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Malignant Osteoclastic Giant Cell-Rich Tumor of Bone

MedGen UID:
887074
•Concept ID:
C4054576
•
Neoplastic Process

Definition

A giant cell tumor that arises from the bone and is characterized by the presence of a malignant cellular component. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Osteoclastic Giant Cell-Rich Tumor of Bone

Professional guidelines

PubMed

Giant cell rich osteosarcoma revisited-diagnostic criteria and histopathologic patterns, Ki67, CDK4, and MDM2 expression, changes in response to bisphosphonate and denosumab treatment.
Chow LT
Virchows Arch 2016 Jun;468(6):741-55. Epub 2016 Mar 22 doi: 10.1007/s00428-016-1926-9. PMID: 27003154

Recent clinical studies

Etiology

Giant cell-rich osteosarcoma: A match pair analysis of 11 new cases and literature review of 56 patients.
Karczewski D, Gonzalez MR, Bedi A, Ready JE, Anderson ME, Lozano-Calderon SA
J Surg Oncol 2023 Oct;128(5):877-890. Epub 2023 Jun 9 doi: 10.1002/jso.27368. PMID: 37292033
Recurrent Fusion of the Genes for High-mobility Group AT-hook 2 (HMGA2) and Nuclear Receptor Co-repressor 2 (NCOR2) in Osteoclastic Giant Cell-rich Tumors of Bone.
Panagopoulos I, Andersen K, Gorunova L, Lund-Iversen M, Lobmaier I, Heim S
Cancer Genomics Proteomics 2022 Mar-Apr;19(2):163-177. doi: 10.21873/cgp.20312. PMID: 35181586Free PMC Article
Soft Tissue Special Issue: Giant Cell-Rich Lesions of the Head and Neck Region.
Lee JC, Huang HY
Head Neck Pathol 2020 Mar;14(1):97-108. Epub 2020 Jan 16 doi: 10.1007/s12105-019-01086-2. PMID: 31950466Free PMC Article
Challenges of denosumab in giant cell tumor of bone, and other giant cell-rich tumors of bone.
Lipplaa A, Dijkstra S, Gelderblom H
Curr Opin Oncol 2019 Jul;31(4):329-335. doi: 10.1097/CCO.0000000000000529. PMID: 30844887Free PMC Article
Giant-cell-rich pseudotumour in Paget's disease.
Gamage NM, Kashima TG, McNally MA, Gibbons CL, Smith R, Ostlere SJ, Athanasou NA
Skeletal Radiol 2013 Apr;42(4):595-9. Epub 2012 Dec 29 doi: 10.1007/s00256-012-1558-3. PMID: 23275027

Diagnosis

Keratin-positive giant cell-rich tumors of soft tissue with HMGA2::NCOR2 fusions.
Weigelt MA, Azzato EM, Habermehl GK, Billings SD, Ko JS, Fritchie KJ
J Cutan Pathol 2023 Nov;50(11):977-982. Epub 2023 Jul 26 doi: 10.1111/cup.14497. PMID: 37496152
Molecular and immunohistochemical testing of bone tumours: review and update.
Kovacs SK, Manassaporn A, Nielsen GP, Hung YP
Histopathology 2023 May;82(6):794-811. Epub 2022 Dec 16 doi: 10.1111/his.14845. PMID: 36424903
Update of pediatric bone tumors: osteogenic tumors and osteoclastic giant cell-rich tumors.
Al-Dasuqi K, Cheng R, Moran J, Irshaid L, Maloney E, Porrino J
Skeletal Radiol 2023 Apr;52(4):671-685. Epub 2022 Nov 3 doi: 10.1007/s00256-022-04221-3. PMID: 36326880
Giant cell tumor of bone in an eighteenth-century Italian mummy.
Ventura L, Petrella E, Piciucchi S, Cilli E, Luiselli D, Feeney RNM, Traversari M
Virchows Arch 2021 Dec;479(6):1255-1261. Epub 2021 Aug 30 doi: 10.1007/s00428-021-03192-5. PMID: 34462806Free PMC Article
Soft Tissue Special Issue: Giant Cell-Rich Lesions of the Head and Neck Region.
Lee JC, Huang HY
Head Neck Pathol 2020 Mar;14(1):97-108. Epub 2020 Jan 16 doi: 10.1007/s12105-019-01086-2. PMID: 31950466Free PMC Article

