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Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases

Patients with Gorham–Stout disease (GSD) present progressive destruction and resorption of bone. Typical bone-related symptoms include swelling, pain and functional impairment in the region involved. The three aspects of GSD etiopathology are osteoclasts, angiogenesis/lymphangiogenesis and osteoblas...

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Detalles Bibliográficos
Autores principales: Angelini, Andrea, Mosele, Nicolò, Pagliarini, Elisa, Ruggieri, Pietro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bioscientifica Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8788153/
https://www.ncbi.nlm.nih.gov/pubmed/35076412
http://dx.doi.org/10.1530/EOR-21-0083
Descripción
Sumario:Patients with Gorham–Stout disease (GSD) present progressive destruction and resorption of bone. Typical bone-related symptoms include swelling, pain and functional impairment in the region involved. The three aspects of GSD etiopathology are osteoclasts, angiogenesis/lymphangiogenesis and osteoblast function. Multi-targeted pharmacological approach includes innovative options and represent milestones of treatment, sometimes associated with radiotherapy. Surgery is mainly used to treat complications: pathologic/impending fractures, spinal instability or deformities and chylothorax. In this narrative review, we highlight current standards in diagnosis, clinical management and therapeutic strategies.