# Therapeutic Challenges in Metastatic Myxofibrosarcoma: A Case-Based Review

**Authors:** Trie Arni Djunadi, Angela Grigos, Svetoslav Bardarov, Shamim Salman

PMC · DOI: 10.7759/cureus.93954 · Cureus · 2025-10-06

## TL;DR

This case study explores the challenges of treating metastatic myxofibrosarcoma, highlighting the need for better understanding and new therapies.

## Contribution

The paper presents a case with genetic mutations suggesting potential for targeted therapies in metastatic myxofibrosarcoma.

## Key findings

- The patient's MFS showed genetic alterations in NF1 and ATM, indicating possible responsiveness to MEK and PARP inhibitors.
- Current treatments for MFS offer limited durable control, underscoring the need for novel therapeutic strategies.

## Abstract

Myxofibrosarcoma (MFS) is a histologically distinct and aggressive subtype of soft tissue sarcoma, most often affecting elderly individuals and commonly presenting in the extremities. Despite standard treatment modalities, including surgical resection, radiotherapy, and chemotherapy, MFS is notable for its high recurrence and metastatic potential.

We present the case of a 64-year-old woman with stage IIIA MFS of the right thigh and progressive pulmonary metastases, despite undergoing radiotherapy, surgery, and systemic chemotherapy. Her disease course was complicated by genetic alterations, including NF1 and ATM mutations, suggesting potential responsiveness to targeted therapies such as MEK and PARP inhibitors. Histological and immunohistochemical findings confirmed a fibrohistiocytic origin, consistent with metastatic MFS.

This case highlights the diagnostic and therapeutic challenges associated with MFS, emphasizing the need for improved molecular characterization and novel therapeutic strategies. While current treatments provide limited durable control, emerging insights into the tumor’s genetic and immune landscape offer hope for more effective, personalized approaches.

## Linked entities

- **Genes:** NF1 (neurofibromin 1) [NCBI Gene 4763], ATM (ATM serine/threonine kinase) [NCBI Gene 472]
- **Diseases:** myxofibrosarcoma (MONDO:0019202)

## Full-text entities

- **Genes:** PARP1 (poly(ADP-ribose) polymerase 1) [NCBI Gene 142] {aka ADPRT, ADPRT 1, ADPRT1, ARTD1, PARP, PARP-1}, ATM (ATM serine/threonine kinase) [NCBI Gene 472] {aka AT1, ATA, ATC, ATD, ATDC, ATE}, NF1 (neurofibromin 1) [NCBI Gene 4763] {aka NFNS, VRNF, WSS}, MAP2K7 (mitogen-activated protein kinase kinase 7) [NCBI Gene 5609] {aka JNKK2, MAPKK7, MEK, MEK 7, MKK7, PRKMK7}
- **Diseases:** tumor (MESH:D009369), pulmonary metastases (MESH:D009362), soft tissue sarcoma (MESH:D012509)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12588600/full.md

## References

19 references — full list in the complete paper: https://tomesphere.com/paper/PMC12588600/full.md

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Source: https://tomesphere.com/paper/PMC12588600