ObjectiveTo improve the diagnosis and treatment of the disease, the clinical symptoms, pathological features, diagnosis and differential diagnosis, treatment and prognosis of massive lung inflammatory myofibroblastic tumor (IMT) were analyzed.MethodsA case, admitted to the Department of Respiratory and Critical Care Medicine of Xiangya Hospital, Central South University, diagnosed as massive lung IMT, was retrospectively analyzed. His clinical and chest radiological data were collected and literature which searched through CNKI, WanFang Med online and PubMed on this subject were reviewed.ResultsThe patient, middle-age male, was presented with cough, dyspnea and weight loss, whose chest radiology was characterized by a large thoracic cavity occupation. He was confirmed with massive lung IMT by several lung biopsy. From above databases, 8 cases were retrieved, including 6 articles in English and 2 articles in Chinese. In 9 cases of massive lung IMT, there were 4 males and 5 females. The age was from 1.5 to 75 years old and the average age was 28 years old. The clinical symptoms were non-specific, and chest imaging was characterized by a large thoracic occupation. One case had distant metastases with bone, adrenal gland and lymph node, and one case had distant metastasis with brain after complete surgery.ConclusionsLung massive IMT has no characteristic clinical and radiological features. And a definite diagnosis depends on pathological biopsy and immunohistochemical analysis. It needs to be differentiated from other thoracic giant tumors. The preferred treatment is complete surgical resection. The prognosis after complete resection is usually good, and probably affected by size.