SUMMARYA case of cervical chordoma in a man of 27 years is described. The clinical features were of progressive spinal cord compression. Treatment was by transoral decompression of the second cervical vertebra and postoperative radiotherapy. The result was favourable and remained so at follow-up after 27 months. The relative merits and scope of the various approaches to the upper cervical vertebrae and the role of radiotherapy and chemotherapy are discussed. CHORDOMA is a rare tumour of the axial skeleton whose clinical features bring it within the scope of the ophthalmologist, otolaryngologist, neurosurgeon, orthopaedic surgeon and general surgeon.Since its identification in 1856 by Luschka, about 1000 cases have been reported and about 10-15 new cases are described each year. The most comprehensive recent review (Utne and Pugh, 1955) covers 505 cases from the world literature and 72 cases from the Mayo Clinic.Clinical features Statistically, chordoma accounts for 5 per cent of all benign bone tumours (Coley, 1960), less than 2 per cent of all nasopharyngeal tumours (Osborn, 1975) and less than 1 per cent of all central nervous system neoplasms (Poppen and King, 1952). The origin of a chordoma is from the notochord or its remnants (Wright, 1967).Any age group may be affected, though the peak incidence is in middle age. The sex distribution is not clear-cut, the series varying in their results. Most chordomas affect the craniocervical or the sacrococcygeal regions.The pathology and histology have been well described by Steegman (1971) and the ultrastructure by Gessaga et al. (1973). Metastasis is rare in head and neck chordoma but it may be local to lymph nodes or within the central nervous system (Holzner, 1954). Histology shows malignant features, but these correlate poorly with clinical behaviour.The diagnosis depends on radiological and histological findings. The clinical picture is one of a local space-occupying lesion arising in bone, usually without local or distant metastasis. Systemic features are undiagnostic, though lassitude and weight loss are fairly frequent. The results of haematological, serological and biochemical investigations are usually normal. There are no specific radiological changes that differentiate a chordoma from other bone tumours. Plain radiography with tomography and contrast studies are of great benefit in defining spread and involvement. Angiography is useful in that it not only excludes other pathological conditions, but also gives some indication as to the vascularity of the tumour.A biopsy may be a prelude to or accompany definitive surgery. Much has been made of the difficulty in diagnosis (Higinbotham et al., 1967), but given a good specimen, the features are unmistakable.
TreatmentIncreasing optimism has been shown concerning the role of radiotherapy (Rosenqvist and Saltnan, 1959;Windeyer, 1959) as operation alone has proved inadequate. The latest reviews (Pearlman and Friedman, 1970; Pearlman et a]., 1972; Phillips and Newman, 1974) suggest that a combination of radical op...