Aims. The modern prevalence of primary tumours causing metastatic bone disease is ill-defined in the oncological literature. Therefore, the purpose of this study is to identify the prevalence of primary tumours in the setting of metastatic bone disease, as well as reported rates of pathological fracture, postoperative complications, 90-day mortality, and 360-day mortality for each primary tumour subtype. Methods. The Premier Healthcare Database was queried to identify all patients who were diagnosed with metastatic bone disease from January 2015 to December 2020. The prevalence of all primary tumour subtypes was tabulated. Rates of long bone pathological fracture, 90-day mortality, and 360-day mortality following surgical treatment of pathological fracture were assessed for each primary tumour subtype. Patient characteristics and postoperative outcomes were analyzed based upon whether patients had impending fractures treated prophylactically versus treated completed fractures. Results. In total, 407,893 unique patients with metastatic bone disease were identified. Of the 14 primary tumours assessed, metastatic bone disease most frequently originated from lung (24.8%), prostatic (19.4%), breast (19.3%), gastrointestinal (9.4%), and urological (6.5%) malignancies. The top five malignant tumours resulting in long bone pathological fracture were renal (5.8%), myeloma (3.4%), female reproductive (3.2%), lung (2.8%), and breast (2.7%). Following treatment of pathological fractures of long bones, 90-day mortality rates were greatest for lung (12.1%), central nervous system (10.5%), lymphoma (10.4%), gastrointestinal (10.1%), and non-renal urinary (10.0%) malignancies. Finally, our study demonstrates improved 90-day and 360-day survival in patients treated for impending pathological fracture compared to completed fracture, as well as significantly lower rates of deep vein thrombosis, pulmonary embolism, urinary tract infection, and blood transfusion. Conclusion. This study defines the contemporary characteristics of primary malignancies resulting in metastatic bone disease. These data should be considered by surgeons when prognosticating patient outcomes during treatment of their metastatic bone disease. Cite this article: Bone Jt Open 2023;4(6):424–431

The distal humerus represents 1% of all primary bone tumours. Endoprosthetic replacement can potentially improve function and provide good pain relief. We present out experience with the custom made Stanmore elbow endoprosthesis used after resection of malignant tumours of the distal humerus. Between 1970–2009 we carried out 19 endoprosthetic replacments for malignant tumours of the distal humerus. 10 were a result of metastasis and 9 were primary bone tumours. 7 patients had a pathological fracture as their first presentation and 3 had pathological fractures after the diagnosis was made. 11 patients died between 3 months to 16 year following surgery. The mean survival of the patient group was 7.1 years (range 3 months to 37 years). 4 patients underwent a revision EPR and one patient underwent two revision EPR's due to loosening. Two patients underwent maintenance procedures (rebushing) due to wear of the poly bushing. We have had no revisions since the design of hte implant was changed to a floppy hinge design. One patient underwent an above elbow amputation four years after surgery due to local recurrence. There were no early post operative infections. One patient developed a sinus requiring multiple wound explorations, one year after insertion of the endoprosthesis for a sarcoma. This patient was infection free till the time of death 3 years later. There were no nerve palsies, periprosthetic fractures or wound problems. The mean TES score was 72% (59–78%) in the surviving patients at review. As the majority of the patients were implanted for metastatic disease the initial reliablity and low complication rate of the procedure, in our series, confirms that this is a suitable reconstruction for patients in significant metastatic pain from a destructive lesion of the distal humerus, rapidly restoring function and relieving pain in a predictable manner

