379-020-0580-6. PMID: 32514165
The t(1;10)(p22;q24) TGFBR3/MGEA5 Translocation in Pleomorphic Hyalinizing Angiectatic Tumor, Myxoinflammatory Fibroblastic Sarcoma, and Hemosiderotic Fibrolipomatous Tumor.
Liu H, Sukov WR, Ro JY
Arch Pathol Lab Med 2019 Feb;143(2):212-221. Epub 2018 Jul 6 doi: 10.5858/arpa.2017-0412-RA. PMID: 29979612
TGFBR3 and MGEA5 rearrangements are much more common in "hybrid" hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcomas than in classical myxoinflammatory fibroblastic sarcomas: a morphological and fluorescence in situ hybridization study.
Zreik RT, Carter JM, Sukov WR, Ahrens WA, Fritchie KJ, Montgomery EA, Weiss SW, Folpe AL
Hum Pathol 2016 Jul;53:14-24. Epub 2016 Mar 2 doi: 10.1016/j.humpath.2016.02.005. PMID: 26980036
Myxoinflammatory fibroblastic sarcoma: morphologic and genetic updates.
Ieremia E, Thway K
Arch Pathol Lab Med 2014 Oct;138(10):1406-11. doi: 10.5858/arpa.2013-0549-RS. PMID: 25268202
TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms.
Carter JM, Sukov WR, Montgomery E, Goldblum JR, Billings SD, Fritchie KJ, Folpe AL
Am J Surg Pathol 2014 Sep;38(9):1182-992. doi: 10.1097/PAS.0000000000000212. PMID: 24705316

Prognosis

Expanding the clinicopathological spectrum of TGFBR3-PLAG1 rearranged salivary gland neoplasms with myoepithelial differentiation including evidence of high-grade transformation.
Rupp NJ, Höller S, Brada M, Vital D, Morand GB, Broglie MA, Huellner MW, Freiberger SN
Genes Chromosomes Cancer 2022 Feb;61(2):94-104. Epub 2021 Nov 17 doi: 10.1002/gcc.23009. PMID: 34755406Free PMC Article
TGFBR3 and MGEA5 rearrangements are much more common in "hybrid" hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcomas than in classical myxoinflammatory fibroblastic sarcomas: a morphological and fluorescence in situ hybridization study.
Zreik RT, Carter JM, Sukov WR, Ahrens WA, Fritchie KJ, Montgomery EA, Weiss SW, Folpe AL
Hum Pathol 2016 Jul;53:14-24. Epub 2016 Mar 2 doi: 10.1016/j.humpath.2016.02.005. PMID: 26980036
Myxoinflammatory fibroblastic sarcoma: morphologic and genetic updates.
Ieremia E, Thway K
Arch Pathol Lab Med 2014 Oct;138(10):1406-11. doi: 10.5858/arpa.2013-0549-RS. PMID: 25268202
TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms.
Carter JM, Sukov WR, Montgomery E, Goldblum JR, Billings SD, Fritchie KJ, Folpe AL
Am J Surg Pathol 2014 Sep;38(9):1182-992. doi: 10.1097/PAS.0000000000000212. PMID: 24705316

Clinical prediction guides

Expanding the clinicopathological spectrum of TGFBR3-PLAG1 rearranged salivary gland neoplasms with myoepithelial differentiation including evidence of high-grade transformation.
Rupp NJ, Höller S, Brada M, Vital D, Morand GB, Broglie MA, Huellner MW, Freiberger SN
Genes Chromosomes Cancer 2022 Feb;61(2):94-104. Epub 2021 Nov 17 doi: 10.1002/gcc.23009. PMID: 34755406Free PMC Article
Myxoinflammatory fibroblastic sarcoma: an immunohistochemical and molecular genetic study of 73 cases.
Suster D, Michal M, Huang H, Ronen S, Springborn S, Debiec-Rychter M, Billings SD, Goldblum JR, Rubin BP, Michal M, Suster S, Mackinnon AC
Mod Pathol 2020 Dec;33(12):2520-2533. Epub 2020 Jun 8 doi: 10.1038/s41379-020-0580-6. PMID: 32514165
Recurrent BRAF Gene Rearrangements in Myxoinflammatory Fibroblastic Sarcomas, but Not Hemosiderotic Fibrolipomatous Tumors.
Kao YC, Ranucci V, Zhang L, Sung YS, Athanasian EA, Swanson D, Dickson BC, Antonescu CR
Am J Surg Pathol 2017 Nov;41(11):1456-1465. doi: 10.1097/PAS.0000000000000899. PMID: 28692601Free PMC Article
TGFBR3 and MGEA5 rearrangements are much more common in "hybrid" hemosiderotic fibrolipomatous tumor-myxoinflammatory fibroblastic sarcomas than in classical myxoinflammatory fibroblastic sarcomas: a morphological and fluorescence in situ hybridization study.
Zreik RT, Carter JM, Sukov WR, Ahrens WA, Fritchie KJ, Montgomery EA, Weiss SW, Folpe AL
Hum Pathol 2016 Jul;53:14-24. Epub 2016 Mar 2 doi: 10.1016/j.humpath.2016.02.005. PMID: 26980036
TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms.
Carter JM, Sukov WR, Montgomery E, Goldblum JR, Billings SD, Fritchie KJ, Folpe AL
Am J Surg Pathol 2014 Sep;38(9):1182-992. doi: 10.1097/PAS.0000000000000212. PMID: 24705316

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