Gorham-Stout Syndrome

Classification according to ICD-10
M89.5-	Osteolysis, any location
D18.1-	Lymphangioma, any location
ICD-10 online (WHO version 2019)

Rounded bone defect of the skullcap in Gorham-Stout syndrome. X-ray and computed tomography above, magnetic resonance imaging below.

The Gorham-Stout syndrome ( GSS ), also Gorham-Stout disease or English vanishing bone disease called, is an extremely rare disease in which one or many bones dissolved and replaced by lymphoid tissue to be replaced. Gorham-Stout syndrome was first described in 1955 by US pathologists L. Whittington Gorham (1885–1968) and Arthur Purdy Stout (1885–1967), after whom the disease is also named.

etiology

The cause of Gorham-Stout syndrome, in which idiopathic angiomatous proliferation in the bone leads to progressive bone resorption , is still largely unclear. Interleukin-6 obviously plays an essential role as a messenger substance in the disease. Increased osteoclast activity or angiomatosis is suspected. The disease is extremely rare. There are about 200 documented cases.

Complications

Chylothorax
Involvement of the spine

therapy

There is currently no widely accepted treatment regimen for Gorham-Stout syndrome. The administration of bisphosphonates , interferon- α2b, radiation and chemotherapy as well as surgical interventions ( pleurodesis in the case of a chylothorax ) are described as interventions.

Treatment (as off-label use ) with bisphosphonates (such as zoledronic acid ) showed improvements in symptoms after several years of use.

Web links

Gorham-Stout Syndrome. In: Orphanet (Rare Disease Database).
Gorham-Stout Syndrome. In: Online Mendelian Inheritance in Man . (English)
Lymphangiomatosis & Gorham's Disease Alliance - Europe (English / multilingual)
Gorham's disease database (English)

Individual evidence

^ LW Gorham and AP Stout: Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone); its relation to hemangiomatosis. ( Memento of the original from October 13, 2008 in the Internet Archive ) Info: The archive link was automatically inserted and not yet checked. Please check the original and archive link according to the instructions and then remove this notice. In: Journal of Bone and Joint Surgery (American Volume) 37, 1955, pp. 985-1004. PMID 13263344 @1@ 2
↑ Kristian Nikolaus Schneider, Georg Gosheger, Dimosthenis Andreou: Clinical snapshot: Gorham-Stout-Syndrome. In: Deutsches Ärzteblatt. Volume 116, Issue 29 f., (July 22) 2019, p. 507.
↑ RD Devlin et al .: Interleukin-6: a potential mediator of the massive osteolysis in patients with Gorham-Stout disease. In: J Clin Endocr 81, 1996, pp. 1893-1897. PMID 8626854
↑ Kristian Nikolaus Schneider, Georg Gosheger, Dimosthenis Andreou: Clinical snapshot: Gorham-Stout-Syndrome. 2019.

[1] LW Gorham and AP Stout: Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone); its relation to hemangiomatosis. ( Memento of the original from October 13, 2008 in the Internet Archive ) Info: The archive link was automatically inserted and not yet checked. Please check the original and archive link according to the instructions and then remove this notice. In: Journal of Bone and Joint Surgery (American Volume) 37, 1955, pp. 985-1004. PMID 13263344 @1@ 2

[2] Kristian Nikolaus Schneider, Georg Gosheger, Dimosthenis Andreou: Clinical snapshot: Gorham-Stout-Syndrome. In: Deutsches Ärzteblatt. Volume 116, Issue 29 f., (July 22) 2019, p. 507.

[3] RD Devlin et al .: Interleukin-6: a potential mediator of the massive osteolysis in patients with Gorham-Stout disease. In: J Clin Endocr 81, 1996, pp. 1893-1897. PMID 8626854

[4] Kristian Nikolaus Schneider, Georg Gosheger, Dimosthenis Andreou: Clinical snapshot: Gorham-Stout-Syndrome. 2019.