1. The so-called adamantinoma of long bones is a clinico-pathological entity, the pathogenesis of which is still in doubt. The case for its being a synovial sarcoma showing epithelial differentiation is in our view unconvincing. 2. The tumour is slowly growing, and of low grade malignancy. Apparent cure has been effected in a third of the total cases recorded by amputation or resection of the diseased bone. 3. These means, however, have not prevented metastases to the lungs and skeleton in a similar number. 4. A case is presented in which a metastasis appeared in the chest twenty-two years after amputation of the leg. This was sensitive to telecobalt irradiation, and is the first case in which a distant metastasis has been proved microscopically

The clinical features of eight patients with myositis ossificans progressiva are described and the effects of treatment with the diphosphonate EHDP, together with surgical removal of ectopic bone, are assessed. Early correct diagnosis remains unusual, mainly because the significance of the short great toes is unrecognised, and because myositis may be mistaken for bruising, sarcoma or mumps. The diphosphonate disodium etidronate (EDHP) was given to all patients in an attempt to suppress calcification of new lesions; in five of them ectopic bone was removed during the treatment. EHDP sometimes delayed the mineralisation of newly formed bone matrix after surgical removal but this delay could not be predicted. The variable effect of EHDP may depend particularly on the amount absorbed and on the activity of new bone formation

1. An operation to re-establish the continuity of the bone after resection of tumours of the lower end of the femur or upper end of the tibia is described. 2. Sound bony union may be obtained, allowing walking with weight bearing after three to four months. 3. The combination of a massive tibial or femoral graft (Juvara procedure) with an intramedullary nail, a massive homogenous graft, and fixation of the patella to the autograft to accelerate its revascularisation. has given constant and rapid bony union in five cases. 4. This possibility gives real value to wide resection in the treatment of severe cases of giant-cell tumours. especially those that are recurrent. osteolytic or suspect of malignancy. 5. Its value in the treatment of sarcoma cannot be assessed in so short a series

1. The detailed findings are presented of a woman aged fifty who had widely distributed ossifying skeletal tumours, the structure and form of which have been shown to be low-grade osteoblastic osteogenic sarcoma. The patient was treated with heavy doses of P. 32. , to which is attributed some clinical improvement, but which induced a fatal aplastic anaemia. 2. The study of the several tumours, together with the history, the radiographs and the post-mortem findings, suggests that the lesions are multifocal skeletal primary tumours rather than numerous osseous metastases secondary to a solitary new growth in any one bone. 3. The focal neoplastic lesion is discussed in the light of personal experience of other osteogenic sarcomata of low grade but of solitary origin, and of the multifocal form of bone sarcoma which may complicate Paget's osteitis deformans

Aims

Accurate estimations of the risk of fracture due to metastatic bone disease in the femur is essential in order to avoid both under-treatment and over-treatment of patients with an impending pathological fracture. The purpose of the current retrospective in vivo study was to use CT-based finite element analyses (CTFEA) to identify a clear quantitative differentiating factor between patients who are at imminent risk of fracturing their femur and those who are not, and to identify the exact location of maximal weakness where the fracture is most likely to occur.

Of 290 Ugandan children and adolescents with proven Burkitt's lymphoma 11 had lesions in the long bones or the pelvis. These started in the medulla as small osteolytic foci which coalesced and penetrated the cortex causing subperiosteal new bone formation in layers or spicules, and giving rise to large soft-tissue masses. Common sites were the femoral and tibial diaphyses and the metaphyses around the knee. Five were in the epiphyses. Other sites were the pelvis, humerus and ulna. One patient had a lymphomatous synovial effusion of the knee. In the lower limbs the lesions were often bilateral and symmetrical. Five patients had pathological fractures. Radiologically the lesions mimicked Ewing's sarcoma, osteosarcoma, osteomyelitis, acute leukaemia, syphilis and yaws, but clinically they were relatively painless, an important differential diagnostic feature. In the five patients with sustained remissions after chemotherapy the lesions and fractures healed well and the growth plates were undamaged

