Annotation Detail

Information
Associated Genes
ALK
Associated Variants
ALK RANBP2-ALK
Associated Disease
inflammatory myofibroblastic tumor
Source Database
CIViC Evidence
Description
ALK-rearrangements are seen in 50% of inflammatory myofibroblastic tumor (IMT) cases. A 44 year old man was diagnosed with IMT. He was treated with surgery and catheter placement for administration of cisplatin, doxyrubicin and mitomycin C. Break-apart FISH showed ALK rearrangement, and immunohistochemical staining was characteristic of RANBP2 rearrangement. After further chemotherapy along with maintenance imatinib, crizotinib was started at 200mg twice daily, and a maximal partial response was achieved 5 months later, with regrowth occurring 2 months later. After further surgery, crizotinib was restarted at 250mg twice daily and almost 2 years later the patient was in complete radiographic remission. Another patient with non-ALK rearranged IMT was administered crizotinib in this study but did not respond.
Variant Origin
somatic
Variant Origin
Somatic
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/1244
Gene URL
https://civic.genome.wustl.edu/links/genes/1
Variant URL
https://civic.genome.wustl.edu/links/variants/514
Rating
4
Evidence Type
Predictive
Disease
Inflammatory Myofibroblastic Tumor
Evidence Direction
Supports
Drug
Crizotinib
Evidence Level
C
Clinical Significance
Sensitivity/Response
Pubmed
20979472
Drugs
Drug NameSensitivitySupported
CrizotinibSensitivitytrue