Many sources recommend that localized IMT be treated with total resection of all<unk> ous tissues. Localized<unk>  recurrences may likewise be treated by total resection. There is little support for adding radiation or systemic chemotherapy to this regimen.<unk> s that are not resectable, occur in inaccessible sites, are multifocal, or have metastasized are treated with aggressive therapeutic regimens. In one retrospective study, 59 patients (all <25 years old) with IMT where treated with surgery. 31 had no residual disease post-surgery; 4 of these patients had local relapses, 3 of whom were again treated surgically and 1 with surgery plus chemotherapy. Nineteen had microscopic residual disease post-surgery. Post-surgery, 6 of these patients were treated with high-dose corticosteroids; 5 with vinblastine + methotrexate chemotherapy; 3 with inhibitors of ALK; 2 with vinorelbine + low-dose cycloheximide or Ifosfamide-based chemotherapy; and 1 with cyclophosphamide + vinchristine + actinomycin D chemotherapy. Of these 19 patients, 4 had complete responses, 8 partial responses, 5 stable disease, and 2 progressive disease. Nine patients had macroscopic disease post-surgery; 5 of these patients received vincristine + methotrexate; 2 received ALK inhibitors; and 1 each received either high-dose corticosteroids or Ifosfamide-based chemotherapy. Of these 9 patients, none had complete responses; 6 had partial responses; 1 had steady disease; and 2 had progressive disease. There were no deaths among the 59 patients. The various drug regimens showed little differences in effectiveness although patients treated with ALK inhibitors trended to have longer response times. Another retrospective study evaluated the response of 17 patients (aged 22–46 years; median age 32 years) with advanced disease to Adriamycin-based chemotherapy regimens, i.e. Adriamycin alone, Adriamycin + Ifosfamide, or Adriamicin + other chemotherapy drugs. No patients had a complete response, 8 patients had partial responses, 4 patients had steady disease, and 5 patients had progressive disease. Progression-free survival and overall survival times for the group were 6.6 and 21.2 months, respectively. The study also evaluated 9 patients (aged 12–31 years, median age 16) treated with methotrexate + vinblastine, methotrexate + vinorelbine, or vinblastine + vinorelbine; 2 patients attained complete responses, 3 attained partial responses, 2 had steady disease, and 2 had progressive disease; this groups' progression-free time was not reached while its overall survival time was 83.4 months. The study concluded that the Adriamycin-based and methotrexate/vinblastine/vinorelbine regimens have a high degree of activity in IMT. Due to the low numbers of patients evaluated, no conclusions could be made on which regimen(s) were most effective. In addition to the report that compared the effect of ALK-inhibitors to other therapy regimens detailed in the previous paragraph, several reports have focused primarily on small numbers of IMT patients treated with an ALK inhibitor. The European Organisation for Research and Treatment of Cancer evaluated the effect of the ALK-inhibitor, crizotinib, on 12 ALK-positive IMT adults who had persistent and/or metastatic disease following surgical and/or drug treatment: 2 patients had complete responses, 4 had partial responses, 5 had steady disease, and none had progressive disease; 9 of these patients had at least 1 year of progression-free survival but one patient died of the disease. A review of previously published IMT patients of all ages found that: 1) 4 patients (3 with unifocal, 1 with multifocal disease) without prior treatment had complete responses to crizotinin; 2) 2 patients (1 with unifocal, 1 with multifocal disease) that had persistent disease after surgery and previously treated with chemotherapy had partial responses to crizotinib while 1 patient previously treated with a corticosteroid, prednisone, continued to have progressive disease on crizotinib; and 3) 6 patients with progressive disease after surgery (due to multifocal or unifocal disease in inaccessible sites) had complete responses (2 cases), partial responses (2 cases), stable disease (1 case), or progressive disease (1 case) in response to crizotinib. Two of these crizotinib-treated patients with progressive disease had near complete responses to second generation ALK inhibitors Another study reviewed 29 pediatric patients (age 15 months to 17 years) who were treated with an ALK inhibitor followed by surgical removal of the<unk>  (5 cases), surgical<unk>  removal followed by an ALK inhibitor (12 cases), or an ALK inhibitor without surgery. Twelve patients had complete responses, 14 partial responses, 2 stable disease, and 2 recurrences after finishing ALK inhibitor treatment. The latter two patients obtained complete responses to retreatment with crizotinib (1 case) or a second generation ALK inhibitor, ceritinib (1 case). A study of 14 pediatric patients with metastatic or inoperable ALK-positive IMT were treated with crizotinib: 5 patients obtained complete responses, 7 partial responses, and 2 stable disease. Over the study period (2–63 months), no patient developed progressive disease. Numerous Medical history studies have had similar results in treated IMT with ALK inhibitors. However, ALK inhibitors have serious side effects; in on study, crizotinib treatment was associated with pneumonia, fever of unknown cause, heart attack, sepsis, abdominal abscess, acute renal insufficiency, and the development of an abnormal EKG (i.e. QT prolongation). Entrectinib, a tyrosine kinase inhibitor that is active on ROS1 and NRTK as well as AKT, has shown clinically significant activity in individual cases of patients with IMT expressing ROS1, NRTK, and/or an ALK fusion proteins.
Inflammatory myofibroblastic tumour
Reticulohistiocytoma
Plexiform angiomyxoid myofibroblastic tumor
Giant-cell reticulohistiocytoma
Adenomatoid tumor
Angiomatoid fibrous histiocytoma
Multinucleate cell angiohistiocytoma
Epithelioid sarcoma