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Low Grade Central Osteosarcoma

MedGen UID:
220427
•Concept ID:
C1266163
•
Neoplastic Process
Synonyms: Intraosseous low grade osteosarcoma; Intraosseous well differentiated osteosarcoma; Intraosseous Well-Differentiated Osteogenic Sarcoma; Intraosseous Well-Differentiated Osteosarcoma; Low Grade Intramedullary Osteosarcoma; Low-grade central osteosarcoma; Low-Grade Intramedullary Osteosarcoma
SNOMED CT: Intraosseous low grade osteosarcoma (128771003); Intraosseous well differentiated osteosarcoma (128771003); Low-grade central osteosarcoma (1290872000)

Definition

A low grade osteosarcoma arising from the medullary portion of the bone. It affects the long bones and is characterized by the presence of fibroblastic stroma and osteoid production. Pain and swelling are the usual sign and symptom. The prognosis is more favorable than conventional osteosarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLow Grade Central Osteosarcoma

Professional guidelines

PubMed

Diagnosis and treatment of low-grade osteosarcoma: experience with nine cases.
Hayashi K, Tsuchiya H, Yamamoto N, Shirai T, Nishida H, Takeuchi A, Kimura H, Miwa S, Inatani H, Okamoto H, Yamada S, Ikeda H, Sawada-Kitamura S, Nojima T, Ooi A, Otsuka T
Int J Clin Oncol 2014 Aug;19(4):731-8. Epub 2013 Jul 24 doi: 10.1007/s10147-013-0592-z. PMID: 23881556
MDM2 and CDK4 immunohistochemistry is a valuable tool in the differential diagnosis of low-grade osteosarcomas and other primary fibro-osseous lesions of the bone.
Dujardin F, Binh MB, Bouvier C, Gomez-Brouchet A, Larousserie F, Muret Ad, Louis-Brennetot C, Aurias A, Coindre JM, Guillou L, Pedeutour F, Duval H, Collin C, de Pinieux G
Mod Pathol 2011 May;24(5):624-37. Epub 2011 Feb 18 doi: 10.1038/modpathol.2010.229. PMID: 21336260

Recent clinical studies

Etiology

Effect of adjuvant chemotherapy on localized dedifferentiated low-grade osteosarcoma: a systematic review.
Tsukamoto S, Righi A, Mavrogenis AF, Masunaga T, Honoki K, Fujii H, Kido A, Tanaka Y, Tanaka Y, Errani C
Musculoskelet Surg 2024 Sep;108(3):241-249. Epub 2024 May 6 doi: 10.1007/s12306-024-00821-5. PMID: 38709428
Diagnostic challenges in low-grade central osteosarcoma.
Khal AA, Aiba H, Righi A, Gambarotti M, Atherley O'Meally AO, Manfrini M, Donati DM, Errani C
Bone Joint J 2024 Jan 1;106-B(1):99-106. doi: 10.1302/0301-620X.106B1.BJJ-2023-0531.R1. PMID: 38160683
Bone and soft tissue tumors at the borderlands of malignancy.
Crim J, Layfield LJ
Skeletal Radiol 2023 Mar;52(3):379-392. Epub 2022 Jun 29 doi: 10.1007/s00256-022-04099-1. PMID: 35767018
Dedifferentiation in low-grade osteosarcoma: a Japanese Musculoskeletal Oncology Group (JMOG) study.
Hirai T, Kobayashi H, Kobayashi E, Saito M, Akiyama T, Kikuta K, Nakai T, Endo M, Tsukamoto S, Hakozaki M, Takenaka S, Nishimura S, Kawashima H, Tanzawa Y, Kawano H, Tanaka S
Int J Clin Oncol 2022 Nov;27(11):1758-1766. Epub 2022 Aug 6 doi: 10.1007/s10147-022-02223-8. PMID: 35932377
A clinical comparison between dedifferentiated low-grade osteosarcoma and conventional osteosarcoma.
Toki S, Kobayashi E, Yoshida A, Ogura K, Wakai S, Yoshimoto S, Yonemori K, Kawai A
Bone Joint J 2019 Jun;101-B(6):745-752. doi: 10.1302/0301-620X.101B6.BJJ-2018-1207.R1. PMID: 31154837

