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Giant cell tumor of soft tissue

MedGen UID:
87264
•Concept ID:
C0334553
•
Neoplastic Process
Synonyms: GCT-ST; Giant cell tumor of soft parts; Giant Cell Tumor of Soft Tissue; giant cell tumor of soft tissue; Osteoclastoma of soft tissue
SNOMED CT: Giant cell tumor of soft tissue (82125002); Giant cell tumor of soft parts (82125002); Giant cell tumor of soft parts (1295264007)
 
Monarch Initiative: MONDO:0006232

Definition

A painless, well circumscribed tumor arising in soft tissue, usually of the upper and lower extremities. Morphologically, it is characterized by a multinodular growth pattern. The cellular infiltrate is composed of mononuclear round or oval cells and multinucleated osteoclast-like giant cells, in a rich vascular stroma. It rarely metastasizes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGiant cell tumor of soft tissue

Professional guidelines

PubMed

Best clinical management of tenosynovial giant cell tumour (TGCT): A consensus paper from the community of experts.
Stacchiotti S, Dürr HR, Schaefer IM, Woertler K, Haas R, Trama A, Caraceni A, Bajpai J, Baldi GG, Bernthal N, Blay JY, Boye K, Broto JM, Chen WT, Dei Tos PA, Desai J, Emhofer S, Eriksson M, Gronchi A, Gelderblom H, Hardes J, Hartmann W, Healey J, Italiano A, Jones RL, Kawai A, Leithner A, Loong H, Mascard E, Morosi C, Otten N, Palmerini E, Patel SR, Reichardt P, Rubin B, Rutkowski P, Sangalli C, Schuster K, Seddon BM, Shkodra M, Staals EL, Tap W, van de Rijn M, van Langevelde K, Vanhoenacker FMM, Wagner A, Wiltink L, Stern S, Van de Sande VM, Bauer S
Cancer Treat Rev 2023 Jan;112:102491. Epub 2022 Dec 6 doi: 10.1016/j.ctrv.2022.102491. PMID: 36502615
Treatment updates on tenosynovial giant cell tumor.
Palmerini E, Staals EL
Curr Opin Oncol 2022 Jul 1;34(4):322-327. doi: 10.1097/CCO.0000000000000853. PMID: 35837703
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article

Recent clinical studies

Etiology

A Case of Metastatic Giant Cell Tumor of Soft Tissue of the Orbit Associated With PALB2 Variant.
Rajaii F, Aronow ME, Campbell AA, Boahene KDO, Gallia GL, Rodriguez FJ
JAMA Ophthalmol 2020 Dec 1;138(12):1322-1324. doi: 10.1001/jamaophthalmol.2020.4308. PMID: 33090178
Giant Cell Tumor of Soft Tissue: A Rare Entity.
Mavrogenis AF, Tsukamoto S, Antoniadou T, Righi A, Errani C
Orthopedics 2019 Jul 1;42(4):e364-e369. doi: 10.3928/01477447-20190624-04. PMID: 31323108
First Case Report of a Huge Giant Cell Tumor of Soft Tissue Originating from the Retroperitoneum.
Kishi S, Monma H, Hori H, Kinugasa S, Fujimoto M, Nakamura T
Am J Case Rep 2018 Jun 5;19:642-650. doi: 10.12659/AJCR.909261. PMID: 29867073Free PMC Article
Unusual aggressive course of a giant cell tumor of soft tissue during immunosuppressive therapy.
Grabellus F, von Winterfeld F, Sheu SY, Metz KA, Jahnke K, Schmid KW
Virchows Arch 2006 Jun;448(6):847-51. Epub 2006 Apr 5 doi: 10.1007/s00428-006-0184-7. PMID: 16596381
Soft tissue giant cell tumor of low malignant potential.
Icihikawa K, Tanino R
Tokai J Exp Clin Med 2004 Sep;29(3):91-5. PMID: 15595466

