# A Rare and Challenging Presentation of an Inflammatory Myofibroblastic Tumour: A Case Report

**Authors:** Rohit Shirgaonkar, Srivatsa Lokeshwaran, Susmita Rakshit

PMC · DOI: 10.1002/rcr2.70232 · Respirology Case Reports · 2025-06-09

## TL;DR

A rare case of Inflammatory Myofibroblastic Tumor (IMT) in a young woman is reported, highlighting the importance of early diagnosis and multidisciplinary treatment for optimal outcomes.

## Contribution

This case report presents a rare and challenging clinical presentation of IMT and emphasizes the role of bronchoscopic interventions and genetic profiling in its management.

## Key findings

- A staged bronchoscopic debulking and stent insertion improved respiratory function and lung re-expansion in the patient.
- ALK inhibitor therapy was initiated as adjuvant treatment following the diagnosis of IMT.
- Early diagnosis and a multidisciplinary approach are critical for managing IMTs due to their potential for recurrence or metastasis.

## Abstract

Inflammatory Myofibroblastic Tumours (IMTs) are rare pulmonary neoplasms, accounting for less than 1% of lung tumours. Patients present with non‐specific symptoms and diverse radiological findings. A 32‐year‐old female presented with progressively worsening cough, haemoptysis, low‐grade fever and increasing shortness of breath. Chest X‐ray revealed a total right lung collapse. Further imaging revealed a mass obstructing the right mainstem bronchus. A staged debulking procedure using rigid bronchoscopy was performed, followed by the insertion of a silicone stent to maintain airway patency. Endobronchial growth turned out to be an Inflammatory Myofibroblastic tumour. The patient showed significant improvement in respiratory function and lung re‐expansion. Adjuvant therapy with ALK inhibitor was initiated. IMTs, while benign histologically, may recur or metastasise, requiring long‐term monitoring. Bronchoscopic interventions offer effective treatment for localised disease, while surgery is preferred for extensive tumours. Early diagnosis, genetic profiling and a multidisciplinary approach are key for optimal outcomes.

Inflammatory Myofibroblastic Tumours (IMTs) are rare pulmonary neoplasms, accounting for less than 1% of lung tumours. Patients present with non‐specific symptoms and diverse radiological findings. We present the diagnostic work‐up and treatment for a rare and challenging case of IMTs in a 32‐year‐old female. Early diagnosis, genetic profiling and a multidisciplinary approach are key for optimal outcomes.

## Full-text entities

- **Genes:** ALK (ALK receptor tyrosine kinase) [NCBI Gene 238] {aka ALK1, CD246, NBLST3}
- **Diseases:** lung tumours (MESH:D008175), lung collapse (MESH:D001261), shortness of breath (MESH:D004417), fever (MESH:D005334), cough (MESH:D003371), IMTs (MESH:D009369)
- **Chemicals:** silicone stent (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

5 references — full list in the complete paper: https://tomesphere.com/paper/PMC12149438/full.md

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Source: https://tomesphere.com/paper/PMC12149438