title: An Unusual Case of Systemic Inflammatory Myofibroblastic Tumor with Successful Treatment with ALK-Inhibitor.
abstract: Systemic inflammatory myofibroblastic tumor is an exceedingly rare entity. A 45-year-old Hispanic female presented with a 6-month history of left-sided thigh pain, low back pain, and generalized weakness. PET/CT scan revealed abnormal activity in the liver, adrenal gland, and pancreas. MRI of the abdomen demonstrated two 6-7 cm masses in the liver. MRI of the lumbar spine demonstrated lesions in the L2 to L4 spinous processes, paraspinal muscles, and subcutaneous tissues, as well as an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease. A biopsy of the liver showed portal tract expansion by a spindle cell proliferation rich in inflammation. Tumor cells showed immunoreactivity for smooth muscle actin and anaplastic lymphoma kinase 1 (ALK1). Tissue from the L5 vertebra showed a process histologically identical to that seen in the liver. FISH analysis of these lesions demonstrated an ALK (2p23) gene rearrangement. The patient was successfully treated with an ALK-inhibitor, Crizotinib, and is now in complete remission. We present the first reported case, to our knowledge, of inflammatory myofibroblastic tumor with systemic manifestations and ALK translocation. This case is a prime example of how personalized medicine has vastly improved patient care through the use of molecular-targeted therapy.
authors.affiliation: Department of Pathology, Immunology and Laboratory Medicine University of Florida College of Medicine, University of Florida College of Medicine, P.O. Box 100275, 1600 SW Archer Road, Gainesville, FL 32610-0275, USA.
authors.forename: Sanjivini V
authors.initials: SV
authors.lastname: Jacob
authors.affiliation: Department of Pathology, Immunology and Laboratory Medicine University of Florida College of Medicine, University of Florida College of Medicine, P.O. Box 100275, 1600 SW Archer Road, Gainesville, FL 32610-0275, USA.
authors.forename: John D
authors.initials: JD
authors.lastname: Reith
authors.affiliation: Lake Erie Osteopathic College of Medicine, Bradenton, FL 34211, USA.
authors.forename: Angerika Y
authors.initials: AY
authors.lastname: Kojima
authors.affiliation: Department of Pathology, North Broward Health, Pompano Beach, FL 33064, USA.
authors.forename: William D
authors.initials: WD
authors.lastname: Williams
authors.affiliation: Department of Pathology, Immunology and Laboratory Medicine University of Florida College of Medicine, University of Florida College of Medicine, P.O. Box 100275, 1600 SW Archer Road, Gainesville, FL 32610-0275, USA.
authors.forename: Chen
authors.initials: C
authors.lastname: Liu
authors.affiliation: Department of Pathology, Immunology and Laboratory Medicine University of Florida College of Medicine, University of Florida College of Medicine, P.O. Box 100275, 1600 SW Archer Road, Gainesville, FL 32610-0275, USA.
authors.forename: Lizette
authors.initials: L
authors.lastname: Vila Duckworth