Abstract.A 64-year-old male presented with increased abdominal fullness and fever. Radiological examination revealed moderate ascites, a tumor with a diameter of 12.5 cm in the mesenteric region, as well as multiple tumors in the thoracic and abdominal para-aortic regions and in the left supraclavicular regions. Pathohistological findings of the biopsy specimen revealed atypical spindle cells accompanied by infiltration of lymphocytes. The plasmacytes were positive for CD68, murine double minute 2 and S-100, while they were negative for α-smooth muscle actin, cyclin-dependent kinase 4 and anaplastic lymphoma kinase. Clinically, the patient presented systemic symptoms and laboratory results indicated an elevation in the inflammatory response, while the CT and MRI findings were consistent with an inflammatory myofibroblastic tumor (IMT). Based on the clinical and histological findings, the patient was diagnosed with IMT. In total, 4 cycles of combination chemotherapy with doxorubicin and ifosfamide were administered. Tumor size reduction by 50% was achieved subsequent to the 4th chemotherapy cycle. In conclusion, successful control of this rare metastatic IMT was achieved by systemic chemotherapy.
IntroductionInflammatory myofibroblastic tumor (IMT) is a relatively rare disease that predominantly occurs in the peritoneum, retroperitoneum and lungs of young people; a previous study of 38 cases reported a median age of 8.5 years (1). This disease is histologically characterized by the proliferation of various spindle cells, which are of mesenchymal cell origin, in myxoid or collagenous stroma with prominent inflammatory infiltrates (2). IMT cells can potentially behave as locally invasive and are rarely metastatic (in <5% of cases) (3). In recent studies, a chromosomal translocation involving 2p23 and subsequent anaplastic lymphoma kinase (ALK) gene rearrangement was identified in ~50% of IMT cases (3,4). The ALK inhibitor, crizotinib, was demonstrated to be an effective therapy for ALK rearrangement-positive IMT cases.Complete surgical resection is typically conducted for localized IMTs (5); however, no standard therapeutic strategy has been established for inoperable or metastatic IMTs. In the present study, an extremely rare case of metastatic IMT that responded successfully to combination chemotherapy of doxorubicin and ifosfamide was reported.
Case reportA 64-year-old male presented with abdominal fullness, fever and severe fatigue in March 2014. These symptoms had worsened within half a month. Following a visit to a primary care doctor in March 2014, a computed tomography (CT) scan revealed a moderate volume of ascites and a mass in the middle of the abdomen (Fig. 1A). Initially, lymphoma was suspected, and oral prednisolone (60 mg/day) was administered for 3 days as a therapy with diagnostic intent; however, no relief of the symptoms was observed. Exploratory laparotomy indicated a protruded large tumor in the mesenterium and 2,500 ml of chylous ascites. Microscopic examination of the tumor biopsy sample was ...