Gorham's Disease or Massive Osteolysis

Patel, Dipak V.

doi:10.3121/cmr.3.2.65

Cited by 214 publications

(303 citation statements)

References 115 publications

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“…We found that there was a male preference (43 males vs. 24 females, the gender ratio was about 1.8). However, some authors believe that there is no gender preference (Patel, 2005;Collins, 2006;Tong et al, 2010). The clinical complaint of GSS varies depending on the affected site, making the diagnosis of this uncommon entity difficult.…”

Section: Discussionmentioning

confidence: 98%

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Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature

Yuan

et al. 2013

J. Zhejiang Univ. Sci. B

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Abstract:Objective: Gorham-Stout syndrome (GSS) is a rare disorder of uncertain etiology and unpredictable prognosis. This study aims to present a comprehensive understanding of this rare entity. Methods: A literature search in PubMed and three Chinese databases was performed to screen histologically proven GSS cases among Chinese residents in the mainland. We analyzed the patients' clinical characteristics, the value of different treatment modalities and their influence on the clinical outcome. Results: Sixty-seven cases were finally enrolled. There were 43 men (64.2%) and 24 women (35.8%). The mean age at diagnosis was 28 years (1.5-71 years). The most common clinical symptoms included pain (n=40, 59.7%), functional impairment (n=13, 19.4%), and swelling (n=12, 17.9%). The radiographic presentation of 37 cases (55.2%) was disappearance of a portion of the bone. The others presented as radiolucent foci in the intramedullary or subcortical regions. A total of 42 cases provided data on therapy, these included surgery (n=27, 40.3%), radiation therapy (n=6, 9.0%), surgery combined with radiation therapy (n=2, 3.0%), and medicine therapy (n=7, 10.4%). For 30 of these 42 cases, follow-up data were available: 21 cases had the disorder locally controlled and 9 had a symptom progression. Fortunately, the disease is not fatal in the majority of cases. Conclusions: GSS has no specific symptoms and it should be taken into consideration when an unclear massive osteolysis occurs. The efficacies of different treatment modalities are still unpredictable and further research is required to assess the values of different treatments.

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Section: Discussionmentioning

confidence: 98%

“…Generally, the prognosis is considered to be good when only the limbs or the pelvic bones are involved (Kulenkampff et al, 1990;Stove and Reichelt, 1995;Rauh and Gross, 1997;Boyer et al, 2005). However, involvement of vital structures may be fatal (Hagberg et al, 1997;Ng and Sell, 2003;Patel, 2005).…”

Section: Introductionmentioning

confidence: 99%

Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature

Yuan

et al. 2013

J. Zhejiang Univ. Sci. B

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“…Radiographic appearance is the most dramatic aspect of the disease. [4] In the initial stage, radiolucent foci appear in the intramedullary or subcortical regions. After that, slowly progressive atrophy, dissolution, fracture, fragmentation and disappearance of a portion of a bone occur, and in the same time, soft tissue atrophy appears.…”

Section: Discussionmentioning

confidence: 99%

“…The treatment of this disease includes three major categories: medicine therapy, radiation and surgery, but there is currently no single recommendation in the contemporary English literature. [2][3][4][5] …”

Section: Introductionmentioning

confidence: 99%

“…This entity is non-hereditary, has no gender or ethnic predilection, and can occur at any age from 1 month to 75 years, with a peak incidence before 40 years of age. [2][3][4] Any part of the skeletal system may be affected, involving both axial and appendicular skeleton, but the shoulder, pelvis and mandible are the most common sites of involvement. [4,5] Imaging features are non-specific and osteolytic feature is the common pattern in conventional and CT imaging.…”

Section: Introductionmentioning

confidence: 99%

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Imaging pattern and histological features of Gorham-Stout Disease of the radius

Bruyere¹,

Garibotto²,

Rougemont³

et al. 2018

JST

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Gorham-Stout Disease (GD) is a very rare disease of unknown etiology characterized by progressive osteolysis and soft tissue involvement. Imaging is non-specific, and diagnosis may be delayed. The evolution of the disease is unpredictable, with progression of the osteolysis, spontaneous regression, or in a few cases re-ossification. We report a case of a 54-year-old woman with GD of the radius. In this rare location, only few cases have been reported with all imaging modalities: conventional radiography, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET-CT). We describe the characteristics of GD in different imaging modalities, as well as the histological features. To the best of our knowledge, we report the first metabolically active lesion in GD, with relevant implications for the differential diagnosis.

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Naevi and other Developmental Defects

Moss

Shahidullah

2010

Rook's Textbook of Dermatology

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Gorham's Disease or Massive Osteolysis

Cited by 214 publications

References 115 publications

Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature

Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature

Imaging pattern and histological features of Gorham-Stout Disease of the radius

Naevi and other Developmental Defects

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