Giant cell-rich tumour with keratin expression and HMGA2::NCOR2 fusion presenting at a rare location, on the scalp of a 29-year-old male

Wilsher, Mark James; Venus, Matthew; Fisher, Cyril

doi:10.1016/j.pathol.2023.10.024

Pathology

2024

DOI: 10.1016/j.pathol.2023.10.024

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Giant cell-rich tumour with keratin expression and HMGA2::NCOR2 fusion presenting at a rare location, on the scalp of a 29-year-old male

Mark James Wilsher,

Matthew Venus,

Cyril Fisher

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2024

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Keratin-Positive Giant Cell-Rich Tumor: A Review and Update

Nishio,

Nakayama,

Koga

et al. 2024

Cancers

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Keratin-positive giant cell-rich tumor (KPGCT) is an extremely rare and recently described mesenchymal neoplasm that occurs in both soft tissue and bone, frequently found in young women. It has locally recurrent potential if incompletely excised but low risk for metastasis. KPGCT is histologically similar to conventional giant cell tumors of soft tissue but shows the presence of keratin-positive mononuclear cells. Interestingly, KPGCT also shares some morphological features with xanthogranulomatous epithelial tumors. These two tumors have recently been shown to harbor an HMGA2–NCOR2 fusion, arguing in favor of a single entity. Surgery is the treatment of choice for localized KPGCT. Therapeutic options for advanced or metastatic disease are unknown. This review provides an overview of the current knowledge on the clinical presentation, pathogenesis, histopathology, and treatment of KPGCT. In addition, we will discuss the differential diagnosis of this emerging entity.

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Keratin-Positive Giant Cell-Rich Tumor: A Review and Update

Nishio,

Nakayama,

Koga

et al. 2024

Cancers

View full text Add to dashboard Cite

show abstract

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Giant cell-rich tumour with keratin expression and HMGA2::NCOR2 fusion presenting at a rare location, on the scalp of a 29-year-old male

Cited by 1 publication

References 12 publications

Keratin-Positive Giant Cell-Rich Tumor: A Review and Update

Keratin-Positive Giant Cell-Rich Tumor: A Review and Update

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