European Musculoskeletal Oncology Society Research Articles

Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study

BackgroundExtra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate.Questions/purposeThis paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation.MethodsThis was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF.ResultsThree hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3–85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group.ConclusionLocal recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

What Opinions Do Tumor Reconstructive Surgeons Have about Sports Activity after Megaprosthetic Replacement in Hip and Knee? Results of the MoReSports Expert Opinion Online Survey.

Sports activity has many benefits in cancer survivors. A key one is having sport activity contribute to the well-being of the individual. However, there are no guidelines about the intensity and kind of postoperative mobility workouts after hip or knee megaprosthetic treatment. Opinion research about sports after modular bone and joint replacement may provide an understanding of surgeons’ attitudes on sports activity after megaprostheses of the hip and knee joint. A web survey with members of three international professional organizations of orthopedic tumor reconstructive surgeons was conducted between September 2016 and January 2018. Members were invited via personalized emails by the European Musculoskeletal Oncology Society (EMSOS), the International Society of Limb Salvage (ISOLS), and the Musculoskeletal Tumor Society (MSTS). The questionnaire included 26 questions. A total of 149 surgeons started the survey, and 76 finished the entire survey (American Association for Public Opinion Research (AAPOR) second response rate (RR2) EMSOS: 12.3%; ISOLS: 21.9%; MSTS: n/a). More than half of the respondents encourage sarcoma survivors after megaprosthetic treatment to reach an activity level that would allow them to regularly participate in active sporting events of University of California, Los Angeles (UCLA) activity level 7 and higher. Orthopedic tumor reconstructive surgeons do fear a number of complications (periprosthetic fracture, allograft failure/fracture, loosening, prosthetic or bearing failure, and early polyethylene wear) due to sports activity after modular bone–joint replacement, but they actually witness fewer complications than they conceptually anticipated. According to the surgeons’ opinions, between four to seven types of sports after surgery could reasonably be recommended depending on the type of hip or knee procedures. This survey provides insights into opinions on what could be recommended, what could be allowed if surgeons and their patients agree on the potential negative outcome, and which sports should definitely not be allowed after hip and knee megaprostheses.

Organization of Bone Sarcoma Care: A Cross-Sectional European Study.

ObjectiveTo assess organization of care in several bone sarcoma centers in Europe affiliated with the European Musculoskeletal Oncology Society (EMSOS) for comparison and to identify potential improvements in organization of care.MethodsData for this observational cross‐sectional study was obtained through healthcare professionals affiliated to EMSOS. The authors formulated 10 questions regarding organization of care. The questions were focused on guidance, multidisciplinary decision‐making, and data storage. A digital questionnaire was synthesized and included quality control. The digital questionnaire was sent to 54 representative members of EMSOS. We did not receive responses from 29 representative countries (53.7%) after one digital invitation and two digital reminders.ResultsWe received data from 25 representatives of bone sarcoma centers from 17 countries across Europe (46.3%). Authorization to perform oncological care in a bone sarcoma center was government issued in 41.2% of cases and based on expertise without governmental influence in 52.9% of cases. In 64.7% of the countries, a national bone tumor guideline regarding for diagnosis and treatment is used in oncological care. A national bone tumor board for extensive case evaluation including classification and advice for treatment is available for 47.1% of the countries. All participating bone sarcoma centers have a mandatory local multidisciplinary meeting before the start of treatment; in 84.0% this meeting takes place once a week. During this multidisciplinary meeting a median of 15 cases (range, 4–40 cases) are discussed. In terms of storage of oncological data, a local registry is used in eight countries (47.1%). A national registry is used in eight countries (47.1%).ConclusionsA national bone tumor board gives bone sarcoma centers with little adherence the opportunity to gain knowledge from a more experienced team. Centralization of care in a bone sarcoma center is important to lower incidences. The optimal size for a bone sarcoma center in terms of patient adherence is not known at present.

