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 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.