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.
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. See also
Anaplastic lymphoma kinase-positive neoplasias
ALK inhibitors

References


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