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Chordoid sarcoma is a rare, distinctive neoplasm that usually occurs in the soft tissue of the extremities. Despite its striking histological resemblance to chordoma at light microscopic level, both clinical and ultrastructural differences exist and chordoid sarcoma may in fact be related to tendosynovial sarcoma. Previous reports of extraskeletal myxoid chondrosarcoma, however, show that this tumour bears a striking light microscopic and ultrastructural similarity to chordoid sarcoma, which suggests that the latter may be a variant of extraskeletal myxoid chondrosarcoma. In this report a further case of chordoid sarcoma is presented; clinical, radiological, histological and ultramicroscopic features support a tendosynovial origin.
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