The Gorham-Stout syndrome (Gorham’s massive osteolysis)

Abstract

T he Gorham-Stout Syndrome (Gorham's massive osteolysis) is a rare condition in which spontaneous, progressive resorption of bone occurs. The aetiology is poorly understood. We report six cases of the condition and present evidence that osteolysis is due to an increased number of stimulated osteoclasts. This suggests that early potent antiresorptive therapy such as with calcitonin or bisphosphonates may prevent local progressive osteolysis.

Painless Destruction of the Shoulder Joint: A Case Report

“…No generally effective therapy is known [24]. In the early stages radiation therapy, biphosphonates or calcitonin can arrest the disease [16]. We could not intervene in this patient as the destruction process had already stopped when the patient presented to us and there was little functional disability.…”

“…The proposed mechanisms include hyperaemic granulation tissue in the bone [12]; underlying endothelial dysplasia of lymphatics, blood vessels or both [13]; local hypoxia and acidosis [14]; and activation of silent hamartoma [15]. Another theory proposes that the disease is the result of deranged osteoclastic activity [16].…”

Gorham-Stout disease of the shoulder girdle and cervico-thoracic spine: fatal course in a 65-year-old woman

“…It is defined by spontaneous, idiopathic, progressive osteolysis not accompanied by new bone production. The first case of this entity was probably reported in 1838 as a "boneless arm" of a male patient with a history of a "vanishing" humerus over 11 years [2,11]. The specific disease entity was defined by Gorham and Stout in 1955 based on a series of 24 patients [3].…”

“…There is a considerable amount of controversy concerning the role of osteoclasts in the pathogenesis of Gorham-Stout disease. Because osteoclasts are the only cells capable of bone resorption and because in some reports a significant number of highly stimulated osteoclasts have been found [5,11,20], a pivotal role of this cell has been assumed by many authors. However, in other reported cases, including our own, osteoclasts were rare.…”

A Rare Variant of Angiogenic Vertebral Body Destruction in a Child: Gorham’s Disease?

“…Мнения о роли остеокластов в деструкции костной ткани при GSD противоречивы: ряд исследователей подтверждает их высокую активность [1,5,6,18,22,26,32,36,42,44,48,50], другие отмечают их отсутствие в оча-гах остеолиза [12,13,21,45,51]. При-чина такого противоречия, возмож-но, обусловлена проведением гисто-логического исследования на разных стадиях процесса [4].…”