Primary versus trauma-induced Gorham-Stout disease.

Abstract

Gorham-Stout disease - also known as "disappearing bone disease" is currently considered a single entity with varying clinical manifestations. We reviewed the existent literature from the earliest historic description(Jackson in 1838) and Gorham and Stout's original series of patients, multiple case reports and series since. After analyzing 212 reported cases, we identified 76 cases with details that recorded either a history of multifocal disease or an identifiable history of preceding trauma. From this review, we have defined two distinct Gorham-Stout entities - those characteristically associated with lymphangiomatosis [a form of GLA (generalized lymphangiomatosis) questionably distinguishable by bone biopsy and radiologic appearance] with multifocal distributed bone lesions, and those others, usually self-limited, first appearing after a traumatic event and always confined to a single bone or closely adjacent one. Multifocal disease is more likely to have chylothorax as a complication. These two Gorham-Stout entities differ in their demographic distribution, clinical history and manifestations, and they follow divergent clinical courses. The prognosis differs, and so should approaches to monitoring as well as acute and long-term treatment. Further research should seek to identify and define the differences in pathology and molecular mechanisms.