Low-grade pulmonary myxoid sarcoma of uncertain histogenesis

The present report describes an unusual case of low-grade pulmonary sarcoma with extensive myxoid change in a 60-year-old man. During 30 months, the tumor enlarged gradually and thereafter rapidly to 9 cm. Preoperative biopsy and cytology gave negative results. The resected mass, located at the periphery of the right upper lobe, was well circumscribed and showed gelatinous without necrosis or hemorrhage. Histologically, the tumor was composed of slightly atypical, spindle-shaped or stellate cells, which were loosely distributed within a prominent myxoid stroma. Epithelial differentiation was not seen. Lacunar structures were occasionally evident, but no cartilaginous matrix was seen. Mitotic figures were infrequent. Immunohistochemistry failed to clarify the nature of the neoplastic cells except vimentin positivity. Histochemically, the myxoid ground substance was composed of hyaluronic acid and acid mucopolysaccharide. Electron microscopy revealed no specific differentiation other than aggregates of filaments, which were seen in a number of neoplastic cells. Flow cytometric analysis of the neoplastic cells revealed a diploid pattern. These findings indicated that the neoplasm was a low-grade myxoid sarcoma; however, a definite diagnosis could not be made. The tumor might have been a variant of extraskeletal myxoid chondrosarcoma, especially considering the histochemical results.