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Chondrosarcoma

MedGen UID:
3054
Concept ID:
C0008479
Neoplastic Process
Synonym: Chondrosarcoma, somatic
SNOMED CT: Chondrosarcoma (443520009); Fibrochondrosarcoma (443520009); Chondrosarcoma (1163016002); Fibrochondrosarcoma (1163016002)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Gene (location): EXT1 (8q24.11)
 
HPO: HP:0006765
Monarch Initiative: MONDO:0008977
OMIM®: 215300
Orphanet: ORPHA55880

Definition

A slowly growing malignant neoplasm derived from cartilage cells. [from HPO]

Clinical features

From HPO
Chondrosarcoma
MedGen UID:
3054
Concept ID:
C0008479
Neoplastic Process
A slowly growing malignant neoplasm derived from cartilage cells.

Conditions with this feature

Chondrosarcoma
MedGen UID:
3054
Concept ID:
C0008479
Neoplastic Process
A slowly growing malignant neoplasm derived from cartilage cells.
Enchondromatosis
MedGen UID:
41775
Concept ID:
C0014084
Finding
Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis. When hemangiomata are associated, the condition is known as Maffucci syndrome (614569). Clinical problems caused by enchondromas include skeletal deformity and the potential for malignant change to osteosarcoma (Schwartz et al., 1987). Classification of the Enchondromatoses In their classification of the enchondromatoses, Spranger et al. (1978) called Ollier disease and Maffucci syndrome types I and II enchondromatosis, respectively; metachondromatosis (156250), type III; spondyloenchondrodysplasia (607944), type IV; enchondromatosis with irregular vertebral lesions, type V; and generalized enchondromatosis, type VI. Halal and Azouz (1991) added 3 tentative categories to the 6 in the classification of Spranger et al. (1978). Pansuriya et al. (2010) suggested a new classification of enchondromatosis (multiple enchondromas).
Multiple congenital exostosis
MedGen UID:
4612
Concept ID:
C0015306
Congenital Abnormality
Hereditary multiple osteochondromas (HMO), previously called hereditary multiple exostoses (HME), is characterized by growths of multiple osteochondromas, benign cartilage-capped bone tumors that grow outward from the metaphyses of long bones. Osteochondromas can be associated with a reduction in skeletal growth, bony deformity, restricted joint motion, shortened stature, premature osteoarthrosis, and compression of peripheral nerves. The median age of diagnosis is three years; nearly all affected individuals are diagnosed by age 12 years. The risk for malignant degeneration to osteochondrosarcoma increases with age, although the lifetime risk for malignant degeneration is low (~2%-5%).
Maffucci syndrome
MedGen UID:
7437
Concept ID:
C0024454
Disease or Syndrome
Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis. When hemangiomata are associated, the condition is known as Maffucci syndrome. Clinical problems caused by enchondromas include skeletal deformity and the potential for malignant change to osteosarcoma (Schwartz et al., 1987). Classification of the Enchondromatoses In their classification of the enchondromatoses, Spranger et al. (1978) called Ollier disease (166000) and Maffucci syndrome types I and II enchondromatosis, respectively; metachondromatosis (156250), type III; and spondyloenchondrodysplasia (607944), type IV; enchondromatosis with irregular vertebral lesions, type V; and generalized enchondromatosis, type VI. Halal and Azouz (1991) added 3 tentative categories to the 6 in the classification of Spranger et al. (1978). Pansuriya et al. (2010) suggested a new classification of enchondromatosis (multiple enchondromas).
Extraskeletal myxoid chondrosarcoma
MedGen UID:
220892
Concept ID:
C1275278
Neoplastic Process
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).
Exostoses, multiple, type 2
MedGen UID:
377018
Concept ID:
C1851413
Disease or Syndrome
Hereditary multiple osteochondromas (HMO), previously called hereditary multiple exostoses (HME), is characterized by growths of multiple osteochondromas, benign cartilage-capped bone tumors that grow outward from the metaphyses of long bones. Osteochondromas can be associated with a reduction in skeletal growth, bony deformity, restricted joint motion, shortened stature, premature osteoarthrosis, and compression of peripheral nerves. The median age of diagnosis is three years; nearly all affected individuals are diagnosed by age 12 years. The risk for malignant degeneration to osteochondrosarcoma increases with age, although the lifetime risk for malignant degeneration is low (~2%-5%).

