# A novel entity of HIPK2::YAP1 pulmonary fibromatosis

## TL;DR

This paper reports a rare case of pulmonary fibromatosis with a HIPK2-YAP1 fusion, highlighting the importance of molecular testing for accurate diagnosis.

## Contribution

The paper identifies HIPK2-YAP1 fusion as a novel molecular entity in pulmonary fibromatosis.

## Key findings

- A 66-year-old male with PF was found to have a HIPK2-YAP1 fusion via NGS.
- Molecular detection confirmed the diagnosis after inconclusive immunohistochemical results.
- The patient remains recurrence-free 14 months post-surgery.

## Abstract

Pulmonary fibromatosis (PF) is a specific variant of fibromatosis, which is rarely reported occurring in the lung. PF with HIPK2-YAP1 fusion was a novel entity.

In this report, a 66-year-old male with PF had been smoking over 40 years. Multiple cords and small nodules in both lungs had been detected in a health examination two years earlier at our hospital. But approximately twofold enlarged in the lingual segment of the upper lobe in the left lung were disclosed in this year. Immunohistochemical analysis demonstrated that the vimentin and β-Catenin were positive in the largest nodule. After underwent a DNA/RNA panel next-generation sequencing (NGS), missense mutations and HIPK2-YAP1 fusion were found in this sample. Ultimately, the case diagnosis as PF with HIPK2-YAP1 fusion after multidisciplinary treatment. Currently, the patient is doing well and recurrence-free at 14 months post-surgery.

It’s difficult for patients with complex morphology to make accurate diagnosis solely based on morphology and immunohistochemistry. But molecular detection is an effective method for further determining pathological subtypes.

The online version contains supplementary material available at 10.1186/s12890-024-03026-5.