Primary dedifferentiated chondrosarcoma of the lung

Extraskeletal dedifferentiated chondrosarcomas (DC) are uncommon tumors that predominantly occur in the head and neck. Herein, we describe a case of pulmonary DC detected in a patient with systemic sclerosis and presenting with a symptomatic large pleural effusion and a pulmonary lobar consolidation on chest imaging. To the best of our knowledge, this is the first report of pulmonary chondrosarcoma involving the pleura and occurring in association with systemic sclerosis. Moreover, this is the second report of a dedifferentiated variant among all reported cases of primary lung chondrosarcoma.