INTRODUCTION Many different pathological conditions may show histo- logically multinucleated giant cells in a background of ovoid to spindle-shaped mesenchymal cells. The range of these diseases includes giant cell tumour, central and peripheral giant cell granuloma, cherubism, and the brown tumour of hyperparathyroidism. The giant cell tumour is a benign, locally aggressive neoplasm of long bones. Radiographically, it presents as a radiolucent lesion with well-defined borders involving the epiphyses. Its course is unpredictable, almost half the lesions recur if treated by curettage only, and metas- tases have been reported. 1 Peripheral and central giant cell lesions are reactive lesions of the jaws. Peripheral lesions are usually related to local factors and are supposed to arise from the con- nective tissue of the gingiva or the periosteum of the alveolar ridge, 2 and central lesions tend to develop in the anterior portion of the mandible of young female patients. 3 Some lesions grow slowly and do not recur, while others grow rapidly and the cortex perforates, and these often recur after excision. 3 Diffuse or focal bone lesions may be a consequence of hyperparathyroidism. 4 The increased production of parathyroid hormone initiates fibroblastic proliferation and osteoclastic activity that mimic the histological pic- ture seen in other giant cell lesions. Cherubism is a genetic disorder that affects children. The lesion is usually symmetrical and is characterized by bilateral expansion of the mandible or the maxilla, or both, that becomes noticeable within the first few years of life. It enlarges progressively until puberty, and gradually resolves by middle age. 5 Although it is often familial, many sporadic cases have been reported. The lesions vary greatly in size, ranging from minor lesions to large, deforming, destructive ones with massive involvement of both jaws. 6,7 Although locally aggressive cherubism has been reported, 7–9 we do not know of any patients who have died of the disease. The purpose of the present paper is to describe an aggressive, multiple, and fatal case of atypical cherubism. CASE REPORT An 8-year-old boy was referred to the Oral Diagnosis Service at the Universidade Federal de Minas Gerais in August 1969 for evaluation of a facial swelling (Fig. 1). Clinical examination showed a well-developed, cooperative boy with enlargement of the jaws and cheeks, and displacement of the eyes (Fig. 1). Intraoral examination showed generalized alveolar swelling and mobility of most teeth (Fig. 2). Radiograph examination showed multiple extensive radiolucent areas in the maxilla, body, and ramus of the mandible. Past medical and familial histories were unremarkable. Haematological and biochemical evaluation, including serum calcium and phosphorus concentra- tions and alkaline phosphatase activity, were within the reference ranges. Histopathological evaluation of an incisional biopsy speci- men showed multiple multinucleated giant cells together with ovoid to spindle-shaped cells within a fine fibrillar collagenous stroma (Fig. 3). There were deposits of haemosiderin and a few mitotic figures. In addition, no perivascular cuffing was noted. The diagnosis was cherubism. One month later he returned with a deforming swelling and was admitted to the Hospital das Clinicas, Universidade Federal de Minas Gerais (Fig. 4). Laboratory investigations had not changed. Endocrinological evaluation at that time included measurement of serum calcium and phosphorus concentrations and alkaline phosphatase acti- vity, calculation of the renal tubular resorption of phosphorus index, and exploration of the parathyroid glands; but no abnor- mality was detected. Because of the provisional diagnosis of cherubism, it was decided at that time to follow up the patient. British Journal of Oral and Maxillofacial Surgery (2002) 40, 45–48 © 2002 The British Association of Oral and Maxillofacial Surgeons doi: 10.1054/bjom.2001.0654, available online at http://www.idealibrary.com on 45 BRITISH Journal of Oral and Maxillofacial Surgery An extreme case of cherubism E. C. Silva,* P. E. A. de Souza,* D. C. Barreto,* R. P. Dias,† R. S. Gomez* *Department of Oral Surgery and Pathology, School of Dentistry; †Endocrinology Service, Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte-MG, Brazil SUMMARY. We describe an 8-year-old boy who presented with severe facial swelling. This progressed rapidly and 17 months later he died of gastrointestinal and pulmonary infections. The diagnosis was initially brown tumour associated with hyperparathyroidism, but this was revised in the light of laboratory investigations that were within the reference ranges, and normal appearance of the parathyroids on exploration to that of an extreme case of cherubism that behaved in a locally aggressive manner. © 2002 The British Association of Oral and Maxillofacial Surgeons