Aims. To evaluate mid-to long-term patient-reported outcome measures (PROMs) of endoprosthetic reconstruction after resection of malignant tumours arising around the knee, and to investigate the risk factors for unfavourable PROMs. Methods. The medical records of 75 patients who underwent surgery between 2000 and 2020 were retrospectively reviewed, and 44 patients who were alive and available for follow-up (at a mean of 9.7 years postoperatively) were included in the study. Leg length discrepancy was measured on whole-leg radiographs, and functional assessment was performed with PROMs (Toronto Extremity Salvage Score (TESS) and Comprehensive Outcome Measure for Musculoskeletal Oncology Lower Extremity (COMMON-LE)) with two different aspects. The thresholds for unfavourable PROMs were determined using anchor questions regarding satisfaction, and the risk factors for unfavourable PROMs were investigated. Results. The thresholds for favourable TESS and COMMON were 64.8 and 70.4 points, respectively. Multivariate analysis showed that age at surgery (p = 0.004) and postoperative leg length discrepancy (p = 0.043) were significant risk factors for unfavourable TESS results, while age at surgery (p < 0.001) was a significant risk factor for unfavourable COMMON-LE results. Following receiver operating characteristic analysis, the threshold for both TESS and COMMON-LE was 29 years of age at surgery. Additionally, a leg length discrepancy of 8.2 mm was the threshold for unfavourable TESS. Conclusion. Patients aged > 29 years at the time of surgery require appropriate preoperative counselling and adequate postoperative physical and socioemotional support. Reconstruction equivalent to the length of the resected bone can reduce the risk of functional disabilities in daily living. Cite this article: Bone Jt Open 2023;4(12):906–913

Purpose of the study: Epithelioid hemangioendothelioma is considered to be a low-grade malignant sarcoma. Surgical treatment is indicated. The multifocal form of the disease is always located on the right side, involving either a limb or the half-body. Based on a case report and a review of the literature, we discuss the nature of this tumor which may or may not be malignant and examine possible indications for non-mutilating surgery.

Case report: A 17-year-old female consulted in 1996 for lytic lesions involving the right lower limb. Most of the lesions were located on the lower portion of the femoral metaphysic and the upper tibia. The lesions appeared as lytic defects with minimal fracture-like lines on the rims. There was also a defect in the lower tibia shaft and in the foot. All lesions involved the right lower limb. Biopsy material obtained in July 1996 confirmed the diagnosis of grade I epithelioid hemangioendothelioma. Search for skeletal and visceral extension was negative. The classical treatment described in the literature is disarticulation of the hip. Considering the static presentation of the lesions, a conservative treatment was attempted. On July 17, 1996, the patient underwent surgery for curettage, liquid nitrogen cyrotherapy, and cement filling of the tibial and lower femoral defects where there was a risk of fracture. The other lesions were not treated. In 1999, the lesion located in the toe became painful and was treated by cement filling. The patient was discharged with simple surveillance. In 2005, i.e. 102 months after cement filling, this young patient was continuing her university education. No new lesion had developed. All lesions remained stable or regressed, including those which had not been treated.

Discussion: Multifocal epithelioid hemangioendothelioma is generally considered as a sarcoma. Careful examination of the literature (less than 50 cases published) shows many discordant elements. First, the lesions are always hemimelic on the right side, never described on the left, an unusual situation for a malignant process. In addition, the clinical courses described to not lead to death by metastasis. The only progression reported involves possible growth of known lesions. The malignant nature of this disease is thus questionable, even though the histological aspect is exactly the same as monofocal epithelioid hemangioendothelioma which can have a much more aggressive, clearly malignant, course.

Conclusion: Surgeons must not propose first-intention amputation for multifocal epithelioid hemangioendothelioma. Because of the favorable outcome observed in our patient warrants, the malignant nature of this condition is questionable.

Purpose: Infection after resection and total knee arthoplasty for malignant bone tumours in children is a serious complication which may compromise limb salvage. The purpose of this work was to study the aetiology, treatment and prognosis of this event.

Material and methods: Among the 169 total knee arthroplasties performed for malignant bone tumours between 1981 and 1999, we selected 17 patients meeting the following criteria: proven infection with identified germ on deep samples or presence of a fistula more than two years after surgery. All of the patients had osteogenic sarcomas (excluding Ewing sarcomas which account for 30% of the tumours in this localisation). The bone tumour involved the femur (n=11) or the tibia (n=6) and required extraarticular (n=14) or transarticular (n=2) resection. Infections were primary (n=9) developing after the first operation, or secondary (n=8) to surgical revision in six, joint wound in one, or haematogeneous dissemination in one patient. The causal germ was identified in thirteen patients (76%) and was a staphylococcus in all cases. Treatment included systemic antibiotics and lavage (n=10), one-procedure change in prosthesis (n=3), removal of the implant with replacement by a spacer (n=2), surgical abstention (n=2), or amputation (n=1).

