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Giant Cell-Rich Osteosarcoma

MedGen UID:
1792108
•Concept ID:
C5555667
•
Neoplastic Process

Definition

An exceedingly rare, high-grade variant of conventional osteosarcoma characterized by the presence of numerous osteoclast-like giant cells and variable amount of tumor osteoid. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGiant Cell-Rich Osteosarcoma

Professional guidelines

PubMed

KPNA2 expression is a potential marker for differential diagnosis between osteosarcomas and other malignant bone tumor mimics.
Jiang L, Liu J, Wei Q, Wang Y
Diagn Pathol 2020 Nov 11;15(1):135. doi: 10.1186/s13000-020-01051-6. PMID: 33176814Free PMC Article
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

Pediatric Spinal Giant Cell-rich Osteosarcoma: Case Report and Brief Literature Review.
Suzuki H, Ikeda H, Nishida N, Funaba M, Fujimoto K, Iwanaga R, Sakai T
Anticancer Res 2023 Oct;43(10):4739-4745. doi: 10.21873/anticanres.16671. PMID: 37772568
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
Malignancy in giant cell tumor of bone: analysis of an open-label phase 2 study of denosumab.
Palmerini E, Seeger LL, Gambarotti M, Righi A, Reichardt P, Bukata S, Blay JY, Dai T, Jandial D, Picci P
BMC Cancer 2021 Jan 22;21(1):89. doi: 10.1186/s12885-020-07739-8. PMID: 33482769Free PMC Article
Aberrant ADAM10 expression correlates with osteosarcoma progression.
Zhao R, Ni D, Tian Y, Ni B, Wang A
Eur J Med Res 2014 Feb 18;19(1):9. doi: 10.1186/2047-783X-19-9. PMID: 24548763Free PMC Article
Giant cell-rich osteosarcoma in long bones: clinical, radiological and pathological features.
Wang CS, Yin QH, Liao JS, Lou JH, Ding XY, Zhu YB
Radiol Med 2013 Dec;118(8):1324-34. Epub 2013 May 27 doi: 10.1007/s11547-013-0936-9. PMID: 23716292

Diagnosis

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
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
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
Giant cell-rich osteosarcoma in long bones: clinical, radiological and pathological features.
Wang CS, Yin QH, Liao JS, Lou JH, Ding XY, Zhu YB
Radiol Med 2013 Dec;118(8):1324-34. Epub 2013 May 27 doi: 10.1007/s11547-013-0936-9. PMID: 23716292
Giant cell-rich osteosarcoma or giant cell reparative granuloma of the mandible?
Fu HH, Zhuang QW, He J, Wang LZ, He Y
J Craniofac Surg 2011 May;22(3):1136-9. doi: 10.1097/SCS.0b013e3182108fbf. PMID: 21586969

Therapy

Malignancy in giant cell tumor of bone: analysis of an open-label phase 2 study of denosumab.
Palmerini E, Seeger LL, Gambarotti M, Righi A, Reichardt P, Bukata S, Blay JY, Dai T, Jandial D, Picci P
BMC Cancer 2021 Jan 22;21(1):89. doi: 10.1186/s12885-020-07739-8. PMID: 33482769Free PMC Article
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
Dedifferentiated parosteal osteosarcoma: the experience of the Rizzoli Institute.
Bertoni F, Bacchini P, Staals EL, Davidovitz P
Cancer 2005 Jun 1;103(11):2373-82. doi: 10.1002/cncr.21039. PMID: 15852358
Giant cell-rich osteosarcoma: a case report.
Sato K, Yamamura S, Iwata H, Sugiura H, Nakashima N, Nagasaka T
Nagoya J Med Sci 1996 Dec;59(3-4):151-7. PMID: 9212641

Prognosis

Pediatric Spinal Giant Cell-rich Osteosarcoma: Case Report and Brief Literature Review.
Suzuki H, Ikeda H, Nishida N, Funaba M, Fujimoto K, Iwanaga R, Sakai T
Anticancer Res 2023 Oct;43(10):4739-4745. doi: 10.21873/anticanres.16671. PMID: 37772568
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
Malignancy in giant cell tumor of bone: analysis of an open-label phase 2 study of denosumab.
Palmerini E, Seeger LL, Gambarotti M, Righi A, Reichardt P, Bukata S, Blay JY, Dai T, Jandial D, Picci P
BMC Cancer 2021 Jan 22;21(1):89. doi: 10.1186/s12885-020-07739-8. PMID: 33482769Free PMC Article
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
Giant cell-rich osteosarcoma in long bones: clinical, radiological and pathological features.
Wang CS, Yin QH, Liao JS, Lou JH, Ding XY, Zhu YB
Radiol Med 2013 Dec;118(8):1324-34. Epub 2013 May 27 doi: 10.1007/s11547-013-0936-9. PMID: 23716292

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
Malignancy in giant cell tumor of bone: analysis of an open-label phase 2 study of denosumab.
Palmerini E, Seeger LL, Gambarotti M, Righi A, Reichardt P, Bukata S, Blay JY, Dai T, Jandial D, Picci P
BMC Cancer 2021 Jan 22;21(1):89. doi: 10.1186/s12885-020-07739-8. PMID: 33482769Free 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
P63 expression as a biomarker discriminating giant cell tumor of bone from other giant cell-rich bone lesions.
Shooshtarizadeh T, Rahimi M, Movahedinia S
Pathol Res Pract 2016 Oct;212(10):876-879. Epub 2016 Jul 14 doi: 10.1016/j.prp.2016.07.007. PMID: 27473669

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