Professional guidelines

PubMed

Wells ME, Eckhoff MD, Kafchinski LA, Polfer EM, Potter BK
JBJS Rev 2021 May 6;9(5) doi: 10.2106/JBJS.RVW.20.00159. PMID: 34881859
MacDonald IJ, Lin CY, Kuo SJ, Su CM, Tang CH
Expert Rev Anticancer Ther 2019 Sep;19(9):773-786. Epub 2019 Sep 6 doi: 10.1080/14737140.2019.1659731. PMID: 31462102
Ferguson JL, Turner SP
Am Fam Physician 2018 Aug 15;98(4):205-213. PMID: 30215968

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Bone Cancer, 2024

Recent clinical studies

Etiology

Weinschenk RC, Wang WL, Lewis VO
J Am Acad Orthop Surg 2021 Jul 1;29(13):553-562. doi: 10.5435/JAAOS-D-20-01188. PMID: 33595238
Choi JH, Ro JY
Adv Anat Pathol 2021 May 1;28(3):119-138. doi: 10.1097/PAP.0000000000000293. PMID: 33480599
Chow WA
F1000Res 2018;7 Epub 2018 Nov 20 doi: 10.12688/f1000research.15953.1. PMID: 30519452Free PMC Article
Leddy LR, Holmes RE
Cancer Treat Res 2014;162:117-30. doi: 10.1007/978-3-319-07323-1_6. PMID: 25070233
Qasem SA, DeYoung BR
Semin Diagn Pathol 2014 Jan;31(1):10-20. Epub 2014 Jan 23 doi: 10.1053/j.semdp.2014.01.006. PMID: 24680178

Diagnosis

Hameed M
Surg Pathol Clin 2021 Dec;14(4):605-617. Epub 2021 Oct 7 doi: 10.1016/j.path.2021.06.005. PMID: 34742483
Wu ZH, Dai JY, Shi JN, Fang MY, Cao J
Medicine (Baltimore) 2019 Nov;98(47):e18043. doi: 10.1097/MD.0000000000018043. PMID: 31764826Free PMC Article
Arora K, Riddle ND
Arch Pathol Lab Med 2018 Nov;142(11):1421-1424. doi: 10.5858/arpa.2017-0109-RS. PMID: 30407856
Jeong W, Kim HJ
J Clin Pathol 2018 Jul;71(7):579-583. Epub 2018 Mar 28 doi: 10.1136/jclinpath-2018-205071. PMID: 29593061Free PMC Article
Mendonça V, Jorge M, Monteiro-Grillo I, Palhano MJ, Feijó S
Clin Transl Oncol 2010 Aug;12(8):576-80. doi: 10.1007/s12094-010-0557-x. PMID: 20709656

Therapy

Subbiah V, Chawla SP, Conley AP, Wilky BA, Tolcher A, Lakhani NJ, Berz D, Andrianov V, Crago W, Holcomb M, Hussain A, Veldstra C, Kalabus J, O'Neill B, Senne L, Rowell E, Heidt AB, Willis KM, Eckelman BP
Clin Cancer Res 2023 Aug 15;29(16):2988-3003. doi: 10.1158/1078-0432.CCR-23-0974. PMID: 37265425Free PMC Article
Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
Tap WD, Villalobos VM, Cote GM, Burris H, Janku F, Mir O, Beeram M, Wagner AJ, Jiang L, Wu B, Choe S, Yen K, Gliser C, Fan B, Agresta S, Pandya SS, Trent JC
J Clin Oncol 2020 May 20;38(15):1693-1701. Epub 2020 Mar 24 doi: 10.1200/JCO.19.02492. PMID: 32208957Free PMC Article
Strauss SJ, Whelan JS
Curr Opin Oncol 2018 Jul;30(4):252-259. doi: 10.1097/CCO.0000000000000456. PMID: 29782347
Kaste SC
Radiol Clin North Am 2011 Jul;49(4):749-65, vi-vii. Epub 2011 Jun 16 doi: 10.1016/j.rcl.2011.05.006. PMID: 21807172Free PMC Article

