S. Lang

Surgical treatment of the haemophilic pseudotumour: A single centre experience

PurposeHaemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study is to present our own clinical experience and treatment results of the haemophilic pseudotumour.MethodsWe retrospectively reviewed the records of 87 patients with bleeding disorders treated between 1967 and 2011 for musculoskeletal complications of congenital bleeding disorders. We identified six patients with a haemophilic pseudotumour who were treated at our department.ResultsThe mean age at surgery was 45.9 (range, 40–61) years. The iliac bone was affected in three patients (one right, two left), the right tibia (distal diaphysis) in one, the right thigh in two and the right ulna (proximal part) in one patient. One patient had two pseudotumours. The perioperative course was easily controllable with adequate factor VIII substitution. At the latest follow-up after 8.4 (range, 4–24) years, normal healing with no recurrence was observed.ConclusionsThe haemophilic pseudotumour is a rare but severe complication of hereditary bleeding disorders. In the international literature the resection and postoperative course are described as challenging and difficult, requiring detailed preoperative planning. It is advisable to perform such operations in specialised centres with close co-operation between surgeons and haematologists.

Tumour response of osteosarcoma to neoadjuvant chemotherapy evaluated by magnetic resonance imaging as prognostic factor for outcome

PurposeThis study evaluated the feasibility of computed magnetic resonance imaging (MRI) volumetry in conventional osteosarcomas. Secondly, we investigated whether computed volumetry provides new prognostic indicators for histological response of osteosarcomas after neoadjuvant chemotherapy.MethodsIn a retrospective cohort study, data from the Vienna Bone Tumour Registry was used. MR images from 14 patients (male:female = 1.8, mean age 19xa0years) were analysed prior to and after neoadjuvant chemotherapy according to current therapy regimens. Histological response to chemotherapy was graded according to the Salzer-Kuntschik classification. Computed volumetry was performed for the intraosseous part, as well as the soft-tissue component and the tumour as a whole.ResultsIn a setting of appropriate radiological equipment, the method has been considered to be well implementable into clinical routine. The mean tumour volume prior to chemotherapy was 321u2009(±351) ml. In good responders (nu2009=u20096), overall tumour volume decreased by 47xa0% (pu2009=u20090.345), whereas poor responders (nu2009=u20098) showed a 19xa0% decrease (pu2009=u20090.128). Neoadjuvant multidrug therapy remarkably changed the tumour composition. This is seen in a decrease of the mean ratio of soft-tissue to intraosseous tumour volume from 8.67 in poor responders and 1.15 in good responders to 1.26 and 0.45 (pu2009=u20090.065), respectively. Interestingly, the bony compartment of good responders showed a volume increase during neoadjuvant chemotherapy (pu2009=u20090.073). However, we did not find prognostic markers for histological tumour response to pre-operative chemotherapy.ConclusionsSeparated volumetry of tumour segments revealed interesting insights into therapy-induced growth patterns. If verified in a larger study population, these results should be taken into account when planning ablative surgery.

Complete local tumor control after curettage of chondroblastoma–a retrospective analysis

BACKGROUNDnChondroblastoma is an uncommon benign bone tumor with an incidence of 1 to 2% among all primary bone tumors. In the past, treatment for chondroblastoma has been highly variable leading to different rates of recurrences. Therefore we aimed to determine: (1) the rate of recurrence, (2) the complication rate, (3) and functional outcome after intralesional curettage of chondroblastoma.nnnHYPOTHESESnIntralesional curettage with high speed burring and packing can avoid local recurrences.nnnPATIENTS AND METHODSnExperiences of 22 patients with chondroblastoma of the bone were retrospectively reviewed. The patient group consisted of 16 men; 6 women; mean age 24years (range; 12-58years) affecting in 15 the lower- (68%) and in seven the upper extremity (32%).nnnRESULTSnThere was no local recurrence or malignant transformation. All patients underwent intralesional curettage, followed by defect filling presenting in 19 patients (87%) excellent clinical and oncological results (mean MSTS 98.9). Complications were seen in two patients. Pain was the main revealing symptom of the chondroblastoma (n=16, 73%). Mean follow-up of all patients was 114months (range, 25 to 480months).nnnDISCUSSIONnAggressive curettage and packing provided excellent local tumor control and functional results in our patients with chondroblastoma. Malignant transformation is extremely rare, however, present in literature but was not seen in any of our patients.nnnLEVEL OF EVIDENCEnLevel IV, retrospective study.