Results: Mean follow-up was eight years (2 – 16 years). On the average, treatment of infection lasted 51 months and required a 3.9 surgical interventions. At last follow-up, infection was considered cured in 70% of the patients who were free of clinical or laboratory signs of infection without antibiotics for at least one year. The arthroplasty could be preserved in one-third of the cases (22% of the primary infections and 50% of the secondary infections). Another treatment, arthrodesis (n=6), Borggreve procedure (n=1), or amputation (n=4), was given in the other two-thirds.

Discussion: The 10% complication rate observed here is in agreement with data in the literature. Development of primary infection is influenced more by the histology of the tumour and the presence of skin wounds (methotrexate) than by tumour site or type of resection. The diagnosis of primary infection is made late, often at the end of the postoperative chemotherapy protocol. Changing the implant is the ideal treatment. Secondary infection is characteristically less difficult to diagnose; infection is recognised earlier and the chances of preserving the implant are better.

Brachial plexus tumours (BPT) and peripheral nerve sheath tumours (PNST) are largely benign in nature, with malignant tumours being rare and presenting significant surgical challenges. Excision of benign tumours may relieve pain and other symptomology. This retrospective study analysed data from 138 PNST and 92 BPT patients managed by a single consultant orthopaedic or plastic surgeon experienced in nerve tumour surgery between January 1999 to December 2019. The most common benign tumours were schwannomas and neurofibromas, with sarcomas being the most common malignant tumour. In the PNST group 30 patients were managed by observation only. Twenty patients underwent trucut biopsy, 21 patients underwent biopsy and surgical excision and 56 patients underwent surgical excision only. There were nine complications, with two significant neurological deficits requiring further surgical intervention. No recurrence of tumours occurred in this group. In the BPT group 16 patients were managed by observation only. Seven patients underwent trucut biopsy, 16 patients biopsy and surgical excision and 44 BPT patients underwent surgical excision only. Sixteen patients had complications with two significant complications requiring urgent further surgical intervention. Seven patients had recurrence of tumours which presented as metastases, with three patients requiring further surgery to remove recurrence of tumours. BPT patients are more complex and present with both benign and malignant lesions and are therefore more prone to complications due to the complex nature of the surgery and higher recurrence rate of tumours than PNST. Benign tumours in both groups can be safely managed conservatively if patients’ symptomology is acceptable

Aims. Malignant tumours of the foot and ankle are rare, but easily missed. NICE guidelines for bone and soft tissue tumours may be less appropriate for the foot and ankle than elsewhere. The purpose of this study was to identify the clinical features and treatment of malignant tumours arising in the foot and ankle to see if guidelines should be modified. Patient and Methods. This was a retrospective review of patients presenting to the Bone and Soft Tissue Tumour Service with a suspected tumour of the foot or ankle. Between March 1998 and July 2009, 132 patients were identified from a prospectively collected database of patients reviewed at a weekly multidisciplinary meeting. Results. Of 132 patients, 43 had benign tumours, 26 malignant tumours and 65 tumour like conditions (eg. ganglions, epidermal cysts, osteophytosis). In the malignant tumour group, the median duration of symptoms prior to presentation was 24 months, with a painful, small but enlarging mass being the most common clinical presentation. In 4 of the 26 cases (12%) unplanned excision had been undertaken prior to referral. Of the 26 malignant tumours, 4 were primary bone tumours (1 Ewing's sarcoma, 1 osteosarcoma and 2 chondrosarcomas) and 22 were soft tissue tumours of which 9 (41%) were synovial sarcomas. In 15 of 26 (58%) of cases the malignant tumour was high grade. In 10 of 26 (39%), amputation was required in order to achieve curative margins and 7 (25%) cases required soft tissue reconstructive surgery following tumour resection. Conclusions. The majority of malignant tumours in the foot and ankle are soft tissue in origin and high grade. Their clinical presentation can make early detection challenging and a high index of suspicion is required. In this review most malignant tumours presented as longstanding, small but enlarging, painful masses. Specific guidelines for investigation and referral may be warranted in addition to the current NICE recommendations