Prognosis

Talati VM, Urban MJ, Patel TR, Wojcik C, Tajudeen BA, Stenson K, Bhayani MK, Al-Khudari S, Husain IA
Otolaryngol Head Neck Surg 2022 Jan;166(1):101-108. Epub 2021 Apr 13 doi: 10.1177/01945998211004578. PMID: 33848444
Grimm NL, Tainter DM, Eward WC, Brigman BE
JBJS Rev 2017 May;5(5):e4. doi: 10.2106/JBJS.RVW.16.00080. PMID: 28538290
Roos E, van Coevorden F, Verhoef C, Wouters MW, Kroon HM, Hogendoorn PC, van Houdt WJ
Ann Surg Oncol 2016 Mar;23(3):811-7. Epub 2015 Oct 23 doi: 10.1245/s10434-015-4932-2. PMID: 26498768
Mavrogenis AF, Ruggieri P, Mercuri M, Papagelopoulos PJ
J Surg Orthop Adv 2011 Summer;20(2):106-11. PMID: 21838071
Mercuri M, Picci P, Campanacci L, Rulli E
Skeletal Radiol 1995 Aug;24(6):409-16. doi: 10.1007/BF00941235. PMID: 7481896

Clinical prediction guides

Rock A, Ali S, Chow WA
Curr Treat Options Oncol 2022 Feb;23(2):199-209. Epub 2022 Feb 21 doi: 10.1007/s11864-022-00951-7. PMID: 35190971
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
Hameed M
Surg Pathol Clin 2021 Dec;14(4):605-617. Epub 2021 Oct 7 doi: 10.1016/j.path.2021.06.005. PMID: 34742483
Jeong W, Kim HJ
J Clin Pathol 2018 Jul;71(7):579-583. Epub 2018 Mar 28 doi: 10.1136/jclinpath-2018-205071. PMID: 29593061Free PMC Article
Liu P, Shen JK, Xu J, Trahan CA, Hornicek FJ, Duan Z
Epigenomics 2016 Nov;8(11):1519-1525. Epub 2016 Sep 30 doi: 10.2217/epi-2016-0071. PMID: 27686001Free PMC Article

Recent systematic reviews

Álvarez-Calderón-Iglesias O, Pérez-Sayáns M, Hurtado-Ruzza R, Lorenzo-Pouso A, Chamorro-Petronacci C
Acta Otorhinolaryngol Ital 2022 Dec;42(6):502-515. doi: 10.14639/0392-100X-N1912. PMID: 36654516Free PMC Article
Baba A, Kurokawa R, Kurokawa M, Ota Y, Lobo R, Srinivasan A
J Neuroimaging 2022 Mar;32(2):213-222. Epub 2022 Jan 12 doi: 10.1111/jon.12963. PMID: 35019171
Heuermann M, Bekker S, Czeczok T, Gregory S, Sharma A
Cancer Rep (Hoboken) 2022 Jul;5(7):e1537. Epub 2021 Sep 2 doi: 10.1002/cnr2.1537. PMID: 34476908Free PMC Article
Rotolo N, Cattoni M, Nardecchia E, De Maio S, Franzi F, Pettenon F, Imperatori A
Acta Chir Belg 2022 Oct;122(5):361-365. Epub 2020 Dec 25 doi: 10.1080/00015458.2020.1863039. PMID: 33306456
Bloch OG, Jian BJ, Yang I, Han SJ, Aranda D, Ahn BJ, Parsa AT
J Clin Neurosci 2009 Dec;16(12):1547-51. Epub 2009 Sep 30 doi: 10.1016/j.jocn.2009.05.003. PMID: 19796952Free PMC Article

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Bone Cancer, 2024

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