Aims. The aim of this study was to report the long-term prognosis of patients with multiple Langerhans cell histiocytosis (LCH) involving the spine, and to analyze the risk factors for progression-free survival (PFS). Methods. We included 28 patients with multiple LCH involving the spine treated between January 2009 and August 2021. Kaplan-Meier methods were applied to estimate overall survival (OS) and PFS. Univariate Cox regression analysis was used to identify variables associated with PFS. Results. Patients with multiple LCH involving the spine accounted for 15.4% (28/182 cases) of all cases of spinal LCH: their lesions primarily involved the thoracic and lumbar spines. The most common symptom was pain, followed by neurological dysfunction. All patients presented with osteolytic bone destruction, and 23 cases were accompanied by a paravertebral soft-tissue mass. The incidence of vertebra plana was low, whereas the oversleeve-like sign was a more common finding. The alkaline phosphatase was significantly higher in patients with single-system multifocal bone LCH than in patients with multisystem LCH. At final follow-up, one patient had been lost to follow-up, two patients had died, three patients had local recurrence, six patients had distant involvement, and 17 patients were alive with disease. The median PFS and OS were 50.5 months (interquartile range (IQR) 23.5 to 63.1) and 60.5 months (IQR 38.0 to 73.3), respectively. Stage (hazard ratio (HR) 4.324; p < 0.001) and chemotherapy (HR 0.203; p < 0.001) were prognostic factors for PFS. Conclusion. Pain is primarily due to segmental instability of the spine from its destruction by LCH. Chemotherapy can significantly improve PFS, and radiotherapy has achieved good results in local control. The LCH lesions in some patients will continue to progress. It may initially appear as an isolated or single-system LCH, but will gradually involve multiple sites or systems. Therefore, long-term follow-up and timely intervention are important for patients with spinal LCH. Cite this article: Bone Joint J 2023;105-B(6):679–687

Little is known about the relationship between head-neck corrosion and its effect on the periprosthetic tissues and distant organs of patients hosting well-functioning devices. The purpose of this study was to investigate in postmortem retrieved specimens the degree and type of taper damage, and the corresponding histologic responses in periprosthetic tissues and distant organs. Fifty postmortem THRs (34 primaries, 16 revisions) retrieved after 0.5 to 26 years were analyzed. Forty-three implants had a CoCrMo stem and seven had a Ti6Al4V stem. All heads were CoCrMo and articulated against polyethylene cups (19 XLPE, 31 UHMWPE). H&E sections of joint pseudocapsules, liver, spleen, kidneys and lymph nodes were graded 1–4 for the intensity of various inflammatory cell infiltrates and tissue characteristics. Corrosion damage of the taper surfaces was assessed using visual scoring and quantitated with an optical coordinate measuring machine. SEM analysis was used to determine the acting corrosion mode. Polyethylene wear was assessed optically. The majority of tapers had minimal to mild damage characterized by local plastic deformation of machining line peaks. Imprinting of the stem topography onto the head taper surface was observed in 18 cases. Column damage on the head taper surface occurred in three cases. All taper surfaces scored moderate or severe exhibited local damage features of fretting and/or pitting corrosion. Moderate or severe corrosion of the head and/or trunnion was present in nine hips. In one asymptomatic patient with bilateral hips, lymphocyte-dominated tissue reactions involving perivascular infiltrates of lymphocytes and plasmacytes were observed. In this patient, mild, focal lymphocytic infiltrates were also present in the liver and kidneys, and there was focal histiocytosis and necrosis of the para-aortic lymph nodes. These two implants, which had been in place for 58.6 and 60.1 months, had severe intergranular corrosion of the CoCrMo trunnion, and column damage and imprinting on the head taper. In the other 41 hips, macrophage responses in the joint pseudocapsule to metallic and/or polyethylene wear particles ranged widely from minimal to marked. Focal necrosis in the pseudocapsules of 12 arthroplasties was related to high concentrations of CoCrMo, TiAl4V, TiO, BaSO4 and polyethylene wear particles. High concentrations of these particles were also detected in para-aortic lymph nodes. Rare to mild macrophages were observed in liver and spleen. This is a comprehensive study of wear and corrosion within well-functioning postmortem retrieved THRs, and the resulting local and distant tissue reactions. One of eight patients with moderate or severe corrosion did have a subclinical inflammatory response dominated by lymphocytes after five years. To what extent such an inflammatory process might progress to become symptomatic is not known. Ionic and particulate products generated by corrosion disseminated systemically. The minor lymphocytic infiltrate in the liver and kidneys of one subject with bilateral severely corroded head-neck junctions might suggest possible metal toxicity. The diagnosis of adverse tissue reactions to corrosion of modular junctions can be challenging. Postmortem retrieval studies add to our understanding of the nature and progression of lymphocyte-dominated adverse local and potentially systemic tissue reactions to corrosion of modular junctions