Purpose of the study: We present our cases of hemangiopericytomas treated surgically in our department of orthopaedic oncology. The purpose of this work was to study this vascular tumour rarely reported in the orthopaedic surgery in order to establish epidemiological, management, and prognostic elements. Material and methods: This was a retrospective descriptive study of patients seen since 1995 with benign (n=4) or malignant (n=16) hemangiopericytoma. The large majority were FNCLCC grade 2. Mean follow-up was ten years. Course before diagnosis was longer for benign tumours (14 years) than malignant tumours (0.9 years). Tumour localization was the lower limb (55%), the upper limb (3%) (constantly involving the scapular girdle) and isolated tumours (sternum, dorsal tumours). Two patients presented metastasis at diagnosis. A biopsy was obtained in all cases. Results: The sample contained soft haemorrhagic tissue in all cases. The typical histology was a proliferating vascular architecture with either a fibrous or muscular component. Immunohistochemistry studies revealed positive labelling for vimentin and CD99. The diagnosis of malignancy was particularly difficult, often because of the poor knowledge of pericyte differentiation. For the benign tumour, treatment consisted in resection followed by clinical and radiographic surveillance every six months. No recurrence was observed at more than five years follow-up. For the malignant tumours, all patients were given neoadjuvant chemotherapy followed by wide resection. Adjuvant chemotherapy, determined according to the resection margins and presence of not of metastasis, was instituted for 60% of patients and associated with radiotherapy for two. The total-remission survival for malignant tumours was 60% at five years. Local recurrence was noted in 18% of patients and development of metastasis in 10%. Mortality was 20%. Discussion: The histology diagnosis was not straightforward, particularly for confirming the benign nature of the tumour; search for specific markers is needed for routine diagnosis. Conclusion: The results obtained in this series provide valuable information for the diagnosis of sarcoma, particularly concerning the controversial issue of the aggressiveness of malignant tumours

Introduction: Malignant tumours of the foot and ankle are rare. The aim of the study was to document one of the largest series of malignant tumours affecting the foot and ankle and to assess the outcomes following limb salvage and amputation. Methods: The study was a retrospective review of the patients with a malignant tumour of the foot and ankle. Demographic details, diagnosis, treatment and outcomes were retrieved from the electronic patient records containing information on over 20 000 patients seen over a 25 year period. Results: Two hundred and twenty five patients had malignant tumours affecting the foot and ankle. It was common in the fifth decade (35 patients). The mean age was 46 years. The commonest diagnosis was synovial sarcoma (40 patients) followed by chondrosarcoma (23 patients) and Ewing’s sarcoma (21 patients). The mean tumour size was 5.6 cm (0.8 to 17.5 cm). 82 patients (37%) underwent an unplanned excision and 13% (29 patients) presented with metastases at diagnosis. Primary bone tumours were 28% (64 patients), soft tissue sarcomas were 62% and metastatic tumours were 8% while lymphoreticular malignancies were (1%). Limb salvage was possible in 71% (156 patients). 29% (65 patients) had a below knee amputation. 7% (15 patients) had a local recurrence. The 5 years survival was 63%. The 5 years survival for the patients who had limb salvage was 68% compared with 54% for the patients who had an amputation (p 0.03). Conclusion: Though amputation can provide better local control, limb salvage surgery improves survival