Impact of the COVID-19 pandemic on patients suffering from musculoskeletal tumours

BackgroundThe aim of the current study was to evaluate the impact of the coronavirus disease (COVID-19) pandemic on musculoskeletal tumor service by conducting an online survey of physicians.MethodsThe survey was conducted among the members of the ISOLS (International Society of Limb Salvage) and the EMSOS (European Musculo-Skeletal Oncology Society). The survey consisted of 20 questions (single, multiple-response, ranked): origin and surgical experience of the participant (four questions), potential disruption of healthcare (12 questions), and influence of the COVID-19 pandemic on the particular physician (four questions). A matrix with four different response options was created for the particular surgical procedures).ResultsOne hundred forty-nine physicians from five continents completed the survey. Of the respondents, 20.1% and 20.7% stated that surgery for life-threatening sarcomas were stopped or delayed, respectively. Even when the malignancy was expected to involve infiltration of a neurovascular bundle or fracture of a bone, still 13.8% and 14.7% of the respondents, respectively, stated that surgery was not performed. In cases of pending fractures of bone tumors, 37.5 to 46.2% of operations were canceled.ConclusionThe SARS-CoV-2 pandemic caused a significant reduction in healthcare (surgery, radiotherapy, chemotherapy) for malignancies of the musculoskeletal system. Delaying or stopping these treatments is life-threatening or can cause severe morbidity, pain, and loss of function. Although the coronavirus disease causes severe medical complications, serious collateral damage including death due to delayed or untreated sarcomas should be avoided.

Results of the 32nd EMSOS Congress (European Musculo Skeletal Oncology Society), Florence, Italy, 2019

Results of the 32nd EMSOS Congress (European Musculo Skeletal Oncology Society), Florence, Italy, 2019

Prevalence and Clinical Features of Mazabraud Syndrome: A Multicenter European Study.

Mazabraud syndrome is a rare disorder, characterized by the presence of fibrous dysplasia (FD) with associated intramuscular myxomas. Data are scarce on the prevalence, clinical features, and natural history of this disorder and outcomes. In this multicenter study, we evaluated a series of patients from 6 European centers. All centers affiliated with the European Musculo-Skeletal Oncology Society (EMSOS) were invited to include data on all patients with Mazabraud syndrome who were seen between 1980 and 2015. The study investigated the prevalence of Mazabraud syndrome, the type, severity, and localization of FD lesions in relation to myxomas, the histopathology of myxomas, and results of GNAS-mutation analysis, when available. Thirty-two patients (22 female) from 6 centers were included. The prevalence of Mazabraud syndrome was 2.2% in the combined cohort of 1,446 patients with FD, and the syndrome was diagnosed at a mean of 10.1 years after diagnosis of FD. The myxomas were predominantly localized in the upper leg. Excision was performed in 20 patients, recurrence occurred in 6 of these patients (30%) at a median of 8.5 years (range, 1.9 to 16.0 years), and revision surgery was necessary in 5 (25%). High cellularity of myxomas was associated with recurrence (p < 0.05). A GNAS mutation was identified in the myxoma tissue of 5 (83%) of 6 patients with GNAS-mutation analysis. This study is the first, to our knowledge, to provide data on the prevalence of Mazabraud syndrome in a relatively large cohort. Although the outcomes of surgical resection were good, a quarter of the patients required revision surgery despite clear resection margins. High cellularity of myxomas was associated with recurrence. GNAS mutations were identified in 83% (5 of 6), emphasizing the shared origin of FD and myxomas. Our data show that patients with FD who have disproportionate complaints, irrespective of FD type, extent, or severity, should be investigated for the possible presence of myxomas. Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Trends in the surgical treatment of pathological fractures of the long bones: based on a questionnaire among members of the Dutch Orthopaedic Society and the European Musculo-Skeletal Oncology Society (EMSOS).

The aim of this study was to assess the current trends in the estimation of survival and the preferred forms of treatment of pathological fractures among national and international general and oncological orthopaedic surgeons, and to explore whether improvements in the management of these patients could be identified in this way. All members of the Dutch Orthopaedic Society (DOS) and the European Musculoskeletal Oncology Society (EMSOS) were invited to complete a web-based questionnaire containing 12 cases. A total of 96 of 948 members of the DOS (10.1%; groups 1 and 2) and 33 of 182 members of the EMSOS (18%; group 3) replied. The estimation of survival was accurate by more than 50% of all three groups, if the expected survival was short (< 3 months) or long (> 12 months). General orthopaedic surgeons preferred using an intramedullary nail for fractures of the humerus and femur, irrespective of the expected survival or the origin of primary tumour or the location of the fracture. Oncological orthopaedic surgeons recommended prosthetic reconstruction in patients with a long expected survival. Identifying patients who require centralized care, as opposed to those who can be adequately treated in a regional centre, can improve the management of patients with pathological fractures. This differentiation should be based on the expected survival, the type and extent of the tumour, and the location of the fracture. Cite this article: Bone Joint J 2018;100-B:1392-8.