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
Giant cell tumor of soft tissue of the colon: a case report and review of the literature.
Lee SW, Lee J, Kim SJ, Hong R
BMC Gastroenterol 2022 Jun 27;22(1):317. doi: 10.1186/s12876-022-02391-x. PMID: 35761215Free PMC Article
A Case of Metastatic Giant Cell Tumor of Soft Tissue of the Orbit Associated With PALB2 Variant.
Rajaii F, Aronow ME, Campbell AA, Boahene KDO, Gallia GL, Rodriguez FJ
JAMA Ophthalmol 2020 Dec 1;138(12):1322-1324. doi: 10.1001/jamaophthalmol.2020.4308. PMID: 33090178
Giant Cell Tumor of Soft Tissue: A Rare Entity.
Mavrogenis AF, Tsukamoto S, Antoniadou T, Righi A, Errani C
Orthopedics 2019 Jul 1;42(4):e364-e369. doi: 10.3928/01477447-20190624-04. PMID: 31323108
Giant cell tumor of soft tissue.
Bresler SC, Wanat KA, Elenitsas R
Cutis 2014 Jun;93(6):278;286-8. PMID: 24999647

Therapy

Efficacy of Pazopanib in the Treatment of Metastatic Malignant Giant Cell Tumor of Soft Tissue: A Case Report.
Iwai T, Oebisu N, Hoshi M, Takada N, Nakamura H
Curr Oncol 2022 Jan 31;29(2):758-765. doi: 10.3390/curroncol29020064. PMID: 35200563Free PMC Article
Unusual aggressive course of a giant cell tumor of soft tissue during immunosuppressive therapy.
Grabellus F, von Winterfeld F, Sheu SY, Metz KA, Jahnke K, Schmid KW
Virchows Arch 2006 Jun;448(6):847-51. Epub 2006 Apr 5 doi: 10.1007/s00428-006-0184-7. PMID: 16596381

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 tumor of soft tissue of the colon: a case report and review of the literature.
Lee SW, Lee J, Kim SJ, Hong R
BMC Gastroenterol 2022 Jun 27;22(1):317. doi: 10.1186/s12876-022-02391-x. PMID: 35761215Free PMC Article
Osteoclast-Like Giant Cells: Focus on Entities Relevant to Dermatopathology and Underlying Pathogenesis.
Hamie L, Abbas O, Kurban M, Bhawan J
Am J Dermatopathol 2021 Mar 1;43(3):163-173. doi: 10.1097/DAD.0000000000001769. PMID: 33595228
Recurrent primary mediastinal giant cell tumor of soft tissue with radiological findings: a rare case report and literature review.
Hu Q, Peng J, Xia L
World J Surg Oncol 2017 Jul 26;15(1):137. doi: 10.1186/s12957-017-1205-5. PMID: 28747182Free PMC Article
Giant cell tumor of soft tissue with pulmonary metastases: pathologic and cytogenetic study.
Guo H, Garcia RA, Perle MA, Amodio J, Greco MA
Pediatr Dev Pathol 2005 Nov-Dec;8(6):718-24. Epub 2005 Nov 21 doi: 10.1007/s10024-005-0014-y. PMID: 16328671

Clinical prediction guides

Malignant giant cell tumor of toe: A case report and review of literature.
Hashimoto K, Nishimura S, Ito T, Akagi M
Medicine (Baltimore) 2022 Jun 3;101(22):e29471. doi: 10.1097/MD.0000000000029471. PMID: 35665736Free PMC Article
Efficacy of Pazopanib in the Treatment of Metastatic Malignant Giant Cell Tumor of Soft Tissue: A Case Report.
Iwai T, Oebisu N, Hoshi M, Takada N, Nakamura H
Curr Oncol 2022 Jan 31;29(2):758-765. doi: 10.3390/curroncol29020064. PMID: 35200563Free PMC Article
Giant cell tumor of soft tissue arising in breast.
May SA, Deavers MT, Resetkova E, Johnson D, Albarracin CT
Ann Diagn Pathol 2007 Oct;11(5):345-9. Epub 2007 Jul 24 doi: 10.1016/j.anndiagpath.2006.03.013. PMID: 17870021
Unusual aggressive course of a giant cell tumor of soft tissue during immunosuppressive therapy.
Grabellus F, von Winterfeld F, Sheu SY, Metz KA, Jahnke K, Schmid KW
Virchows Arch 2006 Jun;448(6):847-51. Epub 2006 Apr 5 doi: 10.1007/s00428-006-0184-7. PMID: 16596381

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