# 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

## TL;DR

This paper reports the first case of a rare tumor called alveolar soft part sarcoma that originated in the spleen, highlighting its unique features and diagnostic methods.

## Contribution

The paper presents the first documented case of primary splenic alveolar soft part sarcoma, expanding the known locations of this rare tumor.

## Key findings

- The tumor showed typical alveolar morphology and TFE3 nuclear staining.
- Molecular testing confirmed the ASPSCR1::TFE3 fusion transcript and TFE3 rearrangement.
- This case raises questions about the cell of origin for alveolar soft part sarcoma.

## Abstract

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.

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.

We described the first case of primary splenic alveolar soft part sarcoma, which questions once again the cell of origin of this rare tumour.