[Gorham-stout disease]

Abstract

Introduction: Gorham-Stout disease is a rare entity characterised by a massive osteolysis and typically starts monocentrically in an isolated bone. Concerning the pathogenesis, haemangiomatosis and an overstimulation of osteoclasts are discussed which cause a progressive destruction of the affected skeleton and involve neighbouring soft tissue in the further progress of the disease.

Case report: This report is about a 45-year-old male who suffered a traumatic insult. X-rays showed an osteolytic lesion in the 4th lumbar vertebra with depression in the ventral roof plate. Further radiological investigation has ruled out a malignant lesion and suggested Gorham-Stout disease as a diagnosis. Therapy has been carried out using bisphosphonates as well as fractioned radiation of the vertebra with 30 Gy under which a remission was achieved.

Discussion: The progress of our case correlates with published data in the literature. Spinal lesions are rare, but possible neurological complications or a possible chylothorax reflect the high mortality rate. We found no complications in our case.

Conclusion: Despite massive osteolysis with resorption of up to a whole bone segment, radiation with a dose of 22.8 Gy and the use of antiresorptive substances, as reported in our case, have often been shown to limit the destructive progression of the disease. A causal therapy of the disease is not known yet.