The inflammatory myofibroblastic tumor (IMT) is a rare, idiopathic, usually benign, mass-forming disease with myofibroblastic proliferation and a varying amount of inflammatory cells. Although it can affect various organs, the biliary tract is a rare localization of primary IMT, clinically, endoscopically and radiologically imitating cholangiocarcinoma. The treatment options are based only on clinical practice experience.

A 70-year-old woman was referred to our center due to progressive fatigue, weight loss, abdominal pain, night sweats, and elevated liver enzymes. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed proximal common hepatic duct and hilar biliary strictures extending bilaterally to lobular bile ducts. Although initial clinical, endoscopic and radiological signs were typical for hilar cholangiocarcinoma, histological examination showed no signs of malignancy. In total, 8 biopsies using different approaches were performed (several biopsies from dominant stricture during ERCP and direct cholangioscopy; ultrasound-guided liver biopsy; diagnostic laparoscopy with liver and lymph node biopsies). Histological examination revealed signs of IMT, and the final diagnosis of biliary IMT was stated. Although IMT is usually a benign disease, in our case, it was complicated. All pharmacological treatment measures were ineffective. The patient still needs permanent stenting, suffers from recurrent infections and mechanical jaundice. Despite that, the patient already survived 24 mo.

IMT presenting with hilar biliary strictures is a unique diagnostic and clinical challenge as it is indistinguishable from cholangiocarcinoma, and there are no evidence-based treatment options. Our goal is to increase the understanding of this rare disease and its possible course.

Inflammatory pseudotumors of the esophagus are extremely rare, and the treatment has been controversial. Herein, we report a case of esophageal inflammatory pseudotumor with low-dose corticosteroid treatment following surgery. A 50-year-old woman with a 3-month history of progressive dysphagia and weight loss, was admitted to our hospital for examination and treatment. Esophagography and endoscopic examination revealed a mass present from the cervical esophagus to the upper thoracic esophagus with severe esophageal stricture. Ultrasound-guided fine needle aspiration cytology, boring biopsy, and mucosal incision-assisted biopsy reveal chronic inflammation, but histological diagnosis was not proven. Surgery was performed to confirm diagnosis and to relieve esophageal stricture. However, because of dense adhesions around the tumor, complete tumor resection was not achieved. Histopathological examination showed an inflammatory infiltrate with plasma cells, eosinophils, neutrophils, and lymphocytes, suggesting an inflammatory pseudotumor. After surgical resection, the esophageal stricture remained, possibly due to the residual tumor. We used a postoperative low-dose steroid treatment that resulted in complete resolution. There has not been any evident sign of recurrence for more than 2 years.

Inflammatory myofibroblastic tumors (IMT) are rare benign tumors, most commonly arising in the lungs and urinary bladder. Many etiologic factors are suspected in their development, but none have been formally demonstrated. Conventional treatment for bladder IMT is complete surgical resection by partial cystectomy or transurethral resection. We report the case of an 8-year-old girl with documented bladder IMT that resolved completely after antibiotic therapy.

Inflammatory myofibroblastic tumor (IMT) is a rare lesion of unclear pathogenesis that shows a wide, highly variable spectrum of clinical behavior. We describe the case of a 17-year-old boy with a large IMT that infiltrated the bladder, ileocecal junction, peritoneum and pelvic retroperitoneal space. The tumor was associated with extensive toughening and thickening of the bladder, and, although it showed a tendency for invasive growth, it affected mainly the bladder and adjacent tissue. To the best of our knowledge, this case report is the first to describe an IMT involving the entire bladder and several adjacent pelviabdominal organs. The bladder wall was tough and could hardly be cut by scalpel. Levels of inflammatory response markers such as C-reactive protein fell after surgery.

Inflammatory pseudotumor (IPT) of the liver is a rare benign lesion for which imaging diagnosis remains a challenge. We report the case of a 39-year-old Algerian woman, who presented epigastric pains combined with fever and jaundice. Ultrasound, CT scan and MRI showed the presence of a 10 cm-long multi-septated cystic mass of the left lobe, with peripheral enhancement. A left-hepatectomy was performed and histopathology revealed an IPT of the liver. During the 4 following years, the patient had three other recurrences of liver IPT at various locations distinct from the original, revealed by the same clinical symptoms. During these relapses, the lesions did regress thanks to a medical treatment. This observation underlines the difficulty of the diagnosis and treatment of liver IPT.

Corticosteroids are the mainstay of treatment of inflammatory pseudotumor (IPT) of the head and neck; however, involvement of the skull base and mandible can be unresponsive to steroids and require surgical resection. IPT is known to usually contain a CD20+ lymphocyte subgroup. Rituximab, a chimeric anti-CD20 antibody, has been successfully utilized in the treatment of other CD20+ diseases, including the similar idiopathic orbital inflammatory disease. This is the first report to describe successful treatment with Rituximab of a recurrent IPT of the mandible with trigeminal spread and leptomeningeal involvement with clinical and radiologic evidence demonstrating a sustained response to therapy.