Extraskeletal osteosarcoma: A European Musculoskeletal Oncology Society study on 266 patients

PurposePrognosis of extraskeletal osteosarcoma (ESOS) is reported to be poorer than that of skeletal osteosarcoma. This multicenter retrospective study aimed to evaluate factors influencing ESOS prognosis. Patients and methodsMembers of the European Musculoskeletal Oncology Society (EMSOS) submitted institutional data on patients with ESOS. ResultsData from 274 patients treated from 1981 to 2014 were collected from 16 EMSOS centres; 266 patients were eligible. Fifty (18.7%) had metastases at diagnosis. Of 216 patients with localised disease, 211 (98%) underwent surgery (R0 = 70.6%, R1 = 27%). Five-year overall survival (OS) for all 266 patients was 47% (95% CI 40–54%). Five-year OS for metastatic patients was 27% (95% CI 13–41%). In the analysis restricted to the 211 localised patients who achieved complete remission after surgery 5-year OS was 51.4% (95% CI 44–59%) and 5-year disease-free survival (DFS) was 43% (95% CI 35–51%). One hundred twenty-one patients (57.3%) received adjuvant or neoadjuvant chemotherapy and 80 patients (37.9%) received radiotherapy. A favourable trend was seen for osteosarcoma-type chemotherapy versus soft tissue sarcoma-type (doxorubicin ± ifosfamide) regimens. For the 211 patients in complete remission after surgery, patient age, tumour size, margins and chemotherapy were positive prognostic factors for DFS and OS by univariate analysis.At multivariate analysis, patient age (≤40 years versus >40 years) (P = 0.05), tumour size (P = 0.0001) and receipt of chemotherapy (P = 0.006) were statistically significant prognostic factors for survival. ConclusionPatient age and tumour size are factors influencing ESOS prognosis. Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin.

Conference report on the 28th annual meeting of the European Musculo-Skeletal Oncology Society, 29 April-1 May 2015, Athens.

The 28th Annual Meeting of the European Musculo-Skeletal Oncology Society was organised in Athens by the local host Professor Papagelopoulos and his team. The main objective of the meeting was to focus on recent advances in the diagnosis and treatment of bone and soft tissue sarcomas. The interdisciplinary nature of the meeting was of great value—surgeons, oncologists, pathologists, radiologists, and basic researchers discussed new strategies in the war on sarcoma. This report will highlight the major findings of this successful meeting.

Highlights from the First Combined 2011 Meeting of the Musculoskeletal Tumor Society and Connective Tissue Oncology Society: Editorial Comment: Cooperation to Move the Hope Curve to the Right

Cooperation. Collaboration. Working together. When we strive to embody these values, we are better. When we do not, we are less. Our patients are our joint responsibility. When we collaborate, caring for them together, they do better. When we do not, they do less well. Sarcomas are a threat to life and limb, and surgery is our most effective treatment. In many ways, it is the ultimate targeted therapy. In most localized tumors, we can achieve a complete response much more effectively than by any other modality. As surgeons, we strategically remove tumors, preserving as much function as possible, and hope for the best. The best does not always come. Often, the complete responses are not sufficiently durable. Whenever possible, we look to our colleagues in other specialties to improve the results from surgery alone. In certain types of sarcomas, there is a role reversal: chemotherapy is the backbone of treatment, with surgery as the adjuvant for local cleanup. For example, with systemic chemotherapy, we tripled the survival rate in osteosarcoma, Ewing’s sarcoma, and rhabdomyosarcoma, compared to the rate achieved with local control alone. Similarly, in a majority of soft tissue sarcomas, we rely on the expertise of our radiation oncology colleagues to improve our ability to control the local tumor. All too often our local and systemic tools are limited, and we are left hoping for the best. New tools to enhance the impact of our care are a necessity. Enter the molecular scalpel. Biotargeting, in the form of directed monoclonal antibodies and small molecule inhibitors, to name just two, have helped save lives and move the hope curve to advantage the survival of more patients with sarcoma. Without the efforts of our research colleagues, such tools would not be available to our patients. The conditions we treat are rare. Consequently, it is only through analysis of our combined experience that we can establish reliable outcome data to determine whether our treatments are effective. This requires cooperation. Leading the way toward this goal are the Radiation Therapy Oncology Group (RTOG), Eastern Cooperative Oncology Group (ECOG), SWOG (formerly the Southwest Oncology Group), Children’s Oncology Group (COG), and the Sarcoma Alliance for Research through Collaboration (SARC), to name a few. Some of us are active in these groups; more of us should be. The Musculoskeletal Tumor Society (MSTS) has convened numerous combined meetings with the International Society of Limb Salvage (ISOLS), the European Musculo-Skeletal Oncology Society (EMSOS), and now the Connective Tissue Oncology Society (CTOS). These combined meetings bring out the best from our learned societies as we search to better understand the pathophysiology of sarcomas and treat them more successfully. We hope you enjoy reading these select symposium papers from the 2011 MSTS/CTOS combined meeting. As you read them, we hope two common themes will resonate: (1) cooperation across disciplines stimulates new ways of thinking and (2) interdisciplinary approaches enhance outcomes. A commitment to transdisciplinary cooperation will promote the synergies that we need to move beyond hope and cure more patients. On behalf of both societies, we trust you will enjoy this symposium. Fig. 1 R. Lor Randall, MD, FACS, Past President, CTOS, and Chair, Orthopaedic Committee, COG, is shown. Fig. 2 John H. Healey, MD, FACS, President, MSTS, and Past Chair, Orthopaedic Committee, COG, is shown.

Incidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients

Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases. A retrospective study was conducted using clinical data of 144 Ollier and 17 Maffucci patients from 13 European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society. Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p = 0.00l). Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with enchondromas located in long bones or axial skeleton, especially the pelvis, have a seriously increased risk of developing chondrosarcoma, and are identified as the population that needs regular screening on early detection of malignant transformation.

Le nuove frontiere della ricerca in oncologia del sistema muscolo-scheletrico

Basic science research holds the promise of rounds of discovery that will help to overcome treatment barriers for primary bone tumors. The management of primary bone sarcomas has achieved a consistently high level around the world. The growth and development of international specialty organizations such as the European Musculoskeletal Oncology Society, Musculoskeletal Tumor Society, Asia-Pacific Tumor Society, International Society of Limb Salvage, and Connective Tissue Oncology Society, as well as of domestic orthopaedic and collaborative study groups, have made great strides in educating the medical and surgical communities about the best practices in the modern care of primary bone tumors. However, these marked improvements have brought us to a plateau over the past two decades. Unacceptably high rates of tumor resistance to chemotherapy and of local and distant relapse remain. Only incremental progress has been achieved in managing the three most common bone cancers: osteogenic sarcoma (OGS), Ewing’s sarcoma, and chondrosarcoma. The management of benign tumors is generally successful, but not universally so. For example, certain benign tumors, such as giant cell tumor and aneurysmal bone cyst, have persistently high local recurrence rates. Basic science research holds the key to solve the refractory clinical problems. Important strides have been made in understanding the etiology and factors contributing to the progression of these primary tumors. This article summarizes many of the exciting ideas and results in primary bone tumor research, focusing on the most common skeletal cancer: OGS.

Dedifferentiated chondrosarcoma: Prognostic factors and outcome from a European group

Background Dedifferentiated chondrosarcoma has a very poor prognosis. Because of its rarity, there are few large studies of outcome which might identify potential prognostic factors. In particular there remains uncertainty about the value of chemotherapy for this condition. Method A retrospective study was done using data supplied by members of the European Musculo Skeletal Oncology Society (EMSOS). We obtained data on 337 patients from nine European centres with this rare condition, with details on patients, treatment and outcome which were then analysed in an attempt to identify prognostic features. Results The median age was 59 years and there was a slight predominance of males (53%). The most common sites were the femur and pelvis. Twenty-nine percent of patients with a long bone tumour had a pathological fracture. 71 patients (21%) had metastases at the time of diagnosis and these patients had a median survival of 5 months with a 10% chance of survival at 2 years. For the 266 patients without metastases at diagnosis, 254 underwent surgery with 79% having limb salvage. Thirty-one percent of these 266 patients had chemotherapy with 47% of those under 60 receiving it. In this group of 266 patients, overall survival was 28% at 10 years and poor prognostic factors were the presence of a pathological fracture at diagnosis, a pelvic location and increasing age. Local recurrence and overall survival were related to inadequate margins of excision. We did not find that the histological subtype, size of the tumour or the use of chemotherapy significantly affected outcome. For all patients the overall survival was 24% at 5 years. Conclusions The prognosis for patients with dedifferentiated chondrosarcoma remains dismal. Surgery with clear margins remains the principal treatment for this condition. Further use of chemotherapy should be within a trial or treatment protocol.

