Skeletal cystic angiomatosis with severe hip joint deformation resembling massive osteolysis

Knoch, Fabian von; Grill, Franz; Herneth, Andreas M.; Kainberger, Franz; Lang, Susanna; Ploier, Robert; Knoch, Marius von

doi:10.1007/s004020100295

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Visceral involvement and macrocystic lymphatic malformations are observed more frequently in CA than in GSD, [ 4 8 42 ] although about 25% of GSD patients develop chylothorax [38] …”

Section: Discussion and Reviewmentioning

confidence: 99%

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Cystic angiomatosis, a heterogeneous condition

et al. 2016

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Background:Cystic angiomatosis (CA) is a rare disorder causing bony cysts. It displays some similarity to Gorham–Stout disease (GSD), but has a much better local prognosis, despite the larger number of cysts. These 2 conditions also differ in terms of their location, visceral involvement, and response to treatment.Methods:We report 4 cases of CA, including 1 sclerosing form, which we compare with cases from a literature review performed with PRISMA methodology.Results:We reviewed 38 articles describing 44 other patients. Mean age at diagnosis for the 48 patients (our 4 patients + the 44 from the review) was 22.5 years, and 28 of the patients were men. The femur was involved in 81% (n = 39), the pelvis in 73% (n = 35), the humerus in 52% (n = 25), the skull in 48% (n = 23), and the vertebrae in 44% (n = 21). Visceral lymphangiomatosis (either clinical, or detected on autopsy) was also reported in 35% (n = 18) of the patients. The spleen was the most frequently involved organ (n = 12), followed by the lungs and pleura (n = 8). Liver cysts and/or chylothorax were rarely reported (5 cases), but were invariably fatal. Radiation therapy on bone or soft tissue masses was ineffective, as was interferon alpha, in the 2 patients in which this drug was tested. The efficacy of bisphosphonate was at best equivocal.Conclusion:The progression of CA is unpredictable and treatments effective against GSD, such as bisphosphonates and radiotherapy, have proved ineffective for this condition. New treatments are thus urgently required.

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“…Visceral involvement and macrocystic lymphatic malformations are observed more frequently in CA than in GSD, [ 4 8 42 ] although about 25% of GSD patients develop chylothorax [38] …”

Section: Discussion and Reviewmentioning

confidence: 99%

“…The lesions are multifocal, well-defined, skeletal intramedullary cysts, with a relatively well-preserved bony cortex, [4] and no periosteal reaction, even if the cysts are located just beneath the cortex. The cysts are oriented over the long axis of the bone, and are classically surrounded by a sclerotic peripheral rim [5] .…”

Section: Introductionmentioning

confidence: 99%

Cystic angiomatosis, a heterogeneous condition

et al. 2016

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Síndrome de Gorham-Stout

Moreno-Regidor¹,

Borrego-Ratero²,

Cabo-Rodríguez³

2004

Revista Española de Cirugía Ortopédica y Traumatología

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Two cases of Gorham-Stout disease with good response to zoledronic acid treatment

Brance¹,

Castiglioni

Cóccaro

et al. 2017

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SummaryGorham-Stout disease -also called vanishing bone syndrome -is a rare bone disease characterized by a progressive intra-osseous proliferation of non-neoplastic vascular tissue resulting in massive osteolysis. Here, we report two clinical cases of Gorham-Stout disease. Case 1: a 56-year-old woman with 20 years of history of pain and swell in elbows, ankles and wrist. Then she was diagnosed as systemic lupus erythematosus (SLE) with glomerulonephritis type III. After other pathologies were ruled out Gorham-Stout disease was diagnosed. Intravenous zoledronic acid (5 mg) was indicated and after third infusion a progressive improvement of pain, mobility and daily activities were observed. Case 2: a 70-years-old man with a history of pain and limited motion in the left shoulder without X-ray abnormality. Six months later pathological fracture in the left humerus occurred and after ruled out other pathologies Gorham-Stout disease was diagnosed. Intravenous zoledronic acid (5 mg) was indicated and a good response was observed after the first infusion. Nowadays just over 200 cases were reported. Gorham-Stout disease was reported in different bones, at different age presentation and severe physical deformities, disabilities, and life-threatening complications can occur. Two cases of Gorham-Stout disease with good response to zoledronic acid was reported in this article.

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Skeletal cystic angiomatosis with severe hip joint deformation resembling massive osteolysis

Cited by 3 publications

References 7 publications

Cystic angiomatosis, a heterogeneous condition

Cystic angiomatosis, a heterogeneous condition

Síndrome de Gorham-Stout

Two cases of Gorham-Stout disease with good response to zoledronic acid treatment

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