Increased zinc accumulation in mineralized osteosarcoma tissue measured by confocal synchrotron radiation micro X-ray fluorescence analysis

Abnormal tissue levels of certain trace elements such as zinc (Zn) were reported in various types of cancer. Little is known about the role of Zn in osteosarcoma. Using confocal synchrotron radiation micro X‐ray fluorescence analysis, we characterized the spatial distribution of Zn in high‐grade sclerosing osteosarcoma of nine patients (four women/five men; seven knee/one humerus/one femur) following chemotherapy and wide surgical resection. Levels were compared with adjacent normal tissue. Quantitative backscattered electron imaging as well as histological examinations was also performed. On average, the ratio of medians of Zn count rates (normalized to calcium) in mineralized tumor tissue was about six times higher than in normal tissue. There was no difference in Zn levels between tumor fraction areas with a low fraction and a high fraction of mineralized tissue, which were clearly depicted using quantitative backscattered electron imaging. Moreover, we found no correlation between the Zn values and the type of tumor regression according to the Salzer‐Kuntschik grading. The underlying mechanism of Zn accumulation remains unclear. Given the emerging data on the role of trace elements in other types of cancer, our novel results warrant further studies on the role of trace elements in bone cancer. Copyright

Long-term outcome following treatment of Adamantinoma and Osteofibrous dysplasia of long bones

INTRODUCTIONnAdamantinoma (AD) is an ultimately rare, low-grade malignant bone tumor. In most cases it occurs in the tibia of young adults. Osteofibrous dysplasia (OFD) is a rare, benign, lesion that is typically seen in children. Histopathology, ultrastructure, and cytogenetics indicate that these lesions are closely related. Yet, etiology remains a matter of debate. Local recurrence rates are high for both entities as published in literature and long-term outcomes are scarce, due to the rarity of the disease.nnnHYPOTHESISnAD should be treated by En-Bloc resection while ODF can be treated by curettage or by observation. Consequently, the aim of the present study was to answer following questions: Were local recurrence rates of both entities different based on a retrospective review within a tertiary referral center for orthopedic oncology?nnnMATERIAL AND METHODSnIn a retrospective cohort study, 10 patients with AD and 5 patients with OFD (including 1 patient with OFD-like-AD) were reviewed. Primary surgeries for patients with AD were: En-bloc resection in 7, curettage in 2 and amputation in 1. In the OFD group, only 2 patients underwent surgery by curettage. Mean follow-up was 16 years (range: 2-47 years). Nine patients had a minimum follow-up of 10 years (mean: 23 years; range: 10-47 years).nnnRESULTSnFour patients with AD (40%) and 2 patients with OFD (40%) - all of them following surgical removal - suffered from local recurrence. In the En bloc resection group of AD, there were 2 LR (29%). All patients of both groups treated with curettage showed LR. One patient with AD had metastasis at time of diagnosis and died of disease. Another patient with AD was diagnosed with metastasis 67 months after surgery and was still alive with disease at latest follow-up (77 month).nnnDISCUSSIONnThe overall prognosis of AD and OFD is good, yet local recurrence rates are high, irrespective of surgical strategy. While an internationally standardized treatment regime is still missing, a more radical surgical approach should be considered, especially when treating AD.nnnLEVEL OF EVIDENCEnRetrospective study; Level IV.

High-Grade Osteosarcoma of the Foot: Presentation, Treatment, Prognostic Factors, and Outcome of 23 Cooperative Osteosarcoma Study Group COSS Patients

Osteosarcoma of the foot is a very rare presentation of a rare tumor entity. In a retrospective analysis, we investigated tumor- and treatment-related variables and outcome of patients registered in the Cooperative Osteosarcoma Study Group (COSS) database between January 1980 and April 2016 who suffered from primary high-grade osteosarcoma of the foot. Among the 23 eligible patients, median age was 32 years (range: 6–58 years), 10 were female, and 13 were male. The tarsus was the most commonly affected site (n=16). Three patients had primary metastases. All patients were operated: 5 underwent primary surgery and 18 received surgery following preoperative chemotherapy. In 21 of the 23 patients, complete surgical remission was achieved. In 4 of 17 patients, a poor response to neoadjuvant chemotherapy was observed in the resected primary tumors. Median follow-up was 4.2 years (range: 0.4–18.5). At the last follow-up, 15 of the 23 patients were alive and 8 had died. Five-year overall and event-free survival estimates were 64% (standard error (SE) 12%) and 54% (SE 13%), which is similar to that observed for osteosarcoma in general. Event-free and overall survival correlated with primary metastatic status and completeness of surgery. Our findings show that high-grade osteosarcoma in the foot has a similar outcome as osteosarcoma of other sites.

C-reactive protein: An independent predictor for dedifferentiated chondrosarcoma: CRP PREDICTS THE DEDIFFERENTIATED CHONDROSARCOMA

Dedifferentiated chondrosarcoma is a rare primary bone malignancy with a very poor prognosis. The aim of the study was to identify pretreatment serum markers as prognostic factors for the overall survival (OS) of patients with dedifferentiated chondrosarcoma. We retrospectively reviewed 33 patients with histologically confirmed dedifferentiated chondrosarcoma treated at our department from 1977 to 2015. Kaplan‐Meier estimation, uni‐ and multivariable Cox proportional hazard model were performed to evaluate the association between serum markers such as the C‐reactive protein and OS. In univariable analysis, CRP was strongly associated with OS (HR 1.35; 95%CI 1.13–1.61; pu2009=u20090.001). This association prevailed after adjustment for AJCC tumor stage (HR 1.31; 95%CI 1.02–1.57; pu2009=u20090.031) in multivariable analysis. In conclusion, our data gave evidence that baseline CRP is an independent predictor for OS in patients with dedifferentiated chondrosarcoma. CRP could be exploited for the clinical prediction of this disease in the future.