# Plexiform’s perplexities: a tale of two plexiform neurofibromas

## TL;DR

This paper discusses two cases of plexiform neurofibroma, a rare nerve tumor, highlighting differences in their clinical presentation and treatment.

## Contribution

The paper presents two distinct clinical cases of plexiform neurofibroma with contrasting diagnostic features and management approaches.

## Key findings

- One patient showed typical cutaneous changes aiding diagnosis, while the other lacked these features.
- Histopathology and immunohistochemistry confirmed the diagnosis in both cases.
- Surgery remains a key treatment for disfiguring plexiform neurofibromas with the need for postoperative follow-up.

## Abstract

Plexiform neurofibroma (PF) is a rare benign variant belonging to a subtype of neurofibromatosis type 1 that forms bulging or deforming masses arising from the peripheral nerve sheath. These masses involve surrounding connective tissue or dermal layers, leading to multiple cutaneous changes and certain characteristic appearances. It is these appearances that aid in the diagnosis of PF. We have encountered two distinct patients diagnosed with this disorder. While one patient was clinically and pathologically confirmed for PF, the other had no characteristic cutaneous changes. The diagnosis was made with postoperative histopathology and confirmed with an immunohistochemical examination. There are various modalities in the management of PFs, with surgery being a mainstay in the treatment of disfiguring large PFs, especially in resource-restrained settings. In view of high recurrence rates, postoperative clinical follow-up is a must. This paper describes these patients’ typical and atypical clinical presentation and subsequent management.