Background: Amputation of the distal fibula for malignant tumors is accepted practice. Few studies have reported limb salvage surgery for malignant tumors of the distal fibula. After distal fibulectomy, the main concerns are local recurrence of the tumor and ankle instability and deformity related to total resection of the lateral malleolus. Our objective was to analyze the oncologic and functional outcome of lateral malleolus en bloc resection for malignant tumors of the distal fibula, with special attention to operative techniques, reconstruction methods, and postoperative complications. Methods: The authors identified ten patients who had malignant tumors of the distal fibula requiring total resection of the lateral malleolus. The patients’ medical records, operative reports, radiographs, and the histologic specimens were reviewed. There were four children (mean age, 7.5 years) and six adults (mean age, 42.16 years). The distal metaphysis was involved in seven patients and the epiphysis in three. There were four osteosarcomas, three chondrosarcomas, two Ewing sarcomas, and one adamantinoma. The most common symptoms at presentation were a palpable mass in nine patients, ankle pain in six, and pathologic fracture in one. The mean duration of symptoms before diagnosis and treatment was nineteen months. All patients had operative treatment; two patients with osteosarcoma had perioperative chemotherapy and one patient with Ewing sarcoma had radiotherapy and chemotherapy and another patient with Ewing sarcoma had chemotherapy only. Two types of “en bloc” resection of the distal fibula were performed. Wide (type II) extra-articular resection was performed in seven patients. Marginal (type I) intra-articular resection of the distal fibula was performed in three patients. After resection, a primary ankle arthrodesis was performed in four adults and postoperative bracing without any reconstruction in four children and two adults. Results: Within a mean follow-up time of 14.4 years (range, three to thirty years), tumor recurred locally in two patients after a marginal type I resection of a chondrosarcoma, and in one after a type II wide extra-articular resection of an osteosarcoma. All ten patients were disease-free at latest follow-up examination. Six patients had reoperation. A below-knee amputation was performed in three patients for chronic osteomyelitis, for local recurrence of chondrosarcoma, and for a late adamantinoma of the tibia. One patient had further soft tissue and bone reconstructive surgery for lateral talus subluxation and cavovarus deformity. Another patient required ankle arthrodesis for recurrent ankle instability and ankle joint degenerative changes. One patient had wide re-resection for local recurrence of an osteosarcoma. All ten patients were ambulatory at latest follow-up evaluation. Four adult patients who underwent primary arthrodesis and one child who had no initial reconstruction and had late ankle arthrodesis had a satisfactory outcome, with an ISOLS functional score of 27.6 (92%). Two adolescents who had postoperative bracing without any soft tissue reconstruction had an ISOLS functional score of 24 (80%) with no ankle pain and satisfactory function; they used an ankle-foot orthosis during sports activities. Three patients who subsequently required below-knee amputation used a below-knee prosthesis for ambulation. Conclusions: Limb salvage surgery for high-grade malignant tumors of the distal fibula can be achieved by wide extra-articular resection. For low-grade malignant tumors or high-grade tumors responding to adjuvant therapy, a more conservative marginal intra-articular resection may be adequate. Primary arthrodesis is indicated in adults after wide extra-articular resection. In children, repair of the lateral soft tissues and reconstruction of the tibiofibular mortise is necessary after tumor resection to avoid late ankle deformity or instability

Primary bone tumours in the elderly population are relatively rare. We reviewed the Leeds regional bone tumour registry between 1990–1999 and found them to constitute only 43 of the 341 (12%) bone tumour cases. Malignant tumours (65%) were more common than benign tumours with primary tumours accounting 92 % and metastatic tumours only 8 % of all the malignancies. Females were more affected than males (55% versus 45 %). Chondrosarcoma was the most frequent tumour, constituting 24% of primary malignant tumours and 18 % of all bone tumours. Chondroma was the most common benign tumour accounting for 50% of all benign tumours, and 11% of all tumours. Survival rate was relatively poor in elderly population with primary malignant tumours. The majority of malignant tumours were in the lower limb (femur 25%, tibia 14 %).The upper limb accounted for 14% and the axial skeleton 5%. Bone tumour registries provide a valuable source of cumulative information about both common and uncommon tumours. Such information could not easily be gathered by personal experience. It is also a very good source of information for research education and service

Primary bone tumours of the clavicle are rare. Currently the existing literature is limited to a single case series and case reports or cases. Information regarding the patient's demographics and tumour types is therefore limited. The aim of this study was to investigate the and also suggest a management protocol for suspected primary bone tumours of the clavicle. We retrospectively reviewed the Scottish Bone Tumour Register from January 1971 to January 2012 and included all primary bone tumours of the clavicle. We identified only sixteen primary bone tumours over forty one year's highlighting the rarity of these tumours. There were ten benign and six malignant tumours with a mean age of 32 years (Range 4 to 66). The average presentation to orthopaedics after onset of symptoms was two months with five patients presenting following a pathological fracture. Malignant tumour types identified were consistent with previous literature with two cases of Ewing's sarcoma and osteosarcoma and a single case of osteosarcoma post radiotherapy and a single case of chondrosarcoma. Benign tumours were treated effectively with intralesional procedures. Malignant tumours were treated with wide local excision and subtotal or total clavicle excision. We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the clavicle. This is the largest series of primary bone tumours of the clavicle in the literature

