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Extraskeletal myxoid chondrosarcoma

MedGen UID:
220892
•Concept ID:
C1275278
•
Neoplastic Process
Synonym: Chondrosarcoma, Extraskeletal Myxoid
SNOMED CT: Extraskeletal myxoid chondrosarcoma (404079008); Chondroid sarcoma (404079008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Genes (locations): NR4A3 (9q31.1); TAF15 (17q12)
 
Monarch Initiative: MONDO:0012825
OMIM®: 612237
Orphanet: ORPHA209916

Definition

Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm of chondroblastic origin. The tumors are most commonly found in middle-aged and elderly individuals, are more common among men, and are often detected as deep-seated lesions in the extremities. Despite their relatively low-grade malignancy, recurrence and metastasis may appear many years after the initial diagnosis. Histologic tissue section examination reveals a mixture of cellular and myxoid stromal components (Panagopoulos et al., 2002). [from OMIM]

Clinical features

From HPO
Chondrosarcoma
MedGen UID:
3054
•Concept ID:
C0008479
•
Neoplastic Process
A slowly growing malignant neoplasm derived from cartilage cells.
See: Feature record | Search on this feature

Professional guidelines

PubMed

Extraskeletal Myxoid Chondrosarcoma: Retrospective Case Series Examining Prognostic Factors, Treatment Approaches, and Oncologic Outcomes.
Brodsky CN, Bucala MD, Abdulfatah EM, Siegel GW
Am J Clin Oncol 2023 Apr 1;46(4):172-177. Epub 2023 Feb 24 doi: 10.1097/COC.0000000000000988. PMID: 36825763
Differential diagnosis of myxoid soft tissue tumors. Experience in the Clinical University Hospital of Valencia.
Alfaro-Cervelló C, Nieto G, Navarro S
Rev Esp Patol 2022 Jul-Sep;55(3):185-191. Epub 2019 Nov 4 doi: 10.1016/j.patol.2019.07.005. PMID: 35779885
SWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis.
Agaimy A
Surg Pathol Clin 2019 Mar;12(1):149-163. doi: 10.1016/j.path.2018.10.006. PMID: 30709441

Recent clinical studies

Etiology

Surgical outcomes of extraskeletal myxoid chondrosarcoma.
Ulucaköy C, Atalay İB, Yapar A, Kaptan AY, Bingöl İ, Doğan M, Ekşioğlu MF
Turk J Med Sci 2022 Aug;52(4):1183-1189. Epub 2022 Aug 10 doi: 10.55730/1300-0144.5422. PMID: 36326382Free PMC Article
Primary extraskeletal myxoid chondrosarcoma of the breast: report of a case and literature review.
Sharma S, Lobo A, Sharma A, Sampat NY, Kumar M, Kajla R, Mohapatra SS, Mohanty SK
Pathologica 2022 Jun;114(3):228-237. doi: 10.32074/1591-951X-303. PMID: 35775709Free PMC Article
Extraskeletal myxoid chondrosarcoma: Clinical features and overall survival.
Brown JM, Rakoczy K, Pretell-Mazzini J
Cancer Treat Res Commun 2022;31:100530. Epub 2022 Feb 5 doi: 10.1016/j.ctarc.2022.100530. PMID: 35144048
Update on chondrosarcomas.
Chow WA
Curr Opin Oncol 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9. PMID: 17545802
Extraskeletal myxoid chondrosarcoma.
McGrory JE, Rock MG, Nascimento AG, Oliveira AM
Clin Orthop Relat Res 2001 Jan;(382):185-90. doi: 10.1097/00003086-200101000-00025. PMID: 11153986

Diagnosis

Surgical outcomes of extraskeletal myxoid chondrosarcoma.
Ulucaköy C, Atalay İB, Yapar A, Kaptan AY, Bingöl İ, Doğan M, Ekşioğlu MF
Turk J Med Sci 2022 Aug;52(4):1183-1189. Epub 2022 Aug 10 doi: 10.55730/1300-0144.5422. PMID: 36326382Free PMC Article
Primary extraskeletal myxoid chondrosarcoma of the breast: report of a case and literature review.
Sharma S, Lobo A, Sharma A, Sampat NY, Kumar M, Kajla R, Mohapatra SS, Mohanty SK
Pathologica 2022 Jun;114(3):228-237. doi: 10.32074/1591-951X-303. PMID: 35775709Free PMC Article
Extraskeletal myxoid chondrosarcoma: Clinical features and overall survival.
Brown JM, Rakoczy K, Pretell-Mazzini J
Cancer Treat Res Commun 2022;31:100530. Epub 2022 Feb 5 doi: 10.1016/j.ctarc.2022.100530. PMID: 35144048
INSM1 expression and its diagnostic significance in extraskeletal myxoid chondrosarcoma.
Yoshida A, Makise N, Wakai S, Kawai A, Hiraoka N
Mod Pathol 2018 May;31(5):744-752. Epub 2018 Jan 12 doi: 10.1038/modpathol.2017.189. PMID: 29327709
Primary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature.
Finos L, Righi A, Frisoni T, Gambarotti M, Ghinelli C, Benini S, Vanel D, Picci P
Pathol Res Pract 2017 May;213(5):461-466. Epub 2017 Feb 11 doi: 10.1016/j.prp.2017.02.008. PMID: 28249774

