Keratin-positive giant cell-rich tumors of soft tissue with HMGA2::NCOR2 fusions.
J Cutan Pathol
; 50(11): 977-982, 2023 Nov.
Article
in 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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Soft Tissue Neoplasms
/
Giant Cell Tumors
Type of study:
Diagnostic_studies
/
Prognostic_studies
Limits:
Adult
/
Female
/
Humans
/
Male
Language:
En
Journal:
J Cutan Pathol
Year:
2023
Type:
Article
Affiliation country:
United States