# Case Report: Parapharyngeal Leiomyosarcoma Mimicking Neurofibroma

**Authors:** Carson Brantley, Claudia N. Gutierrez, Sara Zadeh, Eric Dowling

PMC · DOI: 10.1002/ccr3.72270 · Clinical Case Reports · 2026-03-08

## TL;DR

This case report highlights the difficulty in distinguishing between two rare tumors in the throat area and emphasizes the importance of accurate diagnosis for better outcomes.

## Contribution

The report underscores the necessity of immunohistochemistry for accurate diagnosis of spindle cell tumors in the absence of clear clinical indicators.

## Key findings

- Leiomyosarcomas and neurofibromas in the parapharyngeal space have similar clinical and radiographic features.
- Immunohistochemistry is essential for diagnosis when clinical features are ambiguous.

## Abstract

Leiomyosarcomas and neurofibromas of the parapharyngeal space share similar clinical and radiographic features, making early diagnosis challenging. In patients without neurocutaneous disorders, an adequate tissue sample with immunohistochemistry is crucial for diagnosis. Early detection and treatment of leiomyosarcoma are essential to improve survival and prevent progression of disease.

Neurofibroma and leiomyosarcomas are rare tumors of the head and neck with similar imaging characteristics of T2 hyperintensity on MRI that can present with neurological deficits. However, leiomyosarcomas are malignant, and early diagnosis is critical to improved survival. Immunohistochemistry is crucial for assigning line of differentiation in spindle cell tumors, especially on limited samples.

## Linked entities

- **Diseases:** leiomyosarcoma (MONDO:0005058), neurofibroma (MONDO:0016755)

## Full-text entities

- **Genes:** NF1 (neurofibromin 1) [NCBI Gene 4763] {aka NFNS, VRNF, WSS}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}, DES (desmin) [NCBI Gene 1674] {aka CDCD3, CSM1, CSM2, LGMD1D, LGMD1E, LGMD2R}
- **Diseases:** dyspnea (MESH:D004417), Parapharyngeal space tumors (MESH:D009369), neurocutaneous disorders (MESH:D020752), head and neck leiomyosarcoma (MESH:D006258), spindle cell neoplasms (MESH:D002277), spindled soft tissue neoplasms (MESH:D012983), ptosis (MESH:C564553), Leiomyosarcomas (MESH:D007890), dysphonia (MESH:D055154), hepatic, pulmonary, and osseous metastases (MESH:D009362), dysphagia (MESH:D003680), otalgia (MESH:D004433), pain (MESH:D010146), occlusion (MESH:D001157), hypoglossal nerve dysfunction (MESH:D020437), neurological deficits (MESH:D009461), vocal fold paralysis (MESH:D014826), benign smooth muscle tumors (MESH:D018235), necrosis (MESH:D009336), sarcoma (MESH:D012509), squamous cell carcinoma (MESH:D002294), benign peripheral nerve sheath tumors (MESH:D018317), Cranial neuropathies (MESH:D003389), Neurofibroma (MESH:D009455), hoarseness (MESH:D006685), palatal asymmetry (MESH:D005146), dysarthria (MESH:D004401), nasopharyngeal regurgitation (MESH:D009304), pleomorphic adenoma (MESH:D008949)
- **Chemicals:** H&amp;E (MESH:D006371)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

5 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12967902/full.md

## References

5 references — full list in the complete paper: https://tomesphere.com/paper/PMC12967902/full.md

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Source: https://tomesphere.com/paper/PMC12967902