Gorham-Stout disease (also known as "disappearing bone disease") was first described by Jackson in 1838, but was properly defined by Gorham and Stout in a series of 24 patients in 1954-1955. It is a rare disease of unknown etiology (about 200 cases reported in the literature) characterized by spontaneous progressive resorption of bone without malignant proliferation of vascular structures. The diagnosis is one of exclusion and it is based on combined histological, radiological, and clinical features. Benign vascular proliferation with fatty bone marrow and thinning of bony trabeculae is a typical histological feature. Standard radiographs of disappearing bone disease show progressive bony resorption with adjacent soft tissue involvement. Most cases of Gorham-Stout disease resolve spontaneously, but prognosis remains unpredictable. This study reports 13 cases of Gorham-Stout disease treated in our institution from 1968 to 2008. The aim of the work was to review our series and the literature on this rare disease, as well as to evaluate whether or not an optimal treatment can be identified and recommended.
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http://dx.doi.org/10.1007/s00256-010-1051-9 | DOI Listing |
Pediatr Radiol
January 2025
Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Complex lymphatic anomalies are unique diseases marked by abnormal lymphatic vessel development and growth. Imaging is crucial in the evaluation and management of complex lymphatic anomalies, with dynamic contrast-enhanced MR lymphangiography emerging as a valuable modality for visualizing abnormal lymphatic structures and informing treatment decisions. This article gives an overview of complex lymphatic anomalies and their management strategies, focusing specifically on generalized lymphatic anomaly, Gorham-Stout disease, Kaposiform lymphangiomatosis, and central conducting lymphatic anomalies.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
December 2024
Thoracic Surgery Department, Nottingham University Hospitals NHS Trust, UK.
Gorham-Scout disease (GSD) is a rare skeletal disorder of unknown etiology characterized by progressive osteolysis and excessive lymphovascular proliferation. Chylothorax is a life-threatening complication. A teenager presented with a left pleural effusion on a background of chronic flank collection secondary to lymphovascular malformation.
View Article and Find Full Text PDFBMC Musculoskelet Disord
December 2024
Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, People's Republic of China.
Background: Gorham-Stout disease (GSD) is a rare disease characterized by osteolysis and lymphatic malformations. GSD involving the spine is exceptionally rare and lacks a standard cure. The aim of this article was to report a case of GSD with scoliosis treated via corrective surgery and medication.
View Article and Find Full Text PDFJBJS Case Connect
October 2024
Department of Orthopaedics, Texas Scottish Rite Hospital for Children, Dallas, Texas.
Case: This is a case of a 6-year-old patient diagnosed with Gorham-Stout disease (GSD), a rare lymphangiogenic skeletal disorder, localized to the left femur. Initial nonoperative treatment with pharmaceuticals and bracing was unsuccessful. We describe a definitive operative treatment with radical femoral resection and a modified rotationplasty technique through a tibiopelvic rotational hip arthroplasty.
View Article and Find Full Text PDFJ Inflamm Res
November 2024
Department of orthopedics, the Affiliated Changzhou Second People's Hospital of Nanjing Medical University, Changzhou, People's Republic of China.
Purpose: The polarization of macrophages towards the pro-inflammatory M1 phenotype and osteoclast overactivation play a significant role in the pathogenesis of aseptic loosening of orthopedic implants. This study sought to examine the expression and activation of macrophages and osteoclasts in implant biopsies with respect to epidermal growth factor receptor (EGFR) signaling and to assess the potential of EGFR inhibition in mitigating titanium particle-induced bone resorption in a cranial resorption murine model.
Methods: Bone marrow-derived macrophages (BMDMs) were stimulated with Tumor Necrosis Factor-alpha (TNF-α) and Interferon-gamma (IFN-γ) initially.
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