Introduction: Swellings of the medial end of the clavicle in children and adolescents are common. The vast majority represent either osteomyelitis, or a benign conditions such as eosinophilic granuloma. Their radiographic and early clinical appearance, however, are difficult often to discriminate from Ewing’s sarcoma. Unrewarding biopsies are often performed. Aims: To identify incidences of malignant neoplasm at the medial end of clavicle in patients aged 18 and under from the Scottish Bone Tumour Registry (SBTR), and by questionnaire from European tumour databases. Methods: The SBTR was searched for childhood malignant neoplasms as above. Questionnaires were sent to 171 European regional and national tumour databases. Information requested included details of patients with malignant neoplasms as above, longevity of database operation and population served. An estimate of European child population rates was made based on a conservative (North Italian) calculation of children aged under 18 representing 14.4% of the population (comparison our region 21.9%). Confidence intervals were calculated using Wilson method. Results: The SBTR identified 7 children with neoplasms of the clavicle out of a total register of 4009 cases. Only 1 was malignant and this at the lateral end. Responses were gained from 56 European databases. National databases were less able to identify this detailed anatomical site than regional ones. 30 databases from 12 countries responded with complete data representing 1.619 billion total population person-years. Four malignant tumours at the medial end of clavicle were identified from a total of 233.2 million children-years at risk. Discussion: By extrapolation, in our region (population 779,000) a malignant childhood medial clavicle tumours is estimated to occur once every 342 years (95%CI 91 years – ∞). It is recommended that biopsy of such childhood swellings should be restricted to patients who present with features atypical of infection or benign lesions

The use of routine sampling for histological analysis during revision hip replacement has been standard practice in our unit for many years. It is used to assess for the presence of inflammatory processes that may represent peri-prosthetic infection. Our study examines 152 consecutive patients who underwent revision hip replacement in our centre for all reasons, excluding malignant neoplasm or metastasis. We reviewed the cases from a prospectively collated database, comparing microbiology results with histology results. Both microscopic and macroscopic analysis by specialist musculoskeletal histopathologist was included in our study. We found 17 (11.2%) patients had cultured bacteria from intra-operative samples. Eight patients (5.3%) had histological findings interpreted as infection. Only one patient who had macroscopic and microscopic histology findings suggestive of infection also had culture results that identified a pathogen. Furthermore, the macroscopic analyses by the histopathologist suggested infection in nine patients. Only one patient with positive culture in greater than 2 samples had histological features of infection. Of the 4 patients who were found to have 3 or more samples where an organism was identified only one had histological features of infection. This represents 25% sensitivity when using histology to analyse samples for infection. Of the 8 patients who had both macroscopic and microscopic features of infection only 1 patients cultured bacteria in more than 3 samples (PPV 12.5%). Our experience does not support the routine sampling for histology in revision hip replacement. We suggest it is only beneficial in cases where infection is suspected or where a multi-procedure, staged revision is performed and the surgeon is planning return to theatre for the final stage. This is a substantial paradigm shift from the current practice among revision arthroplasty surgeons in the United Kingdom but will equate to a substantial cost saving

Purpose of the study: Available epidemiological data on hand and wrist tumours are scarce and sometimes discordant. In our unit, these tumours are managed conjointly by hand surgeons and tumour specialists. We conducted an epidemiological study of 624 tumours treated from 1980 to 2008. Material and methods: The recruitment used three methods: diagnostic coding in the database, analysis of discharge letters, study of tumour registries. All hand and wrist tumours treated surgically in our unit were included retrospectively. Exclusion criteria were: patients aged less than 15 years and/or managed in the paediatric surgery unit; poorly identified cases; recurrences. Results: The study population included 624 tumours (375 female and 249 male). Mean age was 48 years (range 16–94). Eight tumours were malignant: 4 skin, 3 soft tissue, 1 bone metastasis of a primary renal tumour. Soft tissue tumour concerned 525 patients (84.1% of the study population). Respectively, 71 tumours concerned bone and 28 skin. There were 221 synovial cysts. The bone tumours exhibited a harmonious distribution for age and gender with a peak from 35 to 50 years and a sex ratio of 1/1. There were 43 chondromas found at all ages, mainly in long bones. Discussion: Our series is the third largest reported. A review of the literature identified the eight largest studies available. For 6452 tumours, 81.7% concerned soft tissues, 13% skin, 4.7% bone tissue. These lesions occurred at all ages with female predominance (60%). Malignant tumours were found in 4.4% of the cases. Exclusion of the paediatric cases and the retrospective nature of the data collection were the main biases of this work. Conclusion: Data on 624 hand and wrist tumours were in agreement with published work. Tumours involved mainly soft tissues. Synovial cysts predominated. Chondromas accounted for 70% of the bone tumours. Malignant tumours were rare (2.9%). For suspect cases, we recommend referral to a specialised centre for the management of malignant tumours of the hand. A pluridisciplinary analysis is indicated to adapt the diagnostic and therapeutic strategy

