{"title":"以难治性胸腔积液表现的Gorham-Stout病的处理:一例报告和文献回顾","authors":"T. Jayakumar, D. Khera, V. Manchanda, K. Rathod","doi":"10.4103/oji.oji_20_19","DOIUrl":null,"url":null,"abstract":"Gorham–Stout disease (GSD) is a very rare disorder of unknown etiology, characterized by the abnormal proliferation of lymphatic endothelial cells, and also called “vanishing bone disease,” “phantom bone disease,” and “massive osteolysis,” as osteolysis is a characteristic feature. Patients present with a variety of symptoms such as swelling, pain, physical disability and deformity, shortness of breath, and neurological symptoms. It is a progressive disease, and owing to its rarity, the research and treatment options are limited. Our patient is a 15-year-old male, who presented with large mass over abdomen and chest, chylothorax, and osteolysis. This patient was initially treated with chest drains, subcutaneous drains, and sclerotherapy. Eventually, symptoms were worsened. After literature search, the patient was diagnosed with GSD, and sirolimus therapy was started. After 2 months of sirolimus therapy, the lesions subsided and well controlled.","PeriodicalId":431823,"journal":{"name":"Oncology Journal of India","volume":"1 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2019-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Management of Gorham–Stout disease presenting as intractable pleural effusion in an adolescent: A case report and review of literature\",\"authors\":\"T. Jayakumar, D. Khera, V. Manchanda, K. Rathod\",\"doi\":\"10.4103/oji.oji_20_19\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Gorham–Stout disease (GSD) is a very rare disorder of unknown etiology, characterized by the abnormal proliferation of lymphatic endothelial cells, and also called “vanishing bone disease,” “phantom bone disease,” and “massive osteolysis,” as osteolysis is a characteristic feature. Patients present with a variety of symptoms such as swelling, pain, physical disability and deformity, shortness of breath, and neurological symptoms. It is a progressive disease, and owing to its rarity, the research and treatment options are limited. Our patient is a 15-year-old male, who presented with large mass over abdomen and chest, chylothorax, and osteolysis. This patient was initially treated with chest drains, subcutaneous drains, and sclerotherapy. Eventually, symptoms were worsened. After literature search, the patient was diagnosed with GSD, and sirolimus therapy was started. After 2 months of sirolimus therapy, the lesions subsided and well controlled.\",\"PeriodicalId\":431823,\"journal\":{\"name\":\"Oncology Journal of India\",\"volume\":\"1 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Oncology Journal of India\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/oji.oji_20_19\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Oncology Journal of India","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/oji.oji_20_19","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Management of Gorham–Stout disease presenting as intractable pleural effusion in an adolescent: A case report and review of literature
Gorham–Stout disease (GSD) is a very rare disorder of unknown etiology, characterized by the abnormal proliferation of lymphatic endothelial cells, and also called “vanishing bone disease,” “phantom bone disease,” and “massive osteolysis,” as osteolysis is a characteristic feature. Patients present with a variety of symptoms such as swelling, pain, physical disability and deformity, shortness of breath, and neurological symptoms. It is a progressive disease, and owing to its rarity, the research and treatment options are limited. Our patient is a 15-year-old male, who presented with large mass over abdomen and chest, chylothorax, and osteolysis. This patient was initially treated with chest drains, subcutaneous drains, and sclerotherapy. Eventually, symptoms were worsened. After literature search, the patient was diagnosed with GSD, and sirolimus therapy was started. After 2 months of sirolimus therapy, the lesions subsided and well controlled.