# Maxillary Intraosseous Plexiform Schwannoma: A Rare Neoplasm Radiographically Imitating an Odontogenic Lesion

**Authors:** Kittiphoj Tikkhanarak, Emily Lanzel, Sherry Timmons, John Hellstein, Thomas Heidenreich, Nidhi Handoo

PMC · DOI: 10.1007/s12105-025-01864-1 · Head and Neck Pathology · 2025-11-14

## TL;DR

A rare case of a maxillary tumor was mistaken for a tooth-related lesion, highlighting the need for histopathology to confirm the diagnosis.

## Contribution

This is the first reported case of intraosseous plexiform schwannoma in the maxilla.

## Key findings

- Radiographic imaging showed a lesion resembling an odontogenic tumor or cyst.
- Histopathology confirmed the diagnosis with characteristic schwannoma features and S100 positivity.

## Abstract

Intraosseous plexiform schwannoma of the jaw is exceedingly rare, with only one previously reported case in the maxilla. We present a case of a 27-year-old male who presented with a painless maxillary swelling. Radiographic findings reveal an unilocular radiolucent lesion mimicking an odontogenic tumor or cyst. Histopathologic examination showed a multinodular spindle cell neoplasm showing Antoni A and B patterns and diffuse S100 positivity, confirming the diagnosis of intraosseous plexiform schwannoma. Although rare, this entity can mimic an odontogenic lesion on imaging. Awareness of this possibility is crucial to expand the differential diagnosis of radiolucencies of the jaw, and this case underscores the importance of histopathologic assessment for accurate diagnosis and appropriate clinical management.

## Linked entities

- **Proteins:** S100A1 (S100 calcium binding protein A1)
- **Diseases:** odontogenic tumor (MONDO:0021192), odontogenic cyst (MONDO:0006328)

## Full-text entities

- **Genes:** S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}
- **Diseases:** odontogenic tumor or cyst (MESH:D009807), Plexiform Schwannoma (MESH:D009442), Odontogenic Lesion (MESH:D009808), Neoplasm (MESH:D009369), maxillary swelling (MESH:D008439), radiolucencies of the jaw (MESH:D007571)

## Full text

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## Figures

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Source: https://tomesphere.com/paper/PMC12618767