Periosteal osteosarcoma – a European review of outcome

Periosteal osteosarcoma is a rare primary malignant bone tumour. Treatment is by surgical excision, but controversy remains about the value of chemotherapy. The members of the European Musculo Skeletal Oncology Society (EMSOS) collaborated to produce a dataset of 119 patients. The predominant site for the tumour was the femur, followed by the tibia. All but 2 patients underwent surgery, with 9 requiring amputation and the others having limb salvage. A total of 81 patients had chemotherapy, of whom 50 had neoadjuvant chemotherapy. There was no standard chemotherapy regime, but all patients receiving chemotherapy were given doxorubicin combined with at least one other agent. The overall survival was 89% at 5 years and 83% at 10 years. Eight patients developed local recurrence, of whom 5 died. Survival was related to appearance of local recurrence ( P < 0.0001) but no other single factor. The use of chemotherapy was not shown to be a prognostic factor, but was used in two-thirds of the patients in this study.

Osteosarcoma over the age of forty

The European Musculo Skeletal Oncology Society (EMSOS) has carried out a retrospective review of patients over the age of 40 years with osteosarcoma. 481 patients from 12 centres or multicentric groups were included. 42 patients had osteosarcoma arising in Paget's disease, median survival was 9 months. Patients with axial or metastatic tumours also did badly whilst 41 patients with radiation-induced osteosarcoma had a prognosis paralleling conventional osteosarcoma matched for patient age and site of the tumour. 238 patients had high grade non-metastatic osteosarcoma and had a survival of 46% at 5 years. Older patients had less chemotherapy and fared worse. Osteosarcoma in the elderly is a curable condition and warrants intensive treatment with chemotherapy and surgical resection.

Malignant fibrous histiocytoma of bone: a retrospective EMSOS study of 125 cases. European Musculo-Skeletal Oncology Society.

In an effort to learn more about malignant fibrous histiocytoma (MFH) of bone and its prognosis with different treatment approaches, the European Musculo-Skeletal Oncology Society (EMSOS) initiated a retrospective survey among its members. Data requested included patient and treatment variables and outcome. The information on all patients with histologically proven, primary, localized osseous extremity MFH was analyzed if surgical tumor removal was performed and disease status was documented for at least one follow-up date. 125 such patients were evaluable (74 male, 51 female; median age 34 years; tumor site femur 81, tibia 26, humerus 12, other 6). Local treatment was surgery only (110) or surgery plus radiotherapy (15). Chemotherapy was used in 97/125. On last follow-up, 85 patients remained in remission, 33 had developed metastases, 6 a local recurrence, and 1 a combined relapse. With a median follow-up of 3.9 years for patients at risk, actuarial 5-year disease-free survival was 59%. In univariate analyses, younger age and the use of chemotherapy were associated with a more favorable outcome, as was limb-salvage surgery. 23 of 66 tumors with information on response to preoperative chemotherapy responded well (> 90% necrosis). Among these 23, only one relapsed.

European Musculoskeletal Oncology Society Amsterdam, October 6-7, 1994

A retrospective EMSOS study to assess risk factors for local recurrence in desmoid tumors was initiated in 1991. Paticnts and methods. 1.51 patients treated at 1 1 EMSOS institutions were studied. The average age was 33 (1-80) years and 82 were women Tumor locations were: upper extremity 39, lower extremity 40, trunk 49, neck 5 , other sites 13, and multiple sites 5. The average tumor size was 7 (1340) cm. 145 patients were operated on. 2 patients received preand 6 patients postoperative radiotherapy. 5 patients received medical treatment ( 1 tamoxifen, 4 chemotherapy). Histopathology review, DNA-cytometry, proliferating cell nuclear antigen (PCNA) and estrogen receptor determination were performed. Re.sdt.s. The diagnosis was confirmed in all cases. All primary tumors were diploid and estrogen receptor negative. A Cox regression analysis showed a 4.0 relative risk for upper extremity location and a 2.5 relative risk for marginal excision, respectively, for occurrence of first local recurrence. Tumor depth, vascular pattern, cellularity and PCNA index were of no prognostic importance.