# Inflammatory myofibroblastic tumour (IMT)

Source: https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/  
OnCo record `inflammatory-myofibroblastic-tumour` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

IMT is a rare tumour, grouped with the sarcomas, of spindle cells mixed with inflammatory cells, most often in the lung or abdomen of children and young adults. Surgery cures most, and about half carry an ALK gene fusion, so the ALK-blocking pill crizotinib is approved for those that cannot be removed, one of the first targeted approvals for a childhood solid tumour.

## Summary

IMT is an intermediate-grade mesenchymal neoplasm of myofibroblastic spindle cells with a plasma cell and lymphocyte infiltrate. Around half harbour ALK rearrangements with diverse partners (TPM3, TPM4, CLTC, RANBP2 and others); most ALK-negative cases carry ROS1, NTRK3, PDGFRB or RET fusions, so nearly every IMT has a druggable kinase fusion. The epithelioid inflammatory myofibroblastic sarcoma variant, driven by RANBP2-ALK or RRBP1-ALK, is aggressive and intra-abdominal. Presentation ranges from an incidental lung mass to fever, weight loss and anaemia from cytokine release.

Complete surgical resection is curative for most patients and remains first line. For unresectable, recurrent or metastatic ALK-positive IMT, crizotinib produced objective responses in the EORTC 90101 CREATE phase 2 (Lancet Respir Med 2018) and in the Children's Oncology Group ADVL0912 study, leading to FDA approval in July 2022 for adults and children aged one year and older, the first approval of an ALK inhibitor for a non-lung indication in children. Second-generation ALK inhibitors (alectinib, ceritinib, lorlatinib) are used at resistance, and ROS1 or NTRK fusion cases respond to crizotinib, entrectinib, larotrectinib or repotrectinib respectively.

Open questions are treatment duration in children who reach complete response, whether neoadjuvant kinase inhibition can make surgery less mutilating, and how to manage the ALK-negative, fusion-negative minority.

## Fields

- Kind: Cancer
- Last checked: 2026-09-10
- Also known as: IMT; Pulmonary inflammatory myofibroblastic tumour; Inflammatory pseudotumour; Epithelioid inflammatory myofibroblastic sarcoma
- Tags: nci-coverage; rare; sarcoma; paediatric
- Group: sarcoma
- Burden: Rare at any age but the most common primary lung tumour of children; also arises in the mesentery, bladder and soft tissue.
- Subtypes: Classic IMT (ALK-rearranged, about half); ALK-negative IMT (ROS1, NTRK3, PDGFRB, RET fusions); Epithelioid inflammatory myofibroblastic sarcoma (RANBP2-ALK or RRBP1-ALK)
- Biomarkers: ALK immunohistochemistry and FISH or RNA fusion panel; ROS1, NTRK, PDGFRB, RET fusions in ALK-negative tumours; Inflammatory markers (anaemia, raised CRP) as systemic markers; Site and resectability

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/inflammatory-myofibroblastic-tumour/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#overview [4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#treating-it [3 settings, 2 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#evidence [1 key paper, 5 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#science [7 targets, 2 pathways]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/#living-with-it [14 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/coming/ [8 medicines, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/data/ [34 connected records]

## Standard of care

- Resectable: Complete surgical excision; no adjuvant therapy in most cases, surveillance imaging for recurrence. ([Limb-salvage surgery and endoprosthetic reconstruction](https://onco.cc/technologies/limb-salvage-surgery/))
- Unresectable, recurrent or metastatic, ALK-positive: Crizotinib (FDA approval July 2022, children 1 year and older and adults); alectinib, ceritinib or lorlatinib at progression. ([Crizotinib](https://onco.cc/drugs/crizotinib/), [Alectinib](https://onco.cc/drugs/alectinib/), [Lorlatinib](https://onco.cc/drugs/lorlatinib/), [Ceritinib](https://onco.cc/drugs/ceritinib/))
- Unresectable, ALK-negative with other fusion: Match to fusion: entrectinib or crizotinib for ROS1, larotrectinib or entrectinib for NTRK, imatinib for PDGFRB; steroids or NSAIDs for symptom control in indolent disease. ([Entrectinib](https://onco.cc/drugs/entrectinib/), [Larotrectinib](https://onco.cc/drugs/larotrectinib/), [Imatinib](https://onco.cc/drugs/imatinib/), [Repotrectinib](https://onco.cc/drugs/repotrectinib/))

## State of the art

- IMT is close to a fully genotype-directed disease: nearly every tumour carries a kinase fusion with an approved inhibitor.
- The 2022 crizotinib approval used adult (CREATE) and paediatric (COG ADVL0912) data together, a model for age-agnostic approvals under the RACE for Children Act.
- Epithelioid inflammatory myofibroblastic sarcoma, once uniformly lethal, responds to ALK inhibition and is managed with sequential ALK inhibitors.
- Surgery remains curative for the majority; drugs are for the minority with unresectable disease.