The systemic effects of diabetes mellitus are well recognised. The heart, kidney, central and peripheral nervous systems, and the distal parts of the limbs are often the site of end-organ damage resulting from ischaemia. Infarction of large muscle groups in the limb, not associated with gangrene, is uncommon. There have been few reported cases other than radiological descriptions of diabetic muscle infarcts. While previous reports have illustrated some of the clinical and radiological characteristics of this condition, the paucity of published cases makes it difficult to determine the most appropriate methods of diagnosis and treatment. During a five-year period we treated 14 patients with diabetes mellitus, aged from 32 to 59 years, who were referred to a musculoskeletal oncology service for suspected soft-tissue sarcoma, but were subsequently found to have a diabetic muscle infarct. Closed needle biopsy was performed in 13 without complications. In 12 patients, the symptoms resolved without surgical treatment

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

Deep gluteal syndrome is an increasingly recognized disease entity, caused by compression of the sciatic or pudendal nerve due to non-discogenic pelvic lesions. It includes the piriformis syndrome, the gemelli-obturator internus syndrome, the ischiofemoral impingement syndrome, and the proximal hamstring syndrome. The concept of the deep gluteal syndrome extends our understanding of posterior hip pain due to nerve entrapment beyond the traditional model of the piriformis syndrome. Nevertheless, there has been terminological confusion and the deep gluteal syndrome has often been undiagnosed or mistaken for other conditions. Careful history-taking, a physical examination including provocation tests, an electrodiagnostic study, and imaging are necessary for an accurate diagnosis.

After excluding spinal lesions, MRI scans of the pelvis are helpful in diagnosing deep gluteal syndrome and identifying pathological conditions entrapping the nerves. It can be conservatively treated with multidisciplinary treatment including rest, the avoidance of provoking activities, medication, injections, and physiotherapy.

Endoscopic or open surgical decompression is recommended in patients with persistent or recurrent symptoms after conservative treatment or in those who may have masses compressing the sciatic nerve.

Many physicians remain unfamiliar with this syndrome and there is a lack of relevant literature. This comprehensive review aims to provide the latest information about the epidemiology, aetiology, pathology, clinical features, diagnosis, and treatment.

Cite this article: Bone Joint J 2020;102-B(5):556–567.

We performed positron emission tomography (PET) with . 18. fluorine-fluoro-2-deoxy-D-glucose (FDG) on 55 patients with tumours involving the musculoskeletal system in order to evaluate its role in operative planning. The standardised uptake value (SUV) of FDG was calculated and, to distinguish malignancies from benign lesions, the cases were divided into high (≥ 1.9) and low (< 1.9) SUV groups. The sensitivity of PET for correctly diagnosing malignancy was 100% with a specificity of 76.9% and an overall accuracy of 83.0%. The mean SUV for metastatic lesions was twice that for primary sarcomas (p < 0.0015). Our results suggest that the SUV may be useful in differentiating malignant tumours from benign lesions. However, some of the latter, such as schwannomas, had high SUVs so that biopsy or wide resection was selected as the first operation. Thus, some other quantitative analysis may be required for preoperative planning in cases of high-SUV neurogenic benign tumours. The reverse transcription-polymerase chain reaction revealed that the RNA message of a key enzyme in glucose metabolism, phosphohexose isomerase (PHI)/autocrine motility factor, was augmented in only high FDG-uptake lesions, suggesting that a high expression of the PHI message may be associated with accumulation of FDG in musculoskeletal tumours

We have investigated the significance of local recurrence on survival in 173 patients with localised soft-tissue sarcomas of the limbs and of the trunk. The overall survival rates at five and ten years were 75.2% and 68.0%, respectively. After definitive surgery at our hospitals, there was local recurrence in 25 patients (14.5%). After inadequate operations elsewhere, there was a higher incidence of late local recurrence (28.3%), in comparison with those with primary tumours treated by us (9.0%), or patients referred to us immediately after inadequate surgery elsewhere (10.2%). Because of small numbers these differences in the survival rates were not statistically significantly different. Univariate survival analysis showed that local recurrence after definitive surgery (p = 0.006) together with the histological grade (p = 0.0002), the size of the tumour (p = 0.002), its depth in relation to deep fascia (p = 0.003), and the surgical margin (p = 0.0001) were the significant prognostic factors. Local recurrence at the initial presentation did not affect survival. Multivariate analysis showed that local recurrence after definitive surgery also lost its apparent prognostic significance

Aims

The aim of this study was to evaluate the prosthesis characteristics and associated conditions that may modify the survival of total femoral endoprosthetic replacements (TFEPR).

Aims

The aim of this study is to evaluate the clinical results of operative intervention for femoral metastases which were selected based on expected survival and to discuss appropriate surgical strategies.

