Primary Low-Grade Fibromyxoid Sarcoma of the Left Upper Lobe Treated With Uniportal Video-Assisted Thoracoscopic Lobectomy.

Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor that usually affects young adults and arises on the proximal extremities and trunk. Primary pulmonary LGFMS is an extremely rare entity. We reported a postsurgical diagnosis of a primary lung LGFMS in a patient with single pulmonary nodule located in the left upper lobe. A uniportal thoracoscopic lobectomy with mediastinal lymph node dissection was performed. The histopathological report supposed the hypothesis of a primary lung LGFMS. The immunohistochemical investigation yielded intense and diffuse positivity of the neoplastic cells for MUC-4. Next Generation Sequencing analysis showed the presence of EWSR1-CREB3L2 gene fusion that was previously reported in literature only in a single case. Histological and molecular diagnosis for LGFMS is challenging and therefore a dedicated pathologist opinion is warranted. Surgery is the gold-standard treatment for primary lung LGFMS and this is the first report about uniportal video-assisted thoracoscopic lobectomy performed for such disease.