Abstract
Malignant fibrous histiocytoma (MFH) usually involves the extremities. Among MFH, primary giant cell malignant fibrous histiocytoma of the lung is extremely rare, with nonspecific symptoms. A 45-year-old woman was first diagnosed as sclerosing hemangioma due to long-term persistent cough and blood in sputum. One year later, the condition became worse and hemoptysis occurred. At the second visit, the same mass was found to be larger than the previous one. Lobectomy was performed and the primary giant cell MFH of the lung was confirmed. Renal and sacrum metastasis occurred six months after operation. The efficacy of hypofractionation radiotherapy and immuno targeted follow-up therapy is significant. At present, there is no standard treatment plan. For the patients in the early stage, surgical resection is the main treatment method; but for the patients in the middle and late stage, the treatment is relatively single. So early diagnosis and treatment are particularly important, In this paper, we first report the use of pembrolizumab combined with anlotinib in a patient with advanced giant cell MFH of the lung, with failure of routine treatment. The development of the disease had successfully delayed and satisfactory results were observed. Therefore, it is very necessary to emphasize the choice of treatment plan to improve the prognosis of patients.