Diagnosis

Diagnostic challenges in low-grade central osteosarcoma.
Khal AA, Aiba H, Righi A, Gambarotti M, Atherley O'Meally AO, Manfrini M, Donati DM, Errani C
Bone Joint J 2024 Jan 1;106-B(1):99-106. doi: 10.1302/0301-620X.106B1.BJJ-2023-0531.R1. PMID: 38160683
Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
Bone and soft tissue tumors at the borderlands of malignancy.
Crim J, Layfield LJ
Skeletal Radiol 2023 Mar;52(3):379-392. Epub 2022 Jun 29 doi: 10.1007/s00256-022-04099-1. PMID: 35767018
Dedifferentiation in low-grade osteosarcoma: a Japanese Musculoskeletal Oncology Group (JMOG) study.
Hirai T, Kobayashi H, Kobayashi E, Saito M, Akiyama T, Kikuta K, Nakai T, Endo M, Tsukamoto S, Hakozaki M, Takenaka S, Nishimura S, Kawashima H, Tanzawa Y, Kawano H, Tanaka S
Int J Clin Oncol 2022 Nov;27(11):1758-1766. Epub 2022 Aug 6 doi: 10.1007/s10147-022-02223-8. PMID: 35932377
Diagnosis and treatment of low-grade osteosarcoma: experience with nine cases.
Hayashi K, Tsuchiya H, Yamamoto N, Shirai T, Nishida H, Takeuchi A, Kimura H, Miwa S, Inatani H, Okamoto H, Yamada S, Ikeda H, Sawada-Kitamura S, Nojima T, Ooi A, Otsuka T
Int J Clin Oncol 2014 Aug;19(4):731-8. Epub 2013 Jul 24 doi: 10.1007/s10147-013-0592-z. PMID: 23881556

Therapy

Effect of adjuvant chemotherapy on localized dedifferentiated low-grade osteosarcoma: a systematic review.
Tsukamoto S, Righi A, Mavrogenis AF, Masunaga T, Honoki K, Fujii H, Kido A, Tanaka Y, Tanaka Y, Errani C
Musculoskelet Surg 2024 Sep;108(3):241-249. Epub 2024 May 6 doi: 10.1007/s12306-024-00821-5. PMID: 38709428
Dedifferentiation in low-grade osteosarcoma: a Japanese Musculoskeletal Oncology Group (JMOG) study.
Hirai T, Kobayashi H, Kobayashi E, Saito M, Akiyama T, Kikuta K, Nakai T, Endo M, Tsukamoto S, Hakozaki M, Takenaka S, Nishimura S, Kawashima H, Tanzawa Y, Kawano H, Tanaka S
Int J Clin Oncol 2022 Nov;27(11):1758-1766. Epub 2022 Aug 6 doi: 10.1007/s10147-022-02223-8. PMID: 35932377
Denosumab-treated giant cell tumor of bone. Its histologic spectrum and potential diagnostic pitfalls.
Roitman PD, Jauk F, Farfalli GL, Albergo JI, Aponte-Tinao LA
Hum Pathol 2017 May;63:89-97. Epub 2017 Feb 22 doi: 10.1016/j.humpath.2017.02.008. PMID: 28235628
Denosumab-treated Giant Cell Tumor of Bone Exhibits Morphologic Overlap With Malignant Giant Cell Tumor of Bone.
Wojcik J, Rosenberg AE, Bredella MA, Choy E, Hornicek FJ, Nielsen GP, Deshpande V
Am J Surg Pathol 2016 Jan;40(1):72-80. doi: 10.1097/PAS.0000000000000506. PMID: 26414220

