Functional results after oncological scapula resections.

INTRODUCTION: Tumors of the scapula are rare representing only 1.6%-2.8% of all skeletal tumor localizations. Wide resection of these tumors is often necessary, and their anatomic location is the most decisive factor for the extent of the resection. Because of the importance of the shoulder girdle for all daily activities, such resections can have major functional consequences. However, only few reports with small numbers of cases are found in the literature. PATIENTS AND METHODS: We retrospectively evaluated 31 consecutive patients with scapular tumors treated surgically for aggressive benign or malignant tumors of the scapula. Patients who had received curettage only were excluded. Four of these 31 patients were lost to follow-up. In 7 of the remaining 27 patients, a total scapulectomy had to be performed, whereas all others received a partial resection. In 1 case, a partial resection and replantation after irradiation was performed. Musculoskeletal Tumor Society Scoring System (MSTS) and Toronto Extremity Salvage Score (TESS) scores were evaluated postoperatively at the time of their follow-up. RESULTS: In 16 men and 11 women, the median age was 46.2 years and the tumor entities were heterogeneous. The median follow-up time was 71 months. Three patients died during follow-up due to their tumor and 1 due to cardiac disease. We found significantly better functional results in the group with incomplete scapula resections as opposed to the scapulectomy group. We did not see a significant functional difference between the patients with benign and those with malignant lesions. Both the MSTS score with median 83.3% (range between 23% and 100%) and the TESS score with 81.6% (ranging from 20.4% to 100%) were at a satisfactory level. The preservation of the supraspinatus muscle was shown to be advantageous for better shoulder function, and younger patients also tended to have better postoperative results. CONCLUSION: The resection of scapular tumors may lead to a significant functional disability of the shoulder girdle and the affected arm in many cases. However, this is represented neither in the MSTS or TESS score nor in the overall acceptance of the patients. Only 1 patient, an artisan, had to change his job. In total, the clinical results are quite good in short- and long-term follow-up. The score results were comparable to other studies.

[F18] FDG-PET/CT for manual or semiautomated GTV delineation of the primary tumor for radiation therapy planning in patients with esophageal cancer: is it useful?

BACKGROUND: Target volume definition of the primary tumor in esophageal cancer is usually based on computed tomography (CT) supported by endoscopy and/or endoscopic ultrasound and can be difficult given the low soft-tissue contrast of CT resulting in large interobserver variability. We evaluated the value of a dedicated planning [F18] FDG-Positron emission tomography/computer tomography (PET/CT) for harmonization of gross tumor volume (GTV) delineation and the feasibility of semiautomated structures for planning purposes in a large cohort. METHODS: Patients receiving a dedicated planning [F18] FDG-PET/CT (06/2011-03/2016) were included. GTV was delineated on CT and on PET/CT (GTVCT and GTVPET/CT, respectively) by three independent radiation oncologists. Interobserver variability was evaluated by comparison of mean GTV and mean tumor lengths, and via Sørensen-Dice coefficients (DSC) for spatial overlap. Semiautomated volumes were constructed based on PET/CT using fixed standardized uptake values (SUV) thresholds (SUV30, 35, and 40) or background- and metabolically corrected PERCIST-TLG and Schaefer algorithms, and compared to manually delineated volumes. RESULTS: 45 cases were evaluated. Mean GTVCT and GTVPET/CT were 59.2/58.0 ml, 65.4/64.1 ml, and 60.4/59.2 ml for observers A-C. No significant difference between CT- and PET/CT-based delineation was found comparing the mean volumes or lengths. Mean Dice coefficients on CT and PET/CT were 0.79/0.77, 0.81/0.78, and 0.8/0.78 for observer pairs AB, AC, and BC, respectively, with no significant differences. Mean GTV volumes delineated semiautomatically with SUV30/SUV35/SUV40/Schaefer's and PERCIST-TLG threshold were 69.1/23.9/18.8/18.6 and 70.9 ml. The best concordance of a semiautomatically delineated structure with the manually delineated GTVCT/GTVPET/CT was observed for PERCIST-TLG. CONCLUSION: We were not able to show that the integration of PET/CT for GTV delineation of the primary tumor resulted in reduced interobserver variability. The PERCIST-TLG algorithm seemed most promising compared to other thresholds for further evaluation of semiautomated delineation of esophageal cancer.

Stereotactic radiotherapy combined with immunotherapy or targeted therapy for metastatic renal cell carcinoma.

