Keratin-positive giant cell-rich tumors of soft tissue with HMGA2::NCOR2 fusions.
J Cutan Pathol
; 50(11): 977-982, 2023 Nov.
Article
em En
| MEDLINE
| ID: mdl-37496152
ABSTRACT
BACKGROUND:
Giant cell tumor of soft tissue (GCT-ST) is a rare soft tissue neoplasm that is morphologically similar to but genetically distinct from giant cell tumor of bone. A novel keratin-positive GCT-ST (KPGCT-ST) harboring HMGA2NCOR2 fusions was recently discovered. Fewer than 30 cases have been described; herein is reported an additional seven.METHODS:
Cases diagnosed as GCT-ST were retrieved from institutional archives and consultation files. The histopathologic characteristics were assessed, and the electronic medical record was reviewed.RESULTS:
Seven tumors were identified in six women and one man with a median age of 23 years. All patients underwent excision; no recurrences or metastases were noted during a median follow-up period of 7 months. Histopathologically, the tumors were characterized by a multinodular proliferation of keratin-positive mononuclear cells with evenly admixed osteoclast-like giant cells and absent neoplastic bone. A fibrous capsule with lymphoid cuffing was frequently seen. Foamy macrophages, inflammation, hemorrhage, and hemosiderin were variably present. The HMGA2NCOR2 fusion was detected in all cases.CONCLUSIONS:
Our findings support previously reported hypotheses that KPGCT-ST is a spectrum of the same entity as the recently described xanthogranulomatous epithelial tumor. Although follow-up data are limited, to date, KPGCT-ST appears to follow an indolent course.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias de Tecidos Moles
/
Tumores de Células Gigantes
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Adult
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Female
/
Humans
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Male
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article