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Malignant tenosynovial giant cell tumor

MedGen UID:
226849
•Concept ID:
C1266168
•
Neoplastic Process
Synonyms: Giant cell tumor of tendon sheath, malignant; giant cell tumor of tendon sheath, malignant; giant cell tumour of tendon sheath, malignant; Malignant Giant Cell Neoplasm of Tendon Sheath; malignant giant cell neoplasm of tendon sheath; Malignant Giant Cell Neoplasm of the Tendon Sheath; malignant giant cell neoplasm of the tendon sheath; Malignant Giant Cell Tumor of Tendon Sheath; malignant giant cell tumor of tendon sheath; Malignant Giant Cell Tumor of the Tendon Sheath; malignant giant cell tumor of the tendon sheath; malignant giant cell tumour of tendon sheath; malignant giant cell tumour of the tendon sheath; Malignant Tendon Sheath Giant Cell Neoplasm; malignant tendon sheath giant cell neoplasm; Malignant Tendon Sheath Giant Cell Tumor; malignant tendon sheath giant cell tumor; malignant tendon sheath giant cell tumour; Malignant Tenosynovial Giant Cell Tumor; malignant tenosynovial giant cell tumor; tenosynovial giant cell tumor, malignant
SNOMED CT: Giant cell tumor of tendon sheath, malignant (128778009); Malignant tenosynovial giant cell tumor (128778009)
 
Monarch Initiative: MONDO:0002401

Definition

An uncommon malignant tumor arising from the tendon sheath. Morphologically, it is characterized by the presence of a cellular infiltrate reminiscent of a giant cell tumor with prominent malignant characteristics. Recurrent giant cell tumors with a sarcomatous dedifferentiation are included in this category as well. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant tenosynovial giant cell tumor

Professional guidelines

PubMed

RNAscope CSF1 chromogenic in situ hybridization: a potentially useful tool in the differential diagnosis of tenosynovial giant cell tumors.
Thangaiah JJ, Koepplin JW, Folpe AL
Hum Pathol 2021 Sep;115:1-9. Epub 2021 May 28 doi: 10.1016/j.humpath.2021.05.010. PMID: 34058245

Recent clinical studies

Diagnosis

RNAscope CSF1 chromogenic in situ hybridization: a potentially useful tool in the differential diagnosis of tenosynovial giant cell tumors.
Thangaiah JJ, Koepplin JW, Folpe AL
Hum Pathol 2021 Sep;115:1-9. Epub 2021 May 28 doi: 10.1016/j.humpath.2021.05.010. PMID: 34058245
Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy.
Nakayama R, Jagannathan JP, Ramaiya N, Ferrone ML, Raut CP, Ready JE, Hornick JL, Wagner AJ
BMC Cancer 2018 Dec 29;18(1):1296. doi: 10.1186/s12885-018-5188-6. PMID: 30594158Free PMC Article
Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study.
Alexiev BA, Tumer Y, Yang GY
Hum Pathol 2017 May;63:144-148. Epub 2016 Dec 15 doi: 10.1016/j.humpath.2016.12.003. PMID: 27989786

Therapy

Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy.
Nakayama R, Jagannathan JP, Ramaiya N, Ferrone ML, Raut CP, Ready JE, Hornick JL, Wagner AJ
BMC Cancer 2018 Dec 29;18(1):1296. doi: 10.1186/s12885-018-5188-6. PMID: 30594158Free PMC Article

Prognosis

RNAscope CSF1 chromogenic in situ hybridization: a potentially useful tool in the differential diagnosis of tenosynovial giant cell tumors.
Thangaiah JJ, Koepplin JW, Folpe AL
Hum Pathol 2021 Sep;115:1-9. Epub 2021 May 28 doi: 10.1016/j.humpath.2021.05.010. PMID: 34058245
Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study.
Alexiev BA, Tumer Y, Yang GY
Hum Pathol 2017 May;63:144-148. Epub 2016 Dec 15 doi: 10.1016/j.humpath.2016.12.003. PMID: 27989786

Clinical prediction guides

RNAscope CSF1 chromogenic in situ hybridization: a potentially useful tool in the differential diagnosis of tenosynovial giant cell tumors.
Thangaiah JJ, Koepplin JW, Folpe AL
Hum Pathol 2021 Sep;115:1-9. Epub 2021 May 28 doi: 10.1016/j.humpath.2021.05.010. PMID: 34058245
Malignant Tenosynovial Giant Cell Tumor: The True "Synovial Sarcoma?" A Clinicopathologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases, Supporting Origin from Synoviocytes.
Al-Ibraheemi A, Ahrens WA, Fritchie K, Dong J, Oliveira AM, Balzer B, Folpe AL
Mod Pathol 2019 Feb;32(2):242-251. Epub 2018 Sep 11 doi: 10.1038/s41379-018-0129-0. PMID: 30206409
Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study.
Alexiev BA, Tumer Y, Yang GY
Hum Pathol 2017 May;63:144-148. Epub 2016 Dec 15 doi: 10.1016/j.humpath.2016.12.003. PMID: 27989786

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