Disappearing bones: A rare form of massive osteolysis

Gorham, L. W.; Wright, Alan; Shultz, H.H.; Maxon, F.C.

doi:10.1016/0002-9343(54)90027-3

Cited by 255 publications

(59 citation statements)

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“…Bone tropism characterizes GSS (3,4) and GCs adhere to OPN, which is strongly recognized by osteoclasts and involved in bone remodeling.…”

Section: Evidence That Gcs Have a Partial Features Of Immature Osteocmentioning

confidence: 99%

“…Because GSS is characterized by prominent vascularization, (3,4) we evaluated whether GCs were angiogenic. Table 2 shows that CM as well as GCs were highly angiogenic in the Matrigel model.…”

Section: Angiogenic Properties Of Gcsmentioning

confidence: 99%

“…GSS is nonhereditary and may occur at any age, although it is most commonly seen in young adults and rarely in the elderly. (1)(2)(3)(4)(5) The outcome is poor in patients with soft tissue involvement, with lesions of the axial skeleton and chylothorax. (5) By clinical, histopathological, and radiographic features, GSS is distinguishable from other bone vascular lesions with osteolysis, including skeletal angiomas, angiosarcoma, essential osteolysis, and hereditary osteolysis.…”

Section: Introductionmentioning

confidence: 99%

“…(2) The etiology of GSS is completely unknown, and the presence and significance of osteoclasts is controversial. (4,(6)(7)(8)(9)(10)(11) Circulating GSS osteoclast precursors show an increased sensitivity to osteoclastogenic factors, including IL-6, IL-1␤, and TNF␣, which results in an augmented ability to reabsorb bone in vitro. (12) More recently, elevated levels of IL-6 in serum of GSS patients have been reported.…”

Section: Introductionmentioning

confidence: 99%

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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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“…Bone tropism characterizes GSS (3,4) and GCs adhere to OPN, which is strongly recognized by osteoclasts and involved in bone remodeling.…”

Section: Evidence That Gcs Have a Partial Features Of Immature Osteocmentioning

confidence: 99%

Section: Angiogenic Properties Of Gcsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Colucci

Taraboletti

Primo

et al. 2006

Journal of Bone and Mineral Research

View full text Add to dashboard Cite

show abstract

“…It has occurred in association with the Ehlers-Danlos syndrome (Newton & Carpenter 1959) (Krikler 1955). He had made no pathological studies of the digits, but Gorham et al (1954) had indicated that in some cases the bone resorption might be due to the presence of thin-walled angiomatous tissue, and he wondered whether the present patient might have these lesions in both gut and fingers. Initially biopsies from several sites failed to show myeloma, but eventually positive histological evidence was obtained from the left scapula.…”

Section: Discussionmentioning

confidence: 89%