## Open problems

- How long to continue ALK inhibition in children with complete response, and whether surgery after response can allow stopping.
- The fusion-negative minority: RNA sequencing to find drivers.
- Rare aggressive epithelioid variants that develop resistance mutations to sequential ALK inhibitors.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Inflammatory_myofibroblastic_tumour
- NCI PDQ: childhood soft tissue sarcoma (includes IMT): https://www.cancer.gov/types/soft-tissue-sarcoma/hp/child-soft-tissue-treatment-pdq
- FDA approval of crizotinib for ALK-positive IMT (archived copy): https://web.archive.org/web/20260213082319/https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-crizotinib-alk-positive-inflammatory-myofibroblastic-tumor
- EORTC 90101 CREATE (Lancet Respir Med 2018): https://doi.org/10.1016/S2213-2600(18)30116-4

## Connected records

- cancers: [Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours)](https://onco.cc/cancers/pleuropulmonary-blastoma/), [Dermatofibrosarcoma protuberans](https://onco.cc/cancers/dermatofibrosarcoma-protuberans/), [Desmoid tumour](https://onco.cc/cancers/desmoid-tumour/), [Gastrointestinal stromal tumour (GIST)](https://onco.cc/cancers/gist/), [Perivascular epithelioid cell tumour (PEComa)](https://onco.cc/cancers/pecoma/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/), [Tenosynovial giant cell tumour (TGCT)](https://onco.cc/cancers/tenosynovial-giant-cell-tumour/)
- technologies: [Limb-salvage surgery and endoprosthetic reconstruction](https://onco.cc/technologies/limb-salvage-surgery/), [RNA sequencing & expression profiling](https://onco.cc/technologies/rna-seq/), [Small-molecule kinase inhibitors](https://onco.cc/technologies/kinase-inhibitors/)
- targets: [ALK](https://onco.cc/targets/alk/), [NTRK](https://onco.cc/targets/ntrk/), [PDGFRA](https://onco.cc/targets/pdgfra/), [RET](https://onco.cc/targets/ret/), [ROS1](https://onco.cc/targets/ros1/)
- drugs: [Alectinib](https://onco.cc/drugs/alectinib/), [Ceritinib](https://onco.cc/drugs/ceritinib/), [Crizotinib](https://onco.cc/drugs/crizotinib/), [Entrectinib](https://onco.cc/drugs/entrectinib/), [Imatinib](https://onco.cc/drugs/imatinib/), [Larotrectinib](https://onco.cc/drugs/larotrectinib/), [Lorlatinib](https://onco.cc/drugs/lorlatinib/), [Repotrectinib](https://onco.cc/drugs/repotrectinib/)
- companies: [Children's Oncology Group (COG)](https://onco.cc/companies/childrens-oncology-group/), [EORTC](https://onco.cc/companies/curie-nki-eortc/), [Pfizer (incl. Seagen)](https://onco.cc/companies/pfizer/)
- pathways: [RAS / RAF / MEK / ERK (MAPK)](https://onco.cc/pathways/ras-mapk/), [Receptor tyrosine kinase activation](https://onco.cc/pathways/rtk-activation/)
- terms: [Gene fusion](https://onco.cc/terms/gene-fusion/), [RACE for Children Act](https://onco.cc/terms/race-for-children-act/), [Rare cancers](https://onco.cc/terms/rare-cancers/)
- bottlenecks: [Rare and paediatric cancers without markets](https://onco.cc/bottlenecks/b-rare-cancers/)
- key papers: [Crizotinib in patients with advanced, inoperable inflammatory myofibroblastic tumours with and without anaplastic lymphoma kinase gene alterations (European Organisation for Research and Treatment of Cancer 90101 CREATE): a multicentre, single-drug, prospective, non-randomised phase 2 trial](https://onco.cc/key-papers/paper-schoffski-lancet-respir-med/)

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JSON: https://onco.cc/api/v1/entities/inflammatory-myofibroblastic-tumour.json