Gorham's syndrome (massive osteolysis): A case report

Fisher, Keith; Pogrel, M. Anthony

doi:10.1016/0278-2391(90)90543-b

Cited by 40 publications

(19 citation statements)

References 12 publications

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“…Death is generally the result of the involvement of the thorax or spinal column, with consequent hemorrhaging in the pleural cavity [5]. Long-term survival with different degrees of physical debilitation is possible for those with peripheral bone disease, such as the case reported here, in which only the mandible was affected [14].…”

Section: Discussionmentioning

confidence: 89%

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Use of zoledronic acid in the treatment of Gorham's disease

Avelar

Martins

Antunes

et al. 2010

International Journal of Pediatric Otorhinolaryngology

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Section: Discussionmentioning

confidence: 89%

“…A large number of treatments have been proposed, including sodium fluoride, vitamin D, calcium, alpha interferon [12], calcitonin and reconstructive surgery with a micro-vascularized graft [10], but the results have been variable [14,25]. Radiotherapy has achieved positive results due to the radio-sensitive nature of endothelial cells.…”

Section: Discussionmentioning

confidence: 98%

Use of zoledronic acid in the treatment of Gorham's disease

Avelar

Martins

Antunes

et al. 2010

International Journal of Pediatric Otorhinolaryngology

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“…The first case has already been published. (23) The other one was a 7-year-old girl who first presented with left maxillary and mandibular hypoplasia and loose teeth. After extensive radiological study and bone biopsy, she was diagnosed as GSS and started on calcitonin injections.…”

Section: Histological Analysismentioning

confidence: 99%

Gorham-Stout Syndrome: A Monocyte-Mediated Cytokine Propelled Disease

Colucci

Taraboletti

Primo

et al. 2006

Journal of Bone and Mineral Research

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We studied the biological features and the immunophenotype of a cell culture established from the lesion of soft tissues of a woman affected by Gorham-Stout syndrome. We found that these cells belonged to a monocytic lineage with some characteristics of immature osteoclasts and were able to release large amounts of osteoclastogenic and angiogenic molecules that may contribute to disease progression.Introduction: Gorham-Stout syndrome is a rare disease characterized by osteolysis and proliferation of vascular or lymphatic vessels, with a severe outcome. Its etiology and the identification of the cell types involved are completely unknown. Materials and Methods: A cell culture from a lesion of soft tissues was established, and its behavior in vitro and in immunodeficient mice was studied. We analyzed (1) the cell phenotype by flow cytometry; (2) the adhesive and migratory properties on different substrates; (3) the ability to differentiate into mature osteoclasts; (4) the production of osteclastogenic and angiogenic molecules; (5) the in vivo angiogenic activity of the cells subcutaneously implanted in mouse in a Matrigel plug; and (6) the ability to recapitulate the disease when transplanted in nude mice. Results and Conclusions:The established culture consisted of a morphologically homogeneous cell population belonging to a monocytic lineage having some features of an osteoclast-like cell type. Cells had an invasive phenotype, were angiogenic, and produced osteoclastogenic (IL-6, TGF-␤1, IL-1␤) and angiogenic (vascular endothelial growth factor-A [VEGF-A], CXCL-8) molecules when challenged with inflammatory cytokines. Immunodeficient mice injected with these cells did not show any bone lesions or vascular alteration, but had high amounts of circulating human IL-6 and VEGF-A. Cells isolated from a cutaneous lymphangiomatosis did not show any of these findings. These data suggest that cells of monocyte-macrophage lineage play an essential role in the pathogenesis of Gorham-Stout disease, whose progression is propelled by cytokine circuits that accelerate angiogenesis and osteoclastogenesis.

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“…3 Children and young adults are most commonly affected, although patients ranging in age from 18 months to 72 years have been reported. 4,5 There is a slight male predilection. 6 The most common sites of involvement are the shoulder and pelvis, 7 although any bone may be affected.…”

Section: Discussionmentioning

confidence: 99%