# Sinonasal Myxoma in an Infant: Observations on Its Distinctiveness and a Discussion on Potential Reclassification As Infantile Intraosseous Myxoid Desmoid Fibromatosis

**Authors:** Ryoichiro Kashiwagi, Hayato Maruguchi, Nibu Ken-ichi, Hiroto Terashi, Tadashi Nomura

PMC · DOI: 10.7759/cureus.65933 · Cureus · 2024-08-01

## TL;DR

This paper discusses a rare case of sinonasal myxoma in an infant and suggests it may be a distinct disease, possibly reclassified as a type of fibromatosis.

## Contribution

The paper proposes a potential reclassification of sinonasal myxoma as infantile intraosseous myxoid desmoid fibromatosis based on distinct clinical and genetic features.

## Key findings

- The case showed distinct clinical and histological features compared to odontogenic myxomas.
- Immunohistochemical staining for β-catenin was positive in this case, unlike in odontogenic myxomas.
- SNMs share genetic mutations with desmoid tumors, suggesting a different disease entity.

## Abstract

Myxomas, when they manifest in the paranasal sinuses and/or maxillae of infants, are classified as sinonasal myxomas (SNMs). We present a case of SNM in the maxilla of a 15-month-old infant. Following the initial surgical intervention, the patient unfortunately experienced a recurrence of the condition. However, a subsequent surgery employing marginal excision was performed, and since then, no further recurrence has been reported. SNM exhibits consistent clinical features and histological characteristics that are distinct from those of odontogenic myxomas. Furthermore, in this case, immunohistochemical staining was positive for β-catenin, whereas odontogenic myxomas are generally negative for β-catenin staining. Another study reported that SNMs share genetic mutations with desmoid tumors, which are not observed in odontogenic myxomas. This suggests that this entity is distinct from odontogenic myxomas, leading us to propose that it may indeed represent a separate disease entity. This fact may lead to the reclassification of the disease and, ultimately, to changes in treatment strategies.

## Linked entities

- **Proteins:** ctnnb1.S (catenin beta 1 S homeolog)

## Full-text entities

- **Genes:** CTNNB1 (catenin beta 1) [NCBI Gene 1499] {aka CTNNB, EVR7, MRD19, NEDSDV, armadillo}
- **Diseases:** Myxomas (MESH:D009232), Myxoid Desmoid Fibromatosis (MESH:D018222), desmoid tumors (MESH:C535944)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

_Full body text omitted from this summary view._ Fetch the complete paper as Markdown: https://tomesphere.com/paper/PMC11365093/full.md

## Figures

13 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11365093/full.md

## References

36 references — full list in the complete paper: https://tomesphere.com/paper/PMC11365093/full.md

---
Source: https://tomesphere.com/paper/PMC11365093