# Low-Grade Myofibroblastic Sarcoma of the tongue: A case report and literature review

**Authors:** Evangelos Kalfarentzos, Venetia Louka, Charalampos Gkilas, Nikolaos Katsoulas, Penelope Korkolopoulou, Nikolaos Kolomvos

PMC · DOI: 10.4317/jced.63065 · Journal of Clinical and Experimental Dentistry · 2025-10-01

## TL;DR

This paper reports a rare case of low-grade myofibroblastic sarcoma in the tongue and reviews existing literature on the condition.

## Contribution

The novelty lies in presenting a new clinical case of LGMS in the tongue and emphasizing the need for long-term follow-up.

## Key findings

- LGMS in the tongue is extremely rare and presents as painless swelling.
- Histological and immunohistochemical features help distinguish LGMS from other sarcomas.
- Complete surgical excision with clear margins is the recommended treatment.

## Abstract

Low-grade myofibroblastic sarcoma (LGMS) is an extremely rare mesenchymal neoplasm. It arises from the differentiation of myofibroblasts and demonstrates a preference for the head and neck region. Herein, we present a case of LGMS in a 52-year-old man with painless swelling in the anterior right area of the tongue. Histologically, the lesion consisted of spindle-shaped cells exhibiting mild to moderate nuclear atypia, arranged in fascicles with a storiform pattern. Neoplastic cells were positive for SMA and negative for EMA, CD34, h-caldesmon, desmin, β-catenin, S100, SOX10, and NF, with a Ki-67 index of 15-20%. Complete surgical excision with clear margins was the treatment of choice. Very few cases of LGMS have been documented in the literature, and regular follow-up for these patients is essential for drawing reliable conclusions regarding local recurrence and the metastatic potential of this tumor.

Key words:Low-grade, myofibroblastic, sarcoma, tongue, myofibroblasts, immunohistochemistry.

## Linked entities

- **Proteins:** SMN1 (survival of motor neuron 1, telomeric), ETFA (electron transfer flavoprotein subunit alpha), CD34 (CD34 molecule), LOC101066771 (desmin-like), ctnnb1.S (catenin beta 1 S homeolog), S100A1 (S100 calcium binding protein A1), SOX10 (SRY-box transcription factor 10), NFASC (neurofascin)

## Full-text entities

- **Genes:** CTNNB1 (catenin beta 1) [NCBI Gene 1499] {aka CTNNB, EVR7, MRD19, NEDSDV, armadillo}, NFASC (neurofascin) [NCBI Gene 23114] {aka NEDCPMD, NF, NRCAML}, S100B (S100 calcium binding protein B) [NCBI Gene 6285] {aka NEF, S100, S100-B, S100beta}, DES (desmin) [NCBI Gene 1674] {aka CDCD3, CSM1, CSM2, LGMD1D, LGMD1E, LGMD2R}, SOX10 (SRY-box transcription factor 10) [NCBI Gene 6663] {aka DOM, PCWH, SOX-10, WS2E, WS4, WS4C}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, CD34 (CD34 molecule) [NCBI Gene 947], SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}
- **Diseases:** swelling (MESH:D004487), LGMS (MESH:D036821), sarcoma (MESH:D012509), mesenchymal neoplasm (MESH:D009369)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12620985/full.md

## References

15 references — full list in the complete paper: https://tomesphere.com/paper/PMC12620985/full.md

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Source: https://tomesphere.com/paper/PMC12620985