Abstract

WITH the exception of leukamia, the vast majority of malignant tumours occurring in children consist of those developing in the central or autonomic nervous systems, the kidneys and the lymph glands (Campbell, Gainsford, Paterson and Steward, I96I). The metastases from these neoplasms are most frequently observed in the liver, the lungs and the skeleton, excluding the jaws. Among the rarest of sites in which malignant tumours have been recorded in children might be listed the ovaries, the thyroid, the salivary glands, the testes, the extra-dural space in the spine, the orbit, excluding the eye, and, above all, the jaws. Yet it is in these very sites that tumour deposits are frequently observed in the tumour syndrome about to be described and discussed. Moreover, tumours are not only commonly found in one or other of these unusual sites, but many of these structures are frequently concomitantly involved in one patient. To add to the bizarre behaviour of this tumour syndrome the lungs are usually spared in a cancer that might appear to be blood-disseminated, and lymph-node enlargement or splenic involvement is exceptional in what has been shown to be a lymphoid tumour. These are but some of the peculiarities of this unusual and yet distinctive syndrome. The main clinical features have already been described (Burkitt, 1958; Burkitt and O'Conor, I96I; Burkitt and Davies, I96I) and a detailed description of the pathology has been published (O'Conor, I96I). Attention has been drawn to certain radiological aspects of the jaw tumours (Davies and Davies, I960), to the unusual geographical distribution (Burkitt, 1958; Burkitt and O'Conor, I96I) and to the suggested significance of this limited distribution (Burkitt and Davies, I96I; Burkitt, I962). A brief review of the recorded observations on this tumour syndrome will be given before discussing its possible significance as a contribution to knowledge of cancer retiology.