Therapy

Identification of Novel PGR-NR4A3 Fusion in Extraskeletal Myxoid Chondrosarcoma and Resultant Patient Benefit From Tamoxifen Therapy.
Wilbur HC, Robinson DR, Wu YM, Kumar-Sinha C, Chinnaiyan AM, Chugh R
JCO Precis Oncol 2022 Aug;6:e2200039. doi: 10.1200/PO.22.00039. PMID: 36103645Free PMC Article
Pazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: a multicentre, single-arm, phase 2 trial.
Stacchiotti S, Ferrari S, Redondo A, Hindi N, Palmerini E, Vaz Salgado MA, Frezza AM, Casali PG, Gutierrez A, Lopez-Pousa A, Grignani G, Italiano A, LeCesne A, Dumont S, Blay JY, Penel N, Bernabeu D, de Alava E, Karanian M, Morosi C, Brich S, Dagrada GP, Vallacchi V, Castelli C, Brenca M, Racanelli D, Maestro R, Collini P, Cruz J, Martin-Broto J
Lancet Oncol 2019 Sep;20(9):1252-1262. Epub 2019 Jul 19 doi: 10.1016/S1470-2045(19)30319-5. PMID: 31331701
Results of sub-analysis of a phase 2 study on trabectedin treatment for extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma.
Morioka H, Takahashi S, Araki N, Sugiura H, Ueda T, Takahashi M, Yonemoto T, Hiraga H, Hiruma T, Kunisada T, Matsumine A, Susa M, Nakayama R, Nishimoto K, Kikuta K, Horiuchi K, Kawai A
BMC Cancer 2016 Jul 14;16:479. doi: 10.1186/s12885-016-2511-y. PMID: 27418251Free PMC Article
Activity of sunitinib in extraskeletal myxoid chondrosarcoma.
Stacchiotti S, Pantaleo MA, Astolfi A, Dagrada GP, Negri T, Dei Tos AP, Indio V, Morosi C, Gronchi A, Colombo C, Conca E, Toffolatti L, Tazzari M, Crippa F, Maestro R, Pilotti S, Casali PG
Eur J Cancer 2014 Jun;50(9):1657-64. Epub 2014 Apr 2 doi: 10.1016/j.ejca.2014.03.013. PMID: 24703573
Emerging therapies for adult soft tissue sarcoma.
Radaelli S, Stacchiotti S, Casali PG, Gronchi A
Expert Rev Anticancer Ther 2014 Jun;14(6):689-704. Epub 2014 Feb 20 doi: 10.1586/14737140.2014.885840. PMID: 24555529

Prognosis

Surgical outcomes of extraskeletal myxoid chondrosarcoma.
Ulucaköy C, Atalay İB, Yapar A, Kaptan AY, Bingöl İ, Doğan M, Ekşioğlu MF
Turk J Med Sci 2022 Aug;52(4):1183-1189. Epub 2022 Aug 10 doi: 10.55730/1300-0144.5422. PMID: 36326382Free PMC Article
Extraskeletal myxoid chondrosarcoma: Clinicopathological features and outcomes from the United States sarcoma collaborative database.
Gusho CA, King D, Ethun CG, Cardona K, Harrison Howard J, Tran TB, Poultsides G, Tseng J, Roggin KK, Fields RC, Cullinan DR, Chouliaras K, Votanopoulos K, Grignol VP, Bedi M
J Surg Oncol 2022 Dec;126(8):1533-1542. Epub 2022 Aug 13 doi: 10.1002/jso.27062. PMID: 35962783
Extraskeletal myxoid chondrosarcoma: Clinical features and overall survival.
Brown JM, Rakoczy K, Pretell-Mazzini J
Cancer Treat Res Commun 2022;31:100530. Epub 2022 Feb 5 doi: 10.1016/j.ctarc.2022.100530. PMID: 35144048
Update on chondrosarcomas.
Chow WA
Curr Opin Oncol 2007 Jul;19(4):371-6. doi: 10.1097/CCO.0b013e32812143d9. PMID: 17545802
Extraskeletal myxoid chondrosarcoma.
McGrory JE, Rock MG, Nascimento AG, Oliveira AM
Clin Orthop Relat Res 2001 Jan;(382):185-90. doi: 10.1097/00003086-200101000-00025. PMID: 11153986

Clinical prediction guides

Extraskeletal myxoid chondrosarcoma: Clinicopathological features and outcomes from the United States sarcoma collaborative database.
Gusho CA, King D, Ethun CG, Cardona K, Harrison Howard J, Tran TB, Poultsides G, Tseng J, Roggin KK, Fields RC, Cullinan DR, Chouliaras K, Votanopoulos K, Grignol VP, Bedi M
J Surg Oncol 2022 Dec;126(8):1533-1542. Epub 2022 Aug 13 doi: 10.1002/jso.27062. PMID: 35962783
Extraskeletal myxoid chondrosarcoma: Clinical features and overall survival.
Brown JM, Rakoczy K, Pretell-Mazzini J
Cancer Treat Res Commun 2022;31:100530. Epub 2022 Feb 5 doi: 10.1016/j.ctarc.2022.100530. PMID: 35144048
SWI/SNF complex-deficient soft tissue neoplasms: An update.
Schaefer IM, Hornick JL
Semin Diagn Pathol 2021 May;38(3):222-231. Epub 2020 Jun 5 doi: 10.1053/j.semdp.2020.05.005. PMID: 32646614Free PMC Article
Next generation sequencing of extraskeletal myxoid chondrosarcoma.
Davis EJ, Wu YM, Robinson D, Schuetze SM, Baker LH, Athanikar J, Cao X, Kunju LP, Chinnaiyan AM, Chugh R
Oncotarget 2017 Mar 28;8(13):21770-21777. doi: 10.18632/oncotarget.15568. PMID: 28423517Free PMC Article
Myxoid and round cell liposarcoma: a spectrum of myxoid adipocytic neoplasia.
Orvieto E, Furlanetto A, Laurino L, Dei Tos AP
Semin Diagn Pathol 2001 Nov;18(4):267-73. PMID: 11757867

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