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Conventional Extraskeletal Myxoid Chondrosarcoma

MedGen UID:
234486
•Concept ID:
C1337010
•
Neoplastic Process
Synonyms: Extraskeletal Well Differentiated Myxoid Chondrosarcoma; Well Differentiated Extraosseous Myxoid Chondrosarcoma; Well Differentiated Extraskeletal Myxoid Chondrosarcoma

Definition

A well differentiated extraskeletal myxoid chondrosarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVConventional Extraskeletal Myxoid Chondrosarcoma

Recent clinical studies

Etiology

Invasive urothelial carcinoma with chordoid features or abundant myxoid stroma: A reappraisal of morphologic spectrum and risk stratification based on molecular classification.
Kim JM, Choi E, Sung SH, Kang H, Park S
Pathol Res Pract 2023 Nov;251:154839. Epub 2023 Oct 2 doi: 10.1016/j.prp.2023.154839. PMID: 37801908
Results of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma.
Chow W, Frankel P, Ruel C, Araujo DM, Milhem M, Okuno S, Hartner L, Undevia S, Staddon A
Cancer 2020 Jan 1;126(1):105-111. Epub 2019 Sep 11 doi: 10.1002/cncr.32515. PMID: 31509242
Clinical benefit of antiangiogenic therapy in advanced and metastatic chondrosarcoma.
Jones RL, Katz D, Loggers ET, Davidson D, Rodler ET, Pollack SM
Med Oncol 2017 Aug 29;34(10):167. doi: 10.1007/s12032-017-1030-2. PMID: 28852958Free PMC Article
Clinicopathological and molecular spectrum of ewing sarcomas/PNETs, including validation of EWSR1 rearrangement by conventional and array FISH technique in certain cases.
Rekhi B, Vogel U, Basak R, Desai SB, Jambhekar NA
Pathol Oncol Res 2014 Jul;20(3):503-16. Epub 2013 Nov 30 doi: 10.1007/s12253-013-9721-2. PMID: 24293381
Update on chondrosarcomas.
Chow WA
Curr Opin Oncol 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9. PMID: 17545802

Diagnosis

GRM1 Immunohistochemistry Distinguishes Chondromyxoid Fibroma From its Histologic Mimics.
Toland AMS, Lam SW, Varma S, Wang A, Howitt BE, Kunder CA, Kerr DA, Szuhai K, Bovée JVMG, Charville GW
Am J Surg Pathol 2022 Oct 1;46(10):1407-1414. Epub 2022 Jun 1 doi: 10.1097/PAS.0000000000001921. PMID: 35650682Free PMC Article
Detection of sarcoma fusions by a next-generation sequencing based-ligation-dependent multiplex RT-PCR assay.
Lanic MD, Le Loarer F, Rainville V, Sater V, Viennot M, Beaussire L, Viailly PJ, Angot E, Hostein I, Jardin F, Ruminy P, Laé M
Mod Pathol 2022 May;35(5):649-663. Epub 2022 Jan 24 doi: 10.1038/s41379-021-00980-x. PMID: 35075283
Bone Tumors Occurring in the Soft Tissues: A Review of the Clinical, Imaging, and Histopathologic Findings.
Veselis CA, Awan O, Thomas A, Ling S, Jonnalagadda P, Aneja A, Ali S
Curr Probl Diagn Radiol 2021 May-Jun;50(3):419-429. Epub 2020 Jun 26 doi: 10.1067/j.cpradiol.2020.06.004. PMID: 32665061
From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation.
Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH
Radiographics 2003 Sep-Oct;23(5):1245-78. doi: 10.1148/rg.235035134. PMID: 12975513
Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 18 cases.
Okamoto S, Hisaoka M, Ishida T, Imamura T, Kanda H, Shimajiri S, Hashimoto H
Hum Pathol 2001 Oct;32(10):1116-24. doi: 10.1053/hupa.2001.28226. PMID: 11679947

Therapy

Results of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma.
Chow W, Frankel P, Ruel C, Araujo DM, Milhem M, Okuno S, Hartner L, Undevia S, Staddon A
Cancer 2020 Jan 1;126(1):105-111. Epub 2019 Sep 11 doi: 10.1002/cncr.32515. PMID: 31509242
Clinical benefit of antiangiogenic therapy in advanced and metastatic chondrosarcoma.
Jones RL, Katz D, Loggers ET, Davidson D, Rodler ET, Pollack SM
Med Oncol 2017 Aug 29;34(10):167. doi: 10.1007/s12032-017-1030-2. PMID: 28852958Free PMC Article