From 1986 to 1991 we fitted 20 children with endoprostheses after resection of malignant bone tumours of the leg; six have reached skeletal maturity and are the subject of this study. Reconstruction of defects in growing limbs in which the eventual shortening can be predicted requires the use of extendable prostheses. The mean age at operation was 11 years (9.2 to 13.7) and the average follow-up period was 6.3 years (4.3 to 7.6). The diagnosis was osteosarcoma in five patients and Ewing's sarcoma in one. All tumours were Enneking stage-IIB. When seen for follow-up all patients were free from disease. The extendable implants used included the Pafford-Lewis prosthesis and the Kotz Modular Femur Tibia Reconstruction system with a compatible, newly-designed growth module. Telescope-like elongation of the prostheses was performed by insertion of a screwdriver through a small skin incision. Active epiphyseal growth in the adjacent growth plate was preserved by using prosthetic stems with a smooth surface. The mean length gained was 13.15 cm (4.5 to 19.5) requiring 53 planned procedures. Seven revision operations were necessary for complications. Functional evaluation showed excellent and good results in all cases. Stress-shielding at the site of anchorage of the prosthesis was more pronounced than in adults. Implantation of extendable endoprostheses in children provides a reasonable alternative to rotationplasty, but limb salvage requires more operations

We describe a method of partial limb salvage for the treatment of large primary malignant tumours of the arm. The tumour-bearing area is resected as a cylindrical segment and the distal arm is then replanted with the necessary shortening. The method is suitable for stage-IIB tumours with or without neurovascular involvement which, because of their extent, could otherwise be adequately treated only by amputation. From 1987 to 1992 we used this method in 12 patients with primary malignant bone or soft-tissue sarcomas. Wide resection margins were achieved in all, but six patients died from their disease at a mean of 21.5 months (6 to 48), none with any local recurrence. Five patients have no evidence of disease at a mean follow-up period of 52.2 months (22 to 78), and one was lost to follow-up at 48 months postoperatively when there was no evidence of disease. The results of the functional evaluation of ten patients with a follow-up of over ten months were excellent in one, good in six and fair in three, by the criteria of Enneking (1987). Recovery after nerve reconstruction was satisfactory in all cases with sensation S3 or higher and motor function M2+ or higher. Detailed evaluation of hand function on the Millesi score rated only 22% (9.6% to 33.7%) as compared with the contralateral side, but the patients were satisfied and refused further operations for the improvement of function. These oncological and functional results allow us to recommend resection-replantation as a valuable alternative to amputation for the treatment of primary malignant tumours of the arm

1. Histochemical staining and correlated biochemical estimations of five hydrolytic enzymes were done on eighteen benign and twenty malignant fibroblastic lesions of bone and soft tissue. 2. Alkaline phosphatase activity was moderate in a fibroma and very high in fibrous dysplasia. In a typical fibrosarcoma the fibroblasts showed no enzyme activity and estimations were low. Exceptions indicated an osteogenic potential in the tumour. 3. ß-glucuronidase, leucine aminopeptidase, and to a less extent non-specific esterase, were more active in malignant than in benign lesions, and the highest activities were found in sarcomata arising in Paget's disease of bone. 4. Acid phosphatase showed no correlation with malignancy and was generally unremarkable except for high activity in osteoclasts, but was raised in two sarcomata that occurred after irradiation of giant-cell tumours. 5. A non-osteogenic fibroma and a fibrous cortical defect, though poorly represented in this series, are not uncommon; they sometimes lead to pathological fracture, but sarcoma is very rare in such lesions. They tend to show more alkaline phosphatase than fibrosarcoma but not the very high activity of fibrous dysplasia, which is related to its osteogenic potential. 6. Fibrous dysplasia most often presents in the five to fifteen age group but seldom leads to malignancy, though this may occur, usually as osteosarcoma, which has a similar high content of alkaline phosphatase. Fibrosarcoma is typically negative or very weak in this enzyme: the exceptional cases with high activity were tumours which were in part osteosarcoma. Generally the demonstration of high alkaline phosphatase activity in a fibroblastic lesion of bone, in the absence of trauma or inflammation, suggests the diagnosis of fibrous dysplasia

Aims

The sacrum is frequently invaded by a pelvic tumour. The aim of this study was to review our experience of treating this group of patients and to identify the feasibility of a new surgical classification in the management of these tumours.