Purpose of the study: Malignant tumours of the thoracic wall are rare. Treatment protocols include extensive surgical resection. In children, these resections can generate very severely progressive scoliosis. We studied the local conditions favouring the development of scoliosis in a consecutive series of eight patients. Material and methods: From November 2004 to December 2007, six boys and two girls, mean age 7.6 years (range 4 months – 15 years) underwent extensive thoracic wall resection for a malignant tumour: Ewing sarcoma (n=5), spindle-cell sarcoma (n=2), neuroblastoma (n=1). All patients received adjuvant chemotherapy followed by resection removing on average 3.1 rigs (range 1 – 4 ribs). Six resections involved the costovertebral angle and three were associated with partial pneumonectomy. The number of ribs resected was noted in Roman numerals. The thoracic wall was divided into three sectors in the horizontal plane according to the level of the resection (A, B, and C, posteriorly to anteriorly). Reconstruction was achieved with a Gortex plaque (n=1), Borreli staples (n=2). Posterior spinal instrumentation was performed in one patient. Despite in sano resection, six patients underwent postoperative radiotherapy. Results: At mean 2.9 years follow-up (range 1 – 5 years), four patients of the eight developed scoliosis convex on the operated side. These patients had IVA resections for three patients (50, 50 and 32° scoliosis) and type IA-IIB for one patients (13° scoliosis). These deformities occurred despite a corset and instrumentation in one patient. None of the patients had a humpback. Patients who did not develop scoliosis had resections IA-IIB, IA-IB, IVC and, IC. Discussion: The type of reconstruction, the histological type, use of complementary postoperative radiotherapy or pulmonary resection did not appear to be factors favouring the development of scoliosis. Conversely, resection in zone A (posterior) over more than two adjacent levels, might be a predictive element of the risk of scoliosis. Conclusion: In the event of a resection of ribs in a posterior zone over more than two levels for resection of a malignant tumour, posterior instrumentation should be discussed. This preliminary work offers evidence for thought but needs to be strengthened with a multicentric study to enable a statistical analysis

Purpose: We report a series of patients with malignant tumours of the pelvis that had a tissue expander inserted in the pelvis to facilitate radical radiotherapy, and report functional outcomes following treatment. Introduction: Surgery for malignant tumours affecting the pelvis is challenging. Some tumours are suitable for internal hemipelvectomy and reconstruction, some require hindquarter amputation and some are inoperable. Overall prognosis is poor with high morbidity and mortality rates. There may be a place for alternative treatment with the insertion of pelvic spacers to facilitate radical radiotherapy. This is indicated in patients who have an inoperable tumour, who decline amputation, or who had an internal hemipelvectomy with close margins and high risk of local recurrence. Methods & Results: We performed a retrospective review of all patients who presented with a malignant tumour of the pelvis and who underwent an insertion of a pelvic spacer followed by local high dose radiotherapy. Available patients were followed up and evaluated using the Musculoskeletal Society Tumour Score (MSTS) and the Toronto Extremity Salvage Score (TESS). There were ten patients; 5 had Ewing’s sarcoma, 3 had osteosarcoma, 1 had spindle cell sarcoma and 1 had alveolar soft part sarcoma. 4 patients had metastases on presentation. The average age was 30 years (14 to 56 years), and average follow-up was 15 months (12 to 24 months). 4 patients died and 6 are still alive. There were no surgical complications. The average length of hospital stay was 6 days (2 to 10 days). Patients averaged an MSTS score of 63% and a TESS of 67%. Conclusion: Radical radiotherapy after spacer insertion offers an alternative to morbid surgery and is associated with good functional outcomes