Prognosis

Bone Tumors Occurring in the Soft Tissues: A Review of the Clinical, Imaging, and Histopathologic Findings.
Veselis CA, Awan O, Thomas A, Ling S, Jonnalagadda P, Aneja A, Ali S
Curr Probl Diagn Radiol 2021 May-Jun;50(3):419-429. Epub 2020 Jun 26 doi: 10.1067/j.cpradiol.2020.06.004. PMID: 32665061
Molecular and Clinicopathologic Heterogeneity of Intracranial Tumors Mimicking Extraskeletal Myxoid Chondrosarcoma.
Velz J, Agaimy A, Frontzek K, Neidert MC, Bozinov O, Wagner U, Fritz C, Coras R, Hofer S, Bode-Lesniewska B, Rushing E
J Neuropathol Exp Neurol 2018 Aug 1;77(8):727-735. doi: 10.1093/jnen/nly050. PMID: 29924341
CD44: survival and metastasis in chondrosarcoma.
Heyse TJ, Malcherczyk D, Moll R, Timmesfeld N, Wapelhorst J, Fuchs-Winkelmann S, Paletta JR, Schofer MD
Osteoarthritis Cartilage 2010 Jun;18(6):849-56. Epub 2010 Feb 18 doi: 10.1016/j.joca.2010.02.007. PMID: 20171296
Update on chondrosarcomas.
Chow WA
Curr Opin Oncol 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9. PMID: 17545802
From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation.
Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH
Radiographics 2003 Sep-Oct;23(5):1245-78. doi: 10.1148/rg.235035134. PMID: 12975513

Clinical prediction guides

GRM1 Immunohistochemistry Distinguishes Chondromyxoid Fibroma From its Histologic Mimics.
Toland AMS, Lam SW, Varma S, Wang A, Howitt BE, Kunder CA, Kerr DA, Szuhai K, Bovée JVMG, Charville GW
Am J Surg Pathol 2022 Oct 1;46(10):1407-1414. Epub 2022 Jun 1 doi: 10.1097/PAS.0000000000001921. PMID: 35650682Free PMC Article
Postoperative Radiation Therapy in Patients with Extracranial Chondrosarcoma: A Joint Study of the French Sarcoma Group and Rare Cancer Network.
Terlizzi M, Le Pechoux C, Salas S, Rapeaud E, Lerouge D, Sunyach MP, Vogin G, Sole CV, Zilli T, Lutsyk M, Mampuya A, Calvo FA, Attal J, Karahissarlian V, De Bari B, Ozsahin M, Baumard F, Krengli M, Gomez-Brouchet A, Sargos P, Rochcongar G, Bazille C, Roth V, Salleron J, Thariat J
Int J Radiat Oncol Biol Phys 2020 Jul 15;107(4):726-735. Epub 2020 Apr 11 doi: 10.1016/j.ijrobp.2020.03.041. PMID: 32289473
Molecular and Clinicopathologic Heterogeneity of Intracranial Tumors Mimicking Extraskeletal Myxoid Chondrosarcoma.
Velz J, Agaimy A, Frontzek K, Neidert MC, Bozinov O, Wagner U, Fritz C, Coras R, Hofer S, Bode-Lesniewska B, Rushing E
J Neuropathol Exp Neurol 2018 Aug 1;77(8):727-735. doi: 10.1093/jnen/nly050. PMID: 29924341
ERG expression in chondrogenic bone and soft tissue tumours.
Shon W, Folpe AL, Fritchie KJ
J Clin Pathol 2015 Feb;68(2):125-9. Epub 2014 Nov 5 doi: 10.1136/jclinpath-2014-202601. PMID: 25378537
Extraskeletal myxoid chondrosarcoma: a light microscopic, immunohistochemical, ultrastructural and immuno-ultrastructural study indicating neuroendocrine differentiation.
Goh YW, Spagnolo DV, Platten M, Caterina P, Fisher C, Oliveira AM, Nascimento AG
Histopathology 2001 Nov;39(5):514-24. doi: 10.1046/j.1365-2559.2001.01277.x. PMID: 11737310

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