# Low-grade fibromyxoid sarcoma, a rare tumor at an unusual site: Case report and review of literature

## TL;DR

This paper reports a rare case of low-grade fibromyxoid sarcoma in the pleura and highlights the importance of accurate diagnosis and potential treatment options.

## Contribution

The paper presents a rare case of pleural LGFMS and suggests endocrine therapy as a potential palliative treatment.

## Key findings

- Pleural LGFMS is exceedingly rare, with only four prior cases reported.
- Accurate diagnosis using MUC4 is crucial due to the tumor's histologically benign appearance.
- Endocrine therapy may be a promising palliative option for unresectable LGFMS.

## Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is a rare fibroblastic neoplasm with an indolent clinical course. It is a distinctive subclass of soft tissue sarcoma with metastasizing potential and sometimes a long interval between tumor presentation and metastasis. This case report describes a 60-year-old female with an unresectable pleural LGFMS initially misdiagnosed as malignant mesothelioma. Pleural LGFMS remains exceedingly rare, with only four prior cases reported. Given its histologically benign appearance, LGFMS poses diagnostic challenges and risks of local recurrence or metastasis. This case underscores the importance of accurate diagnosis using MUC4 and it explores endocrine therapy as a promising palliative option for unresectable LGFMS, contributing valuable insights into management strategies for this rare entity.