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Inflammatory Myofibroblastic Tumor of the Liver: Challenges in the Preoperative Diagnosis and Treatment

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A 48-year-old Chinese woman was presented to our department with a hepatic mass on routine physical examination. She was almost asymptomatic. She denied previous radiotherapy or industrial chemical exposure. Physical… Click to show full abstract

A 48-year-old Chinese woman was presented to our department with a hepatic mass on routine physical examination. She was almost asymptomatic. She denied previous radiotherapy or industrial chemical exposure. Physical examination was unremarkable. Liver function was within reference range. Serological testing for tumor markers, hepatitis B, and C were also negative. Magnetic resonance imaging (MRI) of the upper abdomen showed a 3.2 × 3.5 cm, appearing hypo-intense relative to the liver on T1-weighted images (Fig. 1a) and hyper-intense on T2-weighted images (Fig. 1b). Due to the similar appearance, hepatocellular adenoma, intrahepatic cholangiocarcinoma, or hepatic alveolar echinococcosis was considered for preoperative diagnosis. The patient eventually underwent a liver resection of segment VII. Microscopically, the hepatic lesion exhibited a mixture of chronic inflammatory cells and spindle cells (Fig. 2a). Immunohistochemical analysis showed tumor cells positive for smooth muscle actin (Fig. 2b), anaplastic lymphoma kinase-1 (Fig. 2c), and negative for CD34, CD99, EMA, PCK, supporting the diagnosis of inflammatory myofibroblastic tumor of the liver (IMTL). Postoperative recovery of the patient was well. IMTL is a rare neoplasm and the accurate incidence is not known. The etiology and pathogenesis of IMTL are not completely clear. The clinical presentation includes nonspecific and variable clinical presentation, ranging from asymptomatic to fever, chills, nausea, weight loss, back pain, or right-upper-quadrant abdominal pain. There is no known unique diagnostic clinical, laboratory, or radiological features. Thus, the diagnosis of IMTL is still difficult till now. Percutaneous needle liver biopsy may be able to improve the chance of preoperative diagnosis. Management remains controversial, including surgery, radiotherapy, steroids, chemotherapy, nonsteroidal anti-inflammatory drugs, and antibiotics. IMTL could abate spontaneously and is typically treated conservatively, with a good prognosis. If the diagnosis can be established pre-operatively, antibiotics and nonsteroidal anti-inflammatory drugs are sufficient in certain cases. Recently, anaplastic lymphoma kinase (ALK) inhibitors may provide therapeutic benefit in patients with ALKexpressing IMT. Most authors suggest surgical resection should be the preferred treatment. A study revealed a significant recurrence rate (24.5%), with a low risk of distant metastases. The value of resection has been demonstrated in patients in whom a definitive histological diagnosis could not be made preoperatively or intraoperatively by frozen section. If the diagnosis can be established pre-operatively, radical and extensive surgery may be avoided or at best less radical, thus reducing the surgical complications. Multicenter studies based on the analysis of more effective and practical diagnostic tools are required to establish better programs and subsequently guide the preoperative diagnosis of IMTL. Further studies focusing on the etiology of IMTL to improve diagnosis are also required. * Fu-Yu Li [email protected]

Keywords: preoperative diagnosis; etiology; diagnosis; liver; inflammatory myofibroblastic; tumor

Journal Title: Journal of Gastrointestinal Surgery
Year Published: 2017

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