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Alveolar soft part sarcoma. An immunohistochemical, cytologic and electron-microscopic study and a quantitative DNA
S Persson1, J S Willems, L G Kindblom
1Department of Pathology, Sahlgren Hospital, Gothenburg University, Sweden.
Summary
Alveolar soft part sarcoma (ASPS) cells show rhabdomyomatous differentiation, not epithelial or neuroectodermal. Pleomorphic ASPS variants exhibit tetraploid DNA, unlike diploid ordinary ASPS.
Area of Science:
- Oncology
- Pathology
- Cell Biology
Background:
- Alveolar soft part sarcoma (ASPS) is a rare soft tissue malignancy with uncertain histogenesis.
- Understanding ASPS cell differentiation is crucial for accurate diagnosis and treatment.
Purpose of the Study:
- To investigate the cellular type, differentiation, and histogenesis of ASPS.
- To differentiate ordinary ASPS from its pleomorphic variant using various analytical methods.
Main Methods:
- Light microscopy, ultrastructural analysis, immunohistochemistry, cytology, and quantitative DNA analysis were performed on ten ASPS cases.
- Immunohistochemical markers included desmin, cytokeratins, epithelial membrane antigen, HMFG-1, -2, TPA, S-100 protein, glial fibrillary acidic protein, neurofilaments, vimentin, myoglobin, and neuron-specific enolase (NSE).
- Collagen IV and laminin were used to detect external lamina material.
Main Results:
- Ultrastructural and desmin-positive findings support rhabdomyomatous differentiation in ASPS.
- Negative results for epithelial and neuroectodermal markers exclude these differentiation pathways.
- Pleomorphic ASPS variants showed tetraploid DNA peaks, distinct from the diploid DNA distribution in ordinary ASPS.
- External lamina material was identified around tumor cell nests and vessels.
Conclusions:
- ASPS exhibits rhabdomyomatous differentiation, with pleomorphic variants showing distinct genetic alterations (tetraploidy).
- Cytologic findings in pleomorphic ASPS may mimic carcinoma, highlighting the need for comprehensive diagnostic approaches.
- The study clarifies the histogenesis of ASPS, supporting a myogenic origin.