Extraskeletal chondrosarcoma (ECS) is a rare malignant neoplasm of bone or soft-tissue origin, characterized by the presence of spindle cells admixed with well-differentiated cartilage or chondroid stroma. A case of ECS is reported in a 102-year-old woman who presented with a painful swelling of 2 cm in the left buccal vestibular area. Orthopantomography was insignificant. Biopsy and histopathological examination revealed a tumor composed of an undifferentiated small round cell component that surrounded a myxoid matrix of malignant cartilage. Immunohistochemical studies showed the tumor cells to be positive for nuclear S-100 protein immunostaining, focally positive for vimentin and synaptophysin, and negative for epithelial membrane antigen, desmin, chromogranin, Leu-7, glial fibrillary acid protein, actin muscle-specific, cytokeratin, carcinoembryonic antigen, and CD99 (MIC2). The proliferative index (MIB-1) was 20%. The tumor was treated by surgery with wide margins. There was no evidence of disease at one-year follow-up. This report presents a very rare case of ECS of the left buccal mucosa of the maxilla, and describes the histological characteristics and the immunoprofile.
Extraskeletal Myxoid Chondrosarcomaof the Left Buccal Mucosa
ANGIERO, FRANCESCA
2012-01-01
Abstract
Extraskeletal chondrosarcoma (ECS) is a rare malignant neoplasm of bone or soft-tissue origin, characterized by the presence of spindle cells admixed with well-differentiated cartilage or chondroid stroma. A case of ECS is reported in a 102-year-old woman who presented with a painful swelling of 2 cm in the left buccal vestibular area. Orthopantomography was insignificant. Biopsy and histopathological examination revealed a tumor composed of an undifferentiated small round cell component that surrounded a myxoid matrix of malignant cartilage. Immunohistochemical studies showed the tumor cells to be positive for nuclear S-100 protein immunostaining, focally positive for vimentin and synaptophysin, and negative for epithelial membrane antigen, desmin, chromogranin, Leu-7, glial fibrillary acid protein, actin muscle-specific, cytokeratin, carcinoembryonic antigen, and CD99 (MIC2). The proliferative index (MIB-1) was 20%. The tumor was treated by surgery with wide margins. There was no evidence of disease at one-year follow-up. This report presents a very rare case of ECS of the left buccal mucosa of the maxilla, and describes the histological characteristics and the immunoprofile.
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