Prognosis

Diagnostic challenges in low-grade central osteosarcoma.
Khal AA, Aiba H, Righi A, Gambarotti M, Atherley O'Meally AO, Manfrini M, Donati DM, Errani C
Bone Joint J 2024 Jan 1;106-B(1):99-106. doi: 10.1302/0301-620X.106B1.BJJ-2023-0531.R1. PMID: 38160683
Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
Dedifferentiation in low-grade osteosarcoma: a Japanese Musculoskeletal Oncology Group (JMOG) study.
Hirai T, Kobayashi H, Kobayashi E, Saito M, Akiyama T, Kikuta K, Nakai T, Endo M, Tsukamoto S, Hakozaki M, Takenaka S, Nishimura S, Kawashima H, Tanzawa Y, Kawano H, Tanaka S
Int J Clin Oncol 2022 Nov;27(11):1758-1766. Epub 2022 Aug 6 doi: 10.1007/s10147-022-02223-8. PMID: 35932377
A clinical comparison between dedifferentiated low-grade osteosarcoma and conventional osteosarcoma.
Toki S, Kobayashi E, Yoshida A, Ogura K, Wakai S, Yoshimoto S, Yonemori K, Kawai A
Bone Joint J 2019 Jun;101-B(6):745-752. doi: 10.1302/0301-620X.101B6.BJJ-2018-1207.R1. PMID: 31154837
Syndecan-4 and fibronectin in osteosarcoma.
Na KY, Bacchini P, Bertoni F, Kim YW, Park YK
Pathology 2012 Jun;44(4):325-30. doi: 10.1097/PAT.0b013e328353447b. PMID: 22531343

Clinical prediction guides

Sarcoma care in the era of precision medicine.
Wallander K, Öfverholm I, Boye K, Tsagkozis P, Papakonstantinou A, Lin Y, Haglund de Flon F
J Intern Med 2023 Dec;294(6):690-707. Epub 2023 Sep 7 doi: 10.1111/joim.13717. PMID: 37643281
Low-grade osteosarcoma is predominant in gnathic osteosarcomas: A report of seven cases of osteosarcoma of the jaw.
Sasaki A, Miyashita H, Kawaida M, Kameyama K
Clin Exp Dent Res 2021 Dec;7(6):1175-1182. Epub 2021 May 19 doi: 10.1002/cre2.442. PMID: 34008925Free PMC Article
GNAS mutations are not detected in parosteal and low-grade central osteosarcomas.
Salinas-Souza C, De Andrea C, Bihl M, Kovac M, Pillay N, Forshew T, Gutteridge A, Ye H, Amary MF, Tirabosco R, Toledo SR, Baumhoer D, Flanagan AM
Mod Pathol 2015 Oct;28(10):1336-42. Epub 2015 Aug 7 doi: 10.1038/modpathol.2015.91. PMID: 26248895
Activating GNAS mutations in parosteal osteosarcoma.
Carter JM, Inwards CY, Jin L, Evers B, Wenger DE, Oliveira AM, Fritchie KJ
Am J Surg Pathol 2014 Mar;38(3):402-9. doi: 10.1097/PAS.0000000000000144. PMID: 24525511
Low-grade central osteosarcoma of the mandible: a case study report.
Diniz AF, Filho JA, Alencar Rde C, Garcia RR, Silva MR, Ribeiro-Rotta RF, Silva MA, Batista AC, Mendonça EF
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007 Feb;103(2):246-52. Epub 2006 Jul 11 doi: 10.1016/j.tripleo.2005.11.006. PMID: 17234543

Recent systematic reviews

Effect of adjuvant chemotherapy on localized dedifferentiated low-grade osteosarcoma: a systematic review.
Tsukamoto S, Righi A, Mavrogenis AF, Masunaga T, Honoki K, Fujii H, Kido A, Tanaka Y, Tanaka Y, Errani C
Musculoskelet Surg 2024 Sep;108(3):241-249. Epub 2024 May 6 doi: 10.1007/s12306-024-00821-5. PMID: 38709428

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