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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Resectable | Complete surgical excision; no adjuvant therapy in most cases, surveillance imaging for recurrence. | not mapped |
| Unresectable, recurrent or metastatic, ALK-positive | Crizotinib (FDA approval July 2022, children 1 year and older and adults); alectinib, ceritinib or lorlatinib at progression. | NCCN Category 2A |
| 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. | not mapped |