{Reference Type}: Review
{Title}: An unusual case of primary splenic soft part alveolar sarcoma: case report and review of the literature with emphasis on the spectrum of TFE3-associated neoplasms.
{Author}: Guérin R;Menard AL;Angot E;Piton N;Vera P;Schwarz L;Sabourin JC;Laé M;Thiébaut PA;
{Journal}: Diagn Pathol
{Volume}: 19
{Issue}: 1
{Year}: 2024 Apr 20
{Factor}: 3.196
{DOI}: 10.1186/s13000-024-01483-4
{Abstract}: <B>BACKGROUND: </B>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.<BR><B>METHODS: </B>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.<BR><B>CONCLUSIONS: </B>We described the first case of primary splenic alveolar soft part sarcoma, which questions once again the cell of origin of this rare tumour.