FD appears radiographically as radiolucent areas which later

FD appears radiographically as radiolucent areas which later develop into a partially opaque ground glass appearance [51]. Other features that may be present are endosteal scalloping, bony expansion, and a thick reactive bone ‘rind’ [54]. MR imaging may be a more effective tool at identifying the size of the area affected by FD, and could be useful when the radiographic appearance of suspected FD is ambiguous [54]. FD is usually treated conservatively by maintenance bone density with regular exercise and diet [50]. Medical therapy such as the use of bisphosphonates, particularly pamidronate, may be effective at reducing pain in FD, however there is a need for trials to provide further evidence of this effect [55]. It has been suggested that other medical therapies such as denosumab and pregabalin may be potentially useful treatments at reducing pain from FD [55]. Currently little evidence exists to support these hypotheses. Surgery is considered in patients with progressive symptoms or when the disease threatens important anatomical structures [51]. Treatment is often curettage followed by autologous bone graft. Cortical bone grafts are deemed superior to cancellous bone grafts, by virtue of their ability to resist replacement by the FD lesion [49]. Where FD has resulted in deformity, corrective surgery may be required, for example osteotomy and intramedullary fixation [49,53]. Recurrence occurs in around 18% of cases [56].
Enchondroma Only 2.6% of all benign bone tumours can be considered an enchondroma [52]. These asymptomatic tumours may present at any age, however 59% occur between the ages of 10 and 39 [52]. These tumours consist of masses of hyaline cartilage in a lobular formation, typically presenting in long tubular bones, most commonly the hands and feet [52,57]. These are usually solitary lesions, but it is possible for multiple enchondromas to form, a condition that is described as enchondromatosis or Ollier׳s disease [58]. During diagnosis it is essential to distinguish between a benign enchondroma and low grade chondrosarcoma. Enchondroma occurs more frequently in the hands and feet and chondrosarcoma in the LY 2389575 hydrochloride – however accurate diagnosis is necessary given the different treatment routes required for both lesions [59]. Radiographic features of enchondromas include stippled calcification, endosteal scalloping, with areas of ossification or expanded cortex [52,57]. MRI allows identification of classic features of malignancy such as cortical destruction, soft tissue masses, multilocular appearance, and involvement of flat bone [60]. Cartilaginous islands surrounded by fat may also be a potentially useful diagnostic sign detected on MRI scans [61]. Enchondromas do not routinely require surgical treatment, unless they are symptomatic, increasing in size, or there is a risk of pathological fracture. Typically treatment of enchondromas has involved intralesional excision, followed by filling with an autologous bone graft or synthetic filling [57]. Adjuvant treatments have been used to reduce recurrence rates, however this is not normally necessary as 10 year recurrence is around 0.04% [57,62]. In recent years trials have suggested that curettage without augmentation or reconstruction is a potential new treatment [63]. This evidence suggests that the time for the formation of new bone is similar in groups of patients whether they receive grafts or undergo simple curettage [64]. A recent case series has suggested that a lateral approach during tumour excision, as opposed to the traditional dorsal approach may help to reduce postoperative stiffness [65]. Summary table
Conclusion
Conflicts of interest statement
Introduction Gorham–Stout Disease (GSD), also known as ‘massive osteolysis’ and ‘vanishing bone disease’, is a rare bone condition characterized by spontaneous, idiopathic, and progressive proliferation of thin-walled vascular and lymphatic vessels replacing bone and marrow space by fibrous connective tissue. It leads to bone destruction which may sometimes be followed by new bone production [1]. In 1838 Jackson described the first case of a patient with a “boneless arm” [2]. Clinical and anatomopathological features were described by Gorham and Stout in 1955. They showed that this disease leads to progressive massive osteolysis by invasion of bone by lymphangiomatic tissue [3]. GSD mostly occurs in young adults; mean age is 25 years. Male and female are equally affected, without inheritance pattern or race predilection [4]. Clinical symptoms vary based on the location of bone involvement. This disease is most often regional and may involve several bones around a joint. Evolution may differ from one patient to another, GSD can be benign with a tendency to self-limitation or even spontaneous regression but it can also be very disabling [3]. Known to be ubiquitous, it frequently affects the skull and maxillofacial bones, ribs, cervical vertebrae, shoulder and pelvic girdle bones [5,6]. The affected bone(s) weaken(s) progressively, and pain and spontaneous fractures are the most common clinical features besides swelling and progressive deformity of the affected extremity. When localized at a lower extremity, limb length discrepancy and axial deformation may lead to gait abnormalities and major limping can occur [7]. Depending on location, it may also lead to neurological complications and paralysis (in case of vertebrae involvement), respiratory insufficiency and sometimes to death [8].