Introduction: Surgical treatment options of malignant tumors of the pelvis were traditionally very limited, and often resulted in an amputation. With development of neo/-adjuvant therapies, limb-salvage surgery has become the treatment of choice. Still, the treatment remains challenging, and options for pelvic reconstruction after resection such as allografts, radiated autografts, saddle-prosthesis, custom made prosthesis and modular reconstruction systems are all associated with high complication rates. Aim of our retrospective study was to evaluate the results after reconstruction of the pelvis with a custom-made pelvic megaprosthesis. Materials and Methods: From 1977 to 2008, a total of 92 patients with malignant tumors or metastases of the pelvis were treated by resection and reconstruction with custom-made pelvic megaprostheses at our institution. Mean age was 56.2 years [17–77] with 43 male patients and 59 female. We treated 45 primary tumors and 47 metastases. Primary tumors included chondrosarcoma (45,4%), Ewing’s sarcoma (18,2%), malignant fibrous histiocytoma (9,1%), osteosarcoma (4,5%) and others. In the group with metastases we found renal cell carcinoma (52,4%), mamma-carcinoma (14,3%), thyroid carcinoma (9,5%), oropharyngeal carcinomas (9,5%), and others. Wide resection was performed in 11 patients, marginal-resection in 47, and intralesional-resection in 34 patients. Depending on the bone defect after tumor resection, pelvic megaprostheses were implanted either in the superior part of the iliac wing, the sacrum and/or the lower lumbar spine. Results: Patients were reassessed repeatedly at two different time points resulting in a mean follow-up of 3,6 years [range 0,5 to 8,4years]. A total of 55 patients were available for follow-up, 29 had died and 6 Patients were not available for evalutation (living abroad). In the group with malignant tumors 50% of the patients were alive after 5 years; 50% of patients with metastasis survived at least 2 years. The local recurrence rate was 15%. In 2 of these patients local re-resection was possible, and 5 patients were treated with secondary external hemi-pelvectomy. Aseptic failure of the megaprosthesis was observed in 3%. The MSTS-Score showed good results in 34%, fair results in 42% and poor results in 23%. We found infections in 14%. Nerve palsy occurred in 11%, thombosis in 5%, and dislocation of the prosthesis in 15% of patients. Discussion: Our study demonstrates that reconstruction of pelvic bone defects after tumor resection with custom-made megaprosthesis allows limb-salvage surgery with satisfying functional results. In spite of the relatively high complication rates-which are comparable or even favorable to other reconstruction techniques-we consider the custom-made megaprosthesis our treatment of choice to reconstruct pelvic bone defects

The initiation and progression of malignant tumors are supported by their microenvironment: cancer cells per se cannot explain growth and formation of the primary or metastasis, and a combination of proliferating tumor cells, cancer stem cells, immune cells, mesenchymal stromal cells and/or cancer-associated fibroblasts all contribute to the tumor bulk. The interaction between these multiple players, under different microenvironmental conditions of biochemical and physical stimuli (i.e. oxygen tension, pH, matrix mechanics), regulates the production and biological activity of several soluble factors, extracellular matrix components, and extracellular vesicles that are needed for growth, maintenance, chemoresistance and metastatization of cancer. Both in osteosarcoma and bone metastases from carcinomas this aspect has been only recently explored. In this lecture, I will discuss the role of tumor microenvironment, with a particular focus on the mesenchymal stroma, contributing to bone tumor progression through inherent. The most recent advances in the molecular cues triggered by cytokines, soluble factors, and metabolites that are partially beginning to unravel the axis between stromal elements of mesenchymal origin and bone cancer cells, under different microenvironmental conditions, will be reviewed providing insights likely to be used for novel therapeutic approaches

Aims

The aim of this study was to investigate the safety and efficacy of 3D-printed modular prostheses in patients who underwent joint-sparing limb salvage surgery (JSLSS) for malignant femoral diaphyseal bone tumours.