Introduction:. Sinus histiocytosis with massive lymphadenopathy (SHML) also known as Rosai – Dorfman disease is a disease of bone marrow stem cell origin. It affects lymph nodes primarily. Solitary bone lesions are very rare and can cause diagnostic difficulty. Aim:. To increase the awareness of SHML as a cause of cystic bone lesions. Materials and methods:. A 2 year old presented with 4 months history of pain and swelling of the distal forearm. There was no history of tuberculosis or HIV disease. The swelling was 4 × 3 cm firm, non-fluctuant and slightly tender. There were no lymph nodes. Radiographs showed an oval cystic lesion expanding with a well-defined margin. The ulnar cortex was deficient. CT scan confirmed a cystic lesion with contents of granulation tissue. The Hb and WCC were normal, ESR 20 was, CRP<5 and mantoux was negative. At surgery the lesion was curretted. The contents resembled tuberculous granulation but there was no caseation. The borders were well formed, the ulnar cortex was deficient. Results:. The histology revealed granulation tissue with numerous large histiocytes and immuno chemistry confirmed Rosai Dorfman disease. Healing with sclerosis was seen at 6 months. Discussion:. Rosai Dorfman disease is a systemic disease of bone marrow stem cells and lymphadenopathy is the prominent manifestation. Only ±8% of cases have been reported with bone involvement and 4% of these had no lymphadenopathy. The lesions are cystic and medullary but cortical involvement can occur. Solitary ossseous lesions characterized by a background of histocytes without eosinophils can mimic Langerhans histocytosis, localized osteomyelitis, fibrous dysplasia, tuberculosis, simple or aneurysmal bone cysts and metastatic deposits. Conclusion:. Lesions of haematopoetic origin should be considered in the diagnosis of lucent bone lesions in children

1. The clinical, histological and radiological findings in 129 cases of histiocytosis X have been reviewed and the natural history of the disease is discussed. 2. Certain clinical and histological factors emerge as having prognostic significance. 3. The treatment in thirty-four personally reviewed cases is discussed and some suggestions are made as to the treatment of particular types of case

The clinical and radiological features in three cases of cystic angiomatosis of bone are reported. Although these features are generally diagnostic except from histiocytosis X, the definitive diagnosis must be established by a pathological study, preferably of a segment of an involved rib or fibula. The prognosis varies according to the type of clinical presentation-in particular upon whether the lesions are solely skeletal or whether there is extraskeletal visceral involvement. Whereas these last cases may often prove fatal, those with only skeletal involvement have a favourable prognosis: indeed, the cystic bone lesions may regress without any treatment, as occurred in some cases reported in the literature and in two of our three cases

Introduction Localised Langerhans-cell histiocytosis of bone (eosinophilic granuloma) is a benign tumour-like condition with a variable clinical course. Different forms of treatment have been reported to give satisfactory results. However, previous series all contain patients with a wide age range. Our aim was to investigate the effect of skeletal maturity on the rate of recurrence of isolated eosinophilic granuloma of bone excluding those arising in the spine. Methods We followed-up 32 patients with an isolated eosinophilic granuloma for a mean of five years; 17 were skeletally immature. Results No recurrences were noted in the skeletally immature group even after biopsy alone. By contrast, four of 13 skeletally mature patients had a recurrence and required further surgery. Conclusions This suggests that eosinophilic granuloma has a low rate of recurrence in skeletally immature patients

Localised Langerhans-cell histiocytosis of bone (eosinophilic granuloma) is a benign tumour-like condition with a variable clinical course. Different forms of treatment have been reported to give satisfactory results. However, previous series all contain patients with a wide age range. Our aim was to investigate the effect of skeletal maturity on the rate of recurrence of isolated eosinophilic granuloma of bone excluding those arising in the spine. We followed up 32 patients with an isolated eosinophilic granuloma for a mean of five years; 17 were skeletally immature. No recurrences were noted in the skeletally immature group even after biopsy alone. By contrast, four of 13 skeletally mature patients had a recurrence and required further surgery. This suggests that eosinophilic granuloma has a low rate of recurrence in skeletally immature patients

