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.

The Orthopedic-Vascular Multidisciplinary Approach Improves Patient Safety in Surgery for Musculoskeletal Tumors: A Large-Volume Center Experience.

Objective: Wide-margin resection is mandatory for malignant bone and soft tissue tumors. However, this increases the complexity of resections, especially when vessels are involved. Patients in this high-risk clinical setting could be surgically treated using the multidisciplinary orthopedic-vascular approach. This study was carried out in this healthcare organization to evaluate patient safety in term of oncologic outcomes and reduction of the complication rate. Materials and Methods: We retrospectively reviewed 74 patients (37 males, 37 females; mean age 46 years, range 9-88) who underwent surgical excision for bone/soft tissue malignant tumors closely attached to vascular structures from October 2015 to February 2019. Vascular surgery consisted of isolation of at least one vessel (64 patients), bypass reconstruction (9 patients), and end-to-end anastomosis (1 patient). Mean follow-up was 27 months. Patients' demographics, tumor characteristics, adjuvant treatments, type of orthopedic and vascular procedures, and oncologic and functional outcomes and complications were recorded. Results: Overall survival was 85% at 3 years follow-up. In total, 22 patients experienced at least one major complication requiring further surgery and 13 patients experienced at least one minor complication, whereas 17 reported deviations from the normal postoperative course without the need for pharmacological or interventional treatment. Major complications were higher in pelvic resections compared to limb-salvage procedures (p = 0.0564) and when surgical time was more than 4 h (p = 0.0364) at univariate analysis, whereas the most important multivariate independent predictors for major complications were pelvic resection (p = 0.0196) and preoperative radiotherapy (p = 0.0426). Conclusions: A multidisciplinary ortho-vascular approach for resection of malignant bone and soft tissue tumors tightly attached to important vascular structures should be considered a good clinical practice for patient safety.

Rare aneurysmal bone cysts: multifocal, extraosseous, and surface variants.

Multifocal, extraosseous, and surface aneurysmal bone cysts are rare variants of the primary lesions. The clinicopathological features are similar, and the optimal treatment is surgical. Although local recurrences may occur, the prognosis is excellent. This review article introduces the readers to a rare diagnosis which they may have been previously unfamiliar with, presents the clinicopathological and imaging features of these rare aneurysmal bone cyst variants, and discusses their diagnosis and treatment. The clinicians who treat patients with aneurysmal bone cysts should be familiar with these uncommon entities and their differential diagnosis.

Calcific Myonecrosis of the Leg: A Rare Entity.

Calcific myonecrosis is a rare disease that has been shown to be a late sequela of trauma. This article presents a 68-year-old man with calcific myonecrosis of the leg 40 years after a tibial fracture complicated with peroneal nerve palsy. The soft tissue mass increased in size after another injury to the leg that occurred two years before his presentation. Physical examination at presentation showed a palpable extra-osseous mass at the anterior aspect of the left leg; the mass was not adherent to adjacent soft-tissues and bone, and it was painless but tender to palpation. Radiographs of the left leg showed extensive calcification at the soft-tissue of the anterior and posterior leg. An ultrasonography-guided trocar biopsy was done; histological findings were indicative of calcific myonecrosis. Given the benign entity of the lesion and known high rate of complications, he was recommended for no further treatment except for clinical and imaging observation. Located at the site of the biopsy, he experienced infection with drainage that eventually healed after six months with antibiotics and wound dressing changes. During the last follow-up examination, two years after diagnosis, the patient was asymptomatic without progression of the mass.

Giant cell reparative granuloma of the scapula: report of a case and literature review.

Giant cell reparative granulomas (GCRGs) are non-neoplastic inflammatory lesions, usually observed in the maxilla, mandible or small bones of the hands and feet. These lesions present a wide range of morphology and the misinterpretation with other giant cell lesions can often occur. We report the case of a 47-year-old woman with GCRG in the left scapula, presenting some uncommon features: the location (scapula) and age at presentation, the lack of underlying bone disease such as Paget's disease or fibrous dysplasia, the large aggressive expansile aspect of the lesion. This was a therapeutic study, level IV (case series with no or a historical control group).