OBJECTIVES: To evaluate the safety and efficacy of stereotactic radiotherapy (SRT) in patients with metastatic renal cell carcinoma (mRCC) concurrently receiving targeted therapy (TT) or immunotherapy. PATIENTS AND METHODS: Data on patients with mRCC were extracted from a retrospective international multicentre register study (TOaSTT), investigating SRT concurrent (≤30 days) with TT/immune checkpoint inhibitor (ICI) therapy. Overall survival (OS), progression-free survival (PFS), local metastasis control (LC) and time to systemic therapy switch were analysed using Kaplan-Meier curves and log-rank testing. Clinical and treatment factors influencing survival were analysed using multivariate Cox regression. Acute and late SRT-induced toxicity were defined according to the Common Terminology Criteria for Adverse Events v.4.03. RESULTS: Fifty-three patients who underwent 128 sessions of SRT were included, of whom 58% presented with oligometastatic disease (OMD). ICIs and TT were received by 32% and 68% of patients, respectively. Twenty patients (37%) paused TT for a median (range) of 14 (2-21) days. ICI therapy was not paused in any patient. A median (range) of 1 (1-5) metastatic tumour was treated per patient, with a median (range) SRT dose of 65 (40-129.4) Gy (biologically effective dose). The OS, LC and PFS rates at 1 year were 71%, 75% and 25%, respectively. The median OS and PFS were not significantly different among patients receiving TT vs those receiving ICIs (P = 0.329). New lesions were treated with a repeat radiotherapy course in 46% of patients. After 1 year, 62% of patients remained on the same systemic therapy as at the time of SRT; this was more frequent for ICI therapy compared to TT (83% vs 36%; P = 0.035). OMD was an independent prognostic factor for OS (P = 0.004, 95% confidence interval [CI] 0.035-0.528) and PFS (P = 0.004; 95% CI 0.165-0.717) in multivariate analysis. Eastern Cooperative Oncology Group performance status (ECOG-PS) was the other independent prognostic factor for OS (P = 0.001, 95% CI 0.001-0.351). Acute grade 3 toxicity was observed in two patients, and late grade 3 toxicity in one patient. No grade 4 or 5 toxicity was observed. CONCLUSION: Combined treatment with TT or immunotherapy and concurrent SRT was safe, without signals of increased severe toxicity. As we observed no signal of excess toxicity, full-dose SRT should be considered to achieve optimal metastasis control in patients receiving TT or immunotherapy. Favourable PFS and OS were observed for patients with oligometastatic RCC with a good ECOG-PS, which should form the basis for prospective testing of this treatment strategy in properly designed clinical trials.

Technique and results after immediate orthotopic replantation of extracorporeally irradiated tumor bone autografts with and without fibular augmentation in extremity tumors.

BACKGROUND: Reconstruction of the skeletal defects resulting from the resection of bone tumors remains a considerable challenge and one of the possibilities is the orthotopic replantation of the irradiated bone autograft. One technical option with this technique is the addition of a vital autologous fibular graft, with or without microvascular anastomosis. The aim of our study was to evaluate the clinical results of the treatment of our patient cohort with a specific view to the role of fibular augmentation. METHODS: Twenty-one patients with 22 reconstructions were included. In all cases, the bone tumor was resected with wide margins and in 21 of them irradiated with 300 Gy. In the first case, thermal sterilization in an autoclave was used. The autograft was orthotopically replanted and stabilized with plates and screws. Fifteen patients underwent an additional fibular augmentation, 8 of which received microvascular anastomoses or, alternatively, a locally pedicled fibular interposition. RESULTS: the most common diagnosis was a Ewing sarcoma (8 cases) and the most common location was the femur (12 cases). The mean follow-up time was 70 months (16-154 months). For our statistical analysis, the one case with autoclave sterilization and 3 patients with tumors in small bones were excluded. During follow-up of 18 cases, 55.6% of patients underwent an average of 1.56 revision surgeries. Complete bony integration of the irradiated autografts was achieved in 88.9% of cases after 13.6 months on average. In those cases with successful reintegration, the autograft was shorter (n.s.). Microvascular anastomosis in vascularized fibular strut grafts did not significantly influence the rate of pseudarthrosis. CONCLUSIONS: the replantation of extracorporeally irradiated bone autografts is an established method for the reconstruction of bone defects after tumor resection. Our rate of complications is comparable to those of other studies and with other methods of bone reconstruction (e.g. prosthesis). In our opinion, this method is especially well suited for younger patients with extraarticular bone tumors that allow for joint preservation. However, these patients should be ready to accept longer treatment periods.

Stereotactic body radiotherapy in patients with hepatocellular carcinoma in a multimodal treatment setting.

