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The Rapidly Expanding Group of RB1-Deleted Soft Tissue Tumors: An Updated Review
Sasha Libbrecht1, Jo Van Dorpe1,2, David Creytens1,2
1Department of Pathology, Ghent University Hospital, Ghent University, 9000 Ghent, Belgium.
Abstract:
The classification of soft tissue tumors has evolved considerably in the last decade, largely due to advances in understanding the pathogenetic basis of many of these, sometimes rare, tumors. Deletion of Retinoblastoma 1 (RB1), a well-known tumor suppressor gene, has been implicated in the tumorigenesis of a particular group of soft tissue neoplasms. This group of so-called "RB1-deleted soft tissue tumors" has been rapidly expanding in recent years, currently consisting of spindle cell/pleomorphic lipoma, atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, myofibroblastoma, cellular angiofibroma, and acral fibromyxoma. Most of these neoplasms, except pleomorphic liposarcoma, are considered benign entities and are mainly described in the older adult population. This article will review the currently known morphological, immunohistochemical, and molecular features of this heterogeneous group of mesenchymal tumors with an emphasis on differential diagnosis.
Insights
RB1-deleted soft tissue tumors are a growing group of neoplasms linked to the Retinoblastoma 1 gene. This review covers their features and differential diagnosis.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Soft tissue tumor classification has advanced due to understanding tumor pathogenesis.
- Retinoblastoma 1 (RB1) gene deletion is implicated in specific soft tissue neoplasms.
- RB1-deleted soft tissue tumors are a rapidly expanding group.
Purpose of the Study:
- To review the morphological, immunohistochemical, and molecular features of RB1-deleted soft tissue tumors.
- To emphasize the differential diagnosis of these mesenchymal tumors.
Main Methods:
- Review of current literature on RB1-deleted soft tissue tumors.
- Analysis of morphological characteristics.
- Evaluation of immunohistochemical markers.
- Assessment of molecular findings.
Main Results:
- The group includes spindle cell/pleomorphic lipoma, atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, myofibroblastoma, cellular angiofibroma, and acral fibromyxoma.
- Most RB1-deleted tumors are benign, primarily affecting older adults.
- Pleomorphic liposarcoma is an exception, considered malignant.
Conclusions:
- RB1 deletion defines a heterogeneous group of soft tissue tumors.
- Understanding these features is crucial for accurate diagnosis and management.
- Further research may elucidate more about the pathogenesis and classification of these tumors.
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