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.
In rare cases, the tumors have spontaneously regressed.Individuals with IMT present with a wide range of symptoms (e.g. pain, swelling, a mass, organ dysfunction, etc.) depending on the tumor location(s). Up to 1/3 of these individuals have symptoms of systemic inflammation such as fever, chills, night sweats, and weight loss. Rare cases of IMT have developed in individuals with: a) organizing pneumonia; b) infection by Mycobacterium avium intracellulare or Corynebacterium equi (pneumonia-causing bacteria); Campylobacter jejuni (causes gastroenteritis); Lysinibacillus sphaericus (previously termed Bacillus sphaericus, a rare cause of lung infections and sepsis); Coxiella burneti (causes Q fever); Epstein–Barr virus (causes infectious mononucleosis and Epstein–Barr virus-associated lymphoproliferative malignant diseases); and E. coli-related occlusive phlebitis of intrahepatic veins; or c) previous abdominal surgery; trauma; ventriculoperitoneal shunt in the brain; radiation therapy; and corticosteroid usage. The relationship (i.e. cause or merely association) of these disease relationships to IMT is unknown. Molecular abnormalities
The neoplastic cells in 50–60% of IMT and all cases of EIMS express an abnormal ALK protein made by a somatic recombination in the ALK gene. ALK, i.e. anaplastic lymphoma kinase (also termed protein kinase B), is produced by the ALK gene. In IMT, the ALK gene has merged with a gene located at another site on the same or different chromosome to form a chimeric gene consisting of a part of the new gene and a part of the ALK gene coding for ALKs activity. This chimeric gene overproduces a fusion protein with excessive ALK activity.