Chordoid sarcoma of the soft tissue of the nape of the neck: a case with a 20 year follow-up.

A huge tumor mass arising from the soft tissue of the nape of the neck, which histologically resembled chordoma is reported. This case had a history of over 30 years after onset at around 37 years of age. With frequent recurrence and re-excision after en bloc radical resection, performed at 47 years of age, this female patient expired at the age of 66, 20 years after the first radical operation. At operation, the mass was found to be firmly attached to the dorsal soft tissue. There was no connection with any bony structure, such as the cervical vertebrae or skull. Chordoma-like histological features were unchanged throughout the next 20 years. This unusual malignant neoplasm appears to correspond to the "chordoid tumor" or "chordoid sarcoma" of soft tissue, a term coined by F.W. Stewart in 1948. Alternatively the name "chondroid chordoma" used by Heffelfinger, Dahlin and others may be employed.