PURPOSE: Retrospective evaluation of stereotactic body radiation therapy (SBRT) in patients with hepatocellular carcinoma (HCC). METHODS: We retrospectively analyzed 36 patients (45 lesions) treated between 2011 and 2017. Twenty-seven had previous treatments. Current treatment consisted of SBRT alone (n = 15) or selective transarterial chemoembolization (TACE) followed by SBRT to the same lesions (n = 21). Eight patients received additional local treatments to different lesions. Liver function was predominantly moderately restricted (Child A: 29, Child B: 6, Child C: 1). Treatment planning was based on 4D-computed tomography, dose/fractionation varied depending on location and size, most commonly 3 fractions of 12.5 Gy (65% isodose) and 5 fractions of 8 Gy (80% isodose). RESULTS: Median follow-up was 15 months. Local recurrence was observed in 3 lesions (7%), resulting in 1­and 2­year local control rates of 93%. The only significantly predicting factor was the use of abdominal compression. New hepatic lesions occurred in 19 patients (52%), 1­ and 2­year freedom-from-hepatic-failure (FFHF) was 39% and 32%, respectively. Only the number of treated lesions was predictive for FFHF. Sixteen patients have died, resulting in 1­ and 2­year overall survival (OS) of 64% and 41%, respectively, significantly impacted by the number of treated lesions and Child-Pugh class. Severe acute and late toxicity (≥grade 3) was observed in 3% and 8%, respectively. 6 patients (17%) received liver transplantation (OLT) after SBRT, of whom 5 showed pathological complete remission. CONCLUSION: SBRT (±TACE) in highly pretreated HCC is effective and associated with excellent LC and low toxicity. SBRT may be used as definitive or bridging treatment prior to OLT. Patients with multifocal lesions have significantly decreased 1­ and 2­year FFHF and OS.

[Pre-, Post- and Intraoperative Radiation Therapy in Adult Liposarcomas]. / Indikation zur prä-, post- und intraoperativen Strahlentherapie von Liposarkomen des Erwachsenenalters.

Radiation therapy is an integral part of the treatment of soft tissue sarcomas in adults. The indication for additional radiation therapy has usually been based on known general prognostic factors, such as tumour size, localisation, grading and (anticipated) resection margin. Increasing attention has recently been paid to the role of the histological subtype. Moreover, radiation therapy techniques have distinctly improved, so that in some localisations adequate treatment has become possible for the first time. This review firstly summarises general treatment principles of radiation therapy in soft tissue sarcomas - separately for the extremities and trunk and the retroperitoneum. We discuss the available evidence for the administration of additional radiation per se, the preferred timing (pre- vs. postoperative) and fundamental technical principles, including alternative boosting techniques like intraoperative radiation therapy. Moreover, we provide a detailed discussion of the indication and procedure for additional radiation therapy in liposarcomas. We describe fundamental differences between liposarcomas and other soft tissue sarcomas as well as between the subgroups of liposarcoma (well differentiated, dedifferentiated, myxoid, pleomorphic), and how this affects the indication and procedure for additional radiation therapy, in order to provide a panoramic aid to orientation.

Survival and prognostic factors in conventional G1 chondrosarcoma.

BACKGROUND: Chondrosarcoma is the second most frequent malignant bone tumor. Grade I chondrosarcoma (syn.: atypical cartilaginous tumor) is classified as an intermediately and locally aggressive neoplasm and typically is treated less aggressively (i.e., by intralesional curettage). Does the data regarding local recurrence (LR) and metastatic disease justify this? METHODS: From 1982 to 2014, 37 consecutive patients with G1 chondrosarcoma had been resected or curetted. The margin was defined as R0 (wide resection) or R1 (marginal resection). All patients were followed for evidence of local recurrence or metastatic disease. Overall and recurrence-free survival were calculated, and various potentially prognostic factors were evaluated. RESULTS: In 23 patients (62%), the tumor was widely (R0) resected, whereas in 14 patients, (38%) the resection was marginal (R1). Overall survival was 97% after 5 years, 92% after 10 years, and 67% after 20 years. Five-year local recurrence-free survival was 96%. Ten-year local recurrence-free survival was 83%. Local recurrence-free survival showed a significant correlation to margin status but no correlation to location or age. None of the patients with local recurrence died during the follow-up. One patient had metastatic disease at initial presentation, and a further five patients developed metastatic disease during follow-up. Metastatic disease proofed to be a highly significant factor for survival but was not correlated to local recurrence. CONCLUSIONS: There was no significant correlation between the outcome and the primary tumor location. Marginal resection was a risk factor for LR, but there was no significant difference in the overall survival in patients with or without LR. Metastatic disease (16%) was more common than expected from the literature and a significant predictor for poor overall survival.

Lowering the recurrence rate in pigmented villonodular synovitis: A series of 120 resections.

OBJECTIVES: Tenosynovial giant-cell tumour or pigmented villonodular synovitis is an aggressive synovial proliferative disease, with the knee joint being the most commonly affected joint. The mainstay of therapy is surgical resection. The aim of this study was to evaluate the main patient characteristics, treatment and outcomes in a large single-centre retrospective study, focusing on meticulous aggressive open surgical procedures. METHODS: From 1996 through 2014, 122 surgical interventions were performed in 105 patients. All patients underwent open synovectomy and when the knee joint was affected, combined anterior and