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Journal of Zhejiang University SCIENCE B 2013 Vol.14 No.8 P.729-735

http://doi.org/10.1631/jzus.B1200308


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


Author(s):  Po Hu, Xiang-gui Yuan, Xin-yang Hu, Fa-rong Shen, Jian-an Wang

Affiliation(s):  Cardiovascular Key Lab of Zhejiang Province, Department of Cardiology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China; more

Corresponding email(s):   hu999po@126.com, wang_jian_an@tom.com

Key Words:  Gorham-Stout syndrome (GSS), Massive osteolysis, ChineseThe online version of this article contains supplementary materials


Po Hu, Xiang-gui Yuan, Xin-yang Hu, Fa-rong Shen, Jian-an Wang. Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature[J]. Journal of Zhejiang University Science B, 2013, 14(8): 729-735.

@article{title="Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature",
author="Po Hu, Xiang-gui Yuan, Xin-yang Hu, Fa-rong Shen, Jian-an Wang",
journal="Journal of Zhejiang University Science B",
volume="14",
number="8",
pages="729-735",
year="2013",
publisher="Zhejiang University Press & Springer",
doi="10.1631/jzus.B1200308"
}

%0 Journal Article
%T Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature
%A Po Hu
%A Xiang-gui Yuan
%A Xin-yang Hu
%A Fa-rong Shen
%A Jian-an Wang
%J Journal of Zhejiang University SCIENCE B
%V 14
%N 8
%P 729-735
%@ 1673-1581
%D 2013
%I Zhejiang University Press & Springer
%DOI 10.1631/jzus.B1200308

TY - JOUR
T1 - Gorham-Stout syndrome in mainland China: a case series of 67 patients and review of the literature
A1 - Po Hu
A1 - Xiang-gui Yuan
A1 - Xin-yang Hu
A1 - Fa-rong Shen
A1 - Jian-an Wang
J0 - Journal of Zhejiang University Science B
VL - 14
IS - 8
SP - 729
EP - 735
%@ 1673-1581
Y1 - 2013
PB - Zhejiang University Press & Springer
ER -
DOI - 10.1631/jzus.B1200308


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.

Darkslateblue:Affiliate; Royal Blue:Author; Turquoise:Article

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