[Hand-Schüller-Christian disease with tumor localization in the posterior fossa].

A 19-year-old girl had been suffering since the age of 4 from Hand-Schüller-Christian disease. The condition was diagnosed by biopsy of a cranial bone lacuna. Later on, she developed diabetes insipidus and bilateral exophthalmos, both being typical signs of Hand-Schüller-Christian disease. At the age of 14, an intracranial tumour in the posterior cerebral fossa was discovered by computerized tomography, but in view of her parent's refusal of surgery, she was operated upon only 4 years later for obstructive hydrocephalus with intracranial hypertension. Two years after this operation, she presented with neurological signs of compression of the medulla oblongata, including tetraparesis and respiratory disorders, and the tumour was resected. Following a brief improvement she died of major hydroelectrolytic disorders due to her irreducible unstable diabetes insipidus. A review of the literature on Hand-Schüller-Christian disease and its rare association with intracranial tumours is analysed. The occurrence of such tumours raise important therapeutic problems and make the prognosis considerably worse.