We describe six patients with aggressive granulomatous lesions around cementless total hip prostheses. Two patients previously had a cemented prosthesis in the same hip. The Lord prosthesis was used in five patients, the PCA in one. Both prostheses were made of chrome-cobalt alloy. Pain on weight-bearing occurred on average 3.2 years after the cementless arthroplasty, and at that time radiography revealed aggressive granulomatosis around the proximal femoral stem and the acetabular component in five of the patients; one had a large solitary granuloma in the proximal femur. Revision was performed on average 4.8 years after the cementless arthroplasty. At that time all granulomas had grown large in size; while waiting for revision operation, two femoral stem components fractured. All the granulomas showed a uniform histopathology, which included histiocytosis; the cause for these lesions was thought to be plastic debris from the acetabular socket

1. Forty patients with solitary eosinophilic granuloma of bone from the Hospital for Sick Children, Toronto, have been reviewed. They had clinical and laboratory findings similar to those reported by others except for the erythrocyte sedimentation rate, which was raised in nearly half the patients tested. 2. Radiographs in all cases showed the lesion to be osteolytic. In the skull and cervical spine there was no bony reaction ; in the thoracic and lumbar spine the typical picture of Calvés disease was produced; in long bones the cortex commonly showed endosteal erosion and periosteal reaction. 3. In one patient the lesion progressed so rapidly that the body of a cervical vertebra was destroyed within ten days. 4. The differential diagnosis includes sarcoma. Biopsy is essential. 5. All the patients improved regardless of the treatment they received. Complications were due either to the site of the lesion or to its treatment. 6. Expansion of the lesion after biopsy indicates a more widespread manifestation of histiocytosis X

Langerhans-cell histiocytosis (LCH) is a reactive proliferative disease characterized by the accumulation of abnormal histiocytes. The disease is broadly divided into two groups, unisystem and multisystem disease. The aetiology of LCH is unknown; the disease is currently accepted to be a reactive process rather than a malignancy. Localized LCH of bone is a benign tumour-like condition, which is characterized by a clonal proliferation of Langerhan’s-type histocytes, which infiltrate bone and cause osteolytic lesions. The common bones involved include – skull, pelvis, and diaphysis of long bones. We wanted to determine whether patient demographics at the time of presentation could help determine the clinical course and eventual outcome of the disease. We prospectively reviewed 68 patients with a primary diagnosis of LCH. Forty-six patients had unisystem disease, 22 had multisystem disease. There was a statistically significant difference in the age of presentation between the two groups. There were 6 deaths, all had multisystem disease. Of the 46 patients with unisystem disease, 31 (67.3%) underwent orthopaedic surgical intervention, 26 open biopsies and 5 curettage and bone grafting of lesions of the humeras (2), skin, clavicle and skull (1 each). There was a statistically significant difference in the average length of follow-up, between the 2 groups. We recommend closed and prolonged multidisciplinary follow-up of patient initially presenting with multisystem disease. Patients with unisystem disease can safely be discharged after a short follow-up period

Aims

We aimed to evaluate the utility of ⁶⁸Ga-citrate positron emission tomography (PET)/CT in the differentiation of periprosthetic joint infection (PJI) and aseptic loosening (AL), and compare it with ^99mTc-methylene bisphosphonates (^99mTc-MDP) bone scan.

Back pain is a common symptom in children and adolescents. Here we review the important causes, of which defects and stress reactions of the pars interarticularis are the most common identifiable problems. More serious pathology, including malignancy and infection, needs to be excluded when there is associated systemic illness. Clinical evaluation and management may be difficult and always requires a thorough history and physical examination. Diagnostic imaging is obtained when symptoms are persistent or severe. Imaging is used to reassure the patient, relatives and carers, and to guide management.

Cite this article: Bone Joint J 2014;96-B:717–23.

Giant-cell tumour of the synovium is known to affect the fingers or toes of adults. It has seldom been described in the spine and rarely in the thoracic vertebrae or in a child. The lesions of giant-cell tumour of the synovium have a classical radiological appearance, but require a high index of suspicion for correct recognition. Unlike giant-cell tumour of the synovium at other well-known sites, spinal lesions lack the characteristic papillary architecture, thereby raising other diagnostic possibilities. We describe a giant-cell tumour of the synovium of the left facet joint of a thoracic vertebra in a nine-year-old girl. The tumour was treated successfully by surgical excision.