Therapy

Denosumab in Giant Cell Rich Tumors of Bone: An Open-Label Multicenter Phase II Study.
Lipplaa A, Schreuder WH, Pichardo SEC, Gelderblom H
Oncologist 2023 Nov 2;28(11):1005-e1104. doi: 10.1093/oncolo/oyad196. PMID: 37449658Free PMC Article
Chondroblastoma Expresses RANKL by RNA In Situ Hybridization and May Respond to Denosumab Therapy.
Suster DI, Kurzawa P, Neyaz A, Jarzembowski JA, Lozano-Calderon S, Raskin K, Schwab J, Choy E, Chebib I, Deshpande V
Am J Surg Pathol 2020 Dec;44(12):1581-1590. doi: 10.1097/PAS.0000000000001568. PMID: 32826531
Challenges of denosumab in giant cell tumor of bone, and other giant cell-rich tumors of bone.
Lipplaa A, Dijkstra S, Gelderblom H
Curr Opin Oncol 2019 Jul;31(4):329-335. doi: 10.1097/CCO.0000000000000529. PMID: 30844887Free PMC Article
Diagnostic utility of histone H3.3 G34W, G34R, and G34V mutant-specific antibodies for giant cell tumors of bone.
Yamamoto H, Iwasaki T, Yamada Y, Matsumoto Y, Otsuka H, Yoshimoto M, Kohashi K, Taguchi K, Yokoyama R, Nakashima Y, Oda Y
Hum Pathol 2018 Mar;73:41-50. Epub 2017 Dec 12 doi: 10.1016/j.humpath.2017.11.020. PMID: 29241742
Giant cell rich osteosarcoma revisited-diagnostic criteria and histopathologic patterns, Ki67, CDK4, and MDM2 expression, changes in response to bisphosphonate and denosumab treatment.
Chow LT
Virchows Arch 2016 Jun;468(6):741-55. Epub 2016 Mar 22 doi: 10.1007/s00428-016-1926-9. PMID: 27003154

Prognosis

Keratin-positive giant cell-rich tumors of soft tissue with HMGA2::NCOR2 fusions.
Weigelt MA, Azzato EM, Habermehl GK, Billings SD, Ko JS, Fritchie KJ
J Cutan Pathol 2023 Nov;50(11):977-982. Epub 2023 Jul 26 doi: 10.1111/cup.14497. PMID: 37496152
Giant cell-rich osteosarcoma: A match pair analysis of 11 new cases and literature review of 56 patients.
Karczewski D, Gonzalez MR, Bedi A, Ready JE, Anderson ME, Lozano-Calderon SA
J Surg Oncol 2023 Oct;128(5):877-890. Epub 2023 Jun 9 doi: 10.1002/jso.27368. PMID: 37292033
Update of pediatric bone tumors-other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone.
Wang A, Maloney E, Al-Dasuqi K, Irshaid L, Ahmed A, Haims A, Porrino J
Skeletal Radiol 2023 Aug;52(8):1443-1463. Epub 2023 Jan 19 doi: 10.1007/s00256-023-04286-8. PMID: 36656343
Update of pediatric bone tumors: osteogenic tumors and osteoclastic giant cell-rich tumors.
Al-Dasuqi K, Cheng R, Moran J, Irshaid L, Maloney E, Porrino J
Skeletal Radiol 2023 Apr;52(4):671-685. Epub 2022 Nov 3 doi: 10.1007/s00256-022-04221-3. PMID: 36326880
Giant cell tumor of bone in an eighteenth-century Italian mummy.
Ventura L, Petrella E, Piciucchi S, Cilli E, Luiselli D, Feeney RNM, Traversari M
Virchows Arch 2021 Dec;479(6):1255-1261. Epub 2021 Aug 30 doi: 10.1007/s00428-021-03192-5. PMID: 34462806Free PMC Article

Clinical prediction guides

Giant cell-rich osteosarcoma: A match pair analysis of 11 new cases and literature review of 56 patients.
Karczewski D, Gonzalez MR, Bedi A, Ready JE, Anderson ME, Lozano-Calderon SA
J Surg Oncol 2023 Oct;128(5):877-890. Epub 2023 Jun 9 doi: 10.1002/jso.27368. PMID: 37292033
Significance of Histone H3.3 (G34W)-Mutant Protein in Pathological Diagnosis of Giant Cell Tumor of Bone.
Miskad UA, Syamsul F, Dahlan H, Sungowati NK, Achmad D, Johan MP
Asian Pac J Cancer Prev 2023 May 1;24(5):1737-1741. doi: 10.31557/APJCP.2023.24.5.1737. PMID: 37247296Free PMC Article
Anti-Histone H3.3 G34W antibody is a sensitive and highly specific immunohistochemistry marker for the diagnosis of Giant cell tumor of bone. A validation based on analysis of 198 cases from a single centre in India.
Kamble A, Hui M, Rao KN, Narayanan R, Reddy BR, Uppin SG, Chandrasekhar P
Indian J Pathol Microbiol 2022 Jul-Sep;65(3):617-629. doi: 10.4103/ijpm.ijpm_265_21. PMID: 35900490
Giant cell tumor of bone in an eighteenth-century Italian mummy.
Ventura L, Petrella E, Piciucchi S, Cilli E, Luiselli D, Feeney RNM, Traversari M
Virchows Arch 2021 Dec;479(6):1255-1261. Epub 2021 Aug 30 doi: 10.1007/s00428-021-03192-5. PMID: 34462806Free PMC Article
A rare case of a metastatic giant cell-rich osteosarcoma of the mandible: Update and differential diagnostic considerations.
Santana LADM, Felix FA, de Arruda JAA, da Silva LP, Brito ÉAC, Takeshita WM, Trento CL
Oral Surg Oral Med Oral Pathol Oral Radiol 2021 May;131(5):e163-e169. Epub 2020 Oct 17 doi: 10.1016/j.oooo.2020.10.009. PMID: 33187945

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