Author:
Guérin René,Menard Anne-Lise,Angot Emilie,Piton Nicolas,Vera Pierre,Schwarz Lilian,Sabourin Jean-Christophe,Laé Marick,Thiébaut Pierre-Alain
Abstract
Abstract
Background
Alveolar soft part sarcoma is a rare tumour of soft tissues, mostly localized in muscles or deep soft tissues of the extremities. In rare occasions, this tumour develops in deep tissues of the abdomen or pelvis.
Case presentation
In this case report, we described the case of a 46 year old man who developed a primary splenic alveolar soft part sarcoma. The tumour displayed typical morphological alveolar aspect, as well as immunohistochemical profile notably TFE3 nuclear staining. Detection of ASPSCR1 Exon 7::TFE3 Exon 6 fusion transcript in molecular biology and TFE3 rearrangement in FISH confirmed the diagnosis.
Conclusion
We described the first case of primary splenic alveolar soft part sarcoma, which questions once again the cell of origin of this rare tumour.
Publisher
Springer Science and Business Media LLC
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