Primary pulmonary mesenchymal tumors are rare causes of intrathoracic lesions in newborns. We describe a case of pulmonary spindle-cell tumor with features of infantile fibrosarcoma and discuss the differential diagnosis of spindle-cell lesions in this location. In view of further case reports of the literature, this neoplasia can best be categorized in a spectrum of fibroblastic/myofibroblastic differentiated spindle-cell tumors, with excellent prognosis. Especially in congenital lesions a favorable clinical course is to be expected after complete surgical resection. Additional radio- and/or chemotherapy is not recommended.
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