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Surface bone sarcomas are rare malignant bone tumours. Osseous and cartilaginous surface bone sarcomas are the most common, with parosteal and periosteal osteosarcomas, periosteal chondrosarcomas and secondary peripheral chondrosarcomas being the most frequent.
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Their clinical symptoms are non-specific and include pain for several months, swelling and limited range of motion of the adjacent joints.
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Prompt diagnosis is important, as biological behaviour, imaging and histopathologic characteristics, treatment and prognosis differ considerably from their conventional intramedullary counterparts. Moreover, their imaging characteristics are not infrequently non-characteristic and may be misinterpreted as juxtacortical benign lesions leading to incorrect diagnosis and treatment, with life-threatening repercussions. Molecular studies and histopathological sampling are essential for accurate diagnosis.
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There are still numerous issues regarding the biology, pathophysiology and treatment options of these entities due to their rarity.
Cite this article: EFORT Open Rev 2021;6:905-917. DOI: 10.1302/2058-5241.6.210064
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Giant cell tumour of bone (GCT) is a relatively rare, locally aggressive benign neoplasm observed in the long bone epiphyseal-metaphyseal regions of young adults.
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The optimal treatment strategy for these tumours remains controversial, and a huge amount of contradictory data regarding the functional and oncological outcomes can be found. Therefore, we performed a systematic review intended to investigate the functional and oncological outcomes after surgical treatment of GCTs arising around the knee, namely in the distal femur and proximal tibia.
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A trend towards better oncological control was found using wide resections, nonetheless, curettage-based techniques achieve a highly acceptable recurrence rate with overall better knee function. A slight advantage favouring proximal tibia GCTs regarding the Musculoskeletal Tumor Society (MSTS) score was also observed.
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Prospective studies comparing groups of more homogeneous patients, tumours, and treatment options should be developed to obtain more conclusive and definitive results regarding the optimal strategy for treating GCTs.
Cite this article: EFORT Open Rev 2021;6:641-650. DOI: 10.1302/2058-5241.6.200154
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Myositis ossificans (MO) is a benign bone formation in an extra-skeletal location. The most common subtype of MO, the post-traumatic, usually develops in young males after a traumatic event or sports injury.
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MO may simulate malignant bone lesions such as extra-skeletal or surface osteosarcomas, or soft tissue sarcomas such as synovial sarcoma or undifferentiated pleomorphic sarcoma. In the early phase the diagnosis of MO is challenging because imaging and histopathological findings may be non-characteristic.
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Detailed medical history as well as clinical examination, follow-up imaging studies and histological assessment are crucial for a proper diagnosis. Early and accurate differential diagnosis between MO and malignant soft tissue and bone tumours is important to maximize.
Cite this article: EFORT Open Rev 2021;6:572-583. DOI: 10.1302/2058-5241.6.210002
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In patients with metastatic or unresectable soft tissue and bone sarcoma of extremities and pelvis, survival is generally poor. The aim of the current systematic review was to analyse recent publications on treatment approaches in patients with inoperable and/or metastatic sarcoma.
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Original articles published between 1st January 2011 and 2nd May 2020, using the search terms ‘unresectable sarcoma’, ‘inoperability AND sarcoma’, ‘inoperab* AND sarcoma’, and ‘treatment AND unresectable AND sarcoma’ in PubMed, were potentially eligible. Out of the 839 initial articles (containing 274 duplicates) obtained and 23 further articles identified by cross-reference checking, 588 were screened, of which 447 articles were removed not meeting the inclusion criteria. A further 54 articles were excluded following full-text assessment, resulting in 87 articles finally being analysed.
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Of the 87 articles, 38 were retrospective (43.7%), two prospective (2.3%), six phase I or I/II trials (6.9%), 22 phase II non-randomized trials (27.6%), nine phase II randomized trials (10.3%) and eight phase III randomized trials (9.2%). Besides radio/particle therapy, isolated limb perfusion and conventional chemotherapy, novel therapeutic approaches, including immune checkpoint inhibitors and tyrosine kinase inhibitors were also identified, with partially very promising effects in advanced sarcomas.
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Management of inoperable, advanced or metastatic sarcomas of the pelvis and extremities remains challenging, with the optimal treatment to be defined individually. Besides conventional chemotherapy, some novel therapeutic approaches have promising effects in both bone and soft tissue subtypes. Considering that only a small proportion of studies were randomized, the clinical evidence currently remains moderate and thus calls for further large, randomized clinical trials.
Cite this article: EFORT Open Rev 2020;5:799-814. DOI: 10.1302/2058-5241.5.200069
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Diffuse-type tenosynovial giant-cell tumours of the knee (D-TGCT) have a very high complication rate.
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The recurrence rate for D-TGCT is mainly dependent on an initially successful resection of the lesion.
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The standard of care for this disease involves early surgery with synovectomy. Available surgical techniques may include an arthroscopic or open surgery; however, there is a lack of consensus on which technique should be used, and when.
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Arthroscopic excision is effective in minimizing morbidity and surgery-related complications, while an open surgical technique provides a more successful resection with a lower incidence of local recurrence.
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We could not conclude with confidence which of the surgical techniques is better at stopping a progression towards osteoarthritis and the need for a total knee arthroplasty.
Cite this article: EFORT Open Rev 2020;5:339-346. DOI: 10.1302/2058-5241.5.200005
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Chondrosarcomas in children and adolescents are uncommon and constitute < 5% of all chondrosarcomas. There are very few studies discussing extremity chondrosarcomas in young patients.
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The pelvis is the most common site, followed by the proximal femur.
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As cartilaginous tumours can be quite challenging to diagnose, it is best for these lesions to be discussed in a multidisciplinary meeting which includes a radiologist and a pathologist specializing in bone tumours.
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Treatment principles are similar to those in adults, with adequate surgical excision respecting oncologic principles being the mainstay of treatment. Select extremity Grade I chondrosarcomas may be managed with extended intralesional curettage without increasing the risk for local recurrence or metastatic disease, but case selection is critical and should be based on clinical, imaging and histological characteristics.
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Chondrosarcomas are resistant to chemotherapy and relatively radioresistant. For mesenchymal chondrosarcomas, there may be a role for chemotherapy, though data on this is limited.
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Prognosis and rate of recurrence correlate directly to the adequacy of the surgical resection.
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Chondrosarcomas in younger patients behave in a similar fashion to those in adults, and outcomes in the young are no different from those in adults.
Cite this article: EFORT Open Rev 2020;5:90-95. DOI: 10.1302/2058-5241.5.190052
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Surgery of long bone metastases is associated with a significant risk of perioperative blood loss, which may necessitate blood transfusion.
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Successful embolization (> 70% obliteration of vascularity) can be achieved in 36–75% of cases.
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The reported rate of embolization-related complications is 0–9%.
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Three out of six level III evidence studies showed a reduction in perioperative blood loss and/or blood transfusion requirement after preoperative embolization of renal cell carcinoma metastasis in long bones; three out of six studies did not.
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One level III evidence study did not show a reduction in perioperative blood loss and/or transfusion requirement after preoperative embolization of hepatocellular carcinoma metastases in long bones.
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There were no studies found that support preoperative embolization of thyroid metastases or other frequent long bone metastases (e.g. mamma carcinoma, lung carcinoma, or prostate carcinoma).
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The clinical level of evidence of the studies found is low and randomized studies taking into account primary tumour, location of metastases and type of surgery are therefore desired.
Cite this article: EFORT Open Rev 2020;5:17-25. DOI: 10.1302/2058-5241.5.190013
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Soft tissue tumours of the elbow are mostly benign. Malignant tumours in this area, although uncommon, often present unique clinical and histopathological characteristics that are helpful for diagnosis.
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Management of soft tissue tumours around the elbow may be challenging because of their rarity and the proximity to neurovascular structures. Careful staging, histological diagnosis and treatment are essential to optimize clinical outcome. A missed or delayed diagnosis or an improperly executed biopsy may have devastating consequences for the patient.
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This article reviews the most common benign and malignant soft tissue tumours of the elbow and discusses the clinicopathological findings, imaging features and current therapeutic concepts.
Cite this article: EFORT Open Rev 2019;4:668-677. DOI: 10.1302/2058-5241.4.190002
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Tumour endoprostheses have facilitated limb-salvage procedures in primary bone and soft tissue sarcomas, and are increasingly being used in symptomatic metastases of the long bones.
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The objective of the present review was to analyse articles published over the last three years on tumour endoprostheses and to summarize current knowledge on this topic. The NCBI PubMed webpage was used to identify original articles published between January 2015 and April 2018 in journals with an impact factor in the top 25.9% of the respective category (orthopaedics, multidisciplinary sciences).
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The following search-terms were used: tumour endoprosthesis, advances tumour endoprosthesis, tumour megaprosthesis, prosthetic reconstruction AND tumour. We identified 347 original articles, of which 53 complied with the abovementioned criteria.
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Articles were categorized into (1) tumour endoprostheses in the shoulder girdle, (2) tumour endoprostheses in the proximal femur, (3) tumour endoprostheses of the knee region, (4) tumour endoprostheses in the pelvis, (5) (expandable) prostheses in children and (6) long-term results of tumour endoprostheses.
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The topics of interest covered by the selected studies largely matched with the main research questions stated at a consensus meeting, with survival outcome of orthopaedic implants being the most commonly raised research question.
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As many studies reported on the risk of deep infections, research in the future should also focus on potential preventive methods in endoprosthetic tumour reconstruction.
Cite this article: EFORT Open Rev 2019;4:445-459. DOI: 10.1302/2058-5241.4.180081
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Inadvertent excision of lumps which turn out to be sarcomas is not uncommon.
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Imaging has a limited role in detecting microscopic residual disease but can show the extent of the previous surgical field.
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Standard treatment is wide re-excision, usually combined with radiotherapy.
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Residual tumour is found in an average of 50% of reported cases.
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The presence of residual disease is an adverse prognostic factor.
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All lumps bigger than a golf ball should have a diagnosis prior to excision.
Cite this article: EFORT Open Rev 2019;4 DOI: 10.1302/2058-5241.4.180060
