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

Our plain-English summary of Low-grade fibromyxoid sarcoma, a rare tumor at an unusual site: Case report and review of literature, by R. Mohamedali, N. Nishith, R. Raj, et al (Discoveries, 2025).

The study

• Authors: R. Mohamedali, N. Nishith, R. Raj, A. Sharma, P.K. Somal, R.N. Pawar, S. Sancheti, D.S. Rathore
• Journal: Discoveries
• Published: 2025;13(1):e209
• Read it: PubMed · Publisher · Free full text

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.

Copyright © 2025, Mohamedali et al., Applied Systems and Discoveries Journals.

In plain English

This was a case report with a short literature review from a cancer hospital in New Chandigarh, India. A 60-year-old woman's chest CT scan showed multiple masses on the pleura lining the left chest wall, mediastinum and diaphragm; the largest was about 6.8 x 5.4 cm, and the scan suggested possible malignant mesothelioma. A needle biopsy instead led to a diagnosis of low-grade fibromyxoid sarcoma (LGFMS), a rare soft-tissue cancer. The tumor cells were positive for MUC4 and vimentin and negative for markers including calretinin and WT1. A recommended genetic test for FUS gene rearrangement was not done because of financial constraints. Because the mass could not be removed, she received the hormonal drugs tamoxifen and letrozole; her disease was stable after 10 months. The authors found only four earlier reports of LGFMS in the pleura. The authors concluded that MUC4 is the most specific diagnostic marker, that surgery remains the treatment of choice, and that hormonal therapy may be considered as a palliative option when the tumor cannot be removed. The article did not mention asbestos.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report of one patient with a literature review of previously published cases
• Who was studied: One 60-year-old woman with a pleural-based tumor, reported by authors at Homi Bhabha Cancer Hospital and Research Centre, New Chandigarh, Punjab, India. Dates of care are not given.
• Exposure: The article does not report or discuss asbestos or any occupational exposure. The only family history given is lung and esophageal carcinoma in relatives.
• Main results:
  • In this patient, the chest CT showed multiple pleural deposits and masses in the left costal, mediastinal and diaphragmatic pleura. These are the parts of the pleura lining the chest wall, mediastinum and diaphragm. The largest measured about 6.8 x 5.4 cm along the diaphragmatic pleura, and the possibility of malignant mesothelioma was suggested.
  • On biopsy, this patient's tumor cells were positive for MUC4 and vimentin, focally positive for CD99 and BCL2, and negative for Calretinin, WT1 and other markers. The MIB-1 labeling index was 1-2%.
  • This patient's final diagnosis was low-grade fibromyxoid sarcoma.
  • Because the mass was unresectable, this patient received tamoxifen and letrozole. Her disease remained stable over 10 months of follow-up every three months.
  • In the authors' literature review, LGFMS in the pleura had been reported in only four earlier cases.
• The authors' conclusion: The authors concluded that MUC4 is the most specific diagnostic marker, that surgical resection remains the treatment of choice for LGFMS, and that endocrine therapy may be considered as a palliative option for unresectable, recurrent or metastatic disease.
• Limitations the authors state:
  • The authors state that the FUS gene rearrangement test, which would further support the diagnosis, was not done because of financial constraints.

Topics

Medical terms in this study, explained in our Lexicon: Sarcoma, Neoplasm, Metastasis, Unresectable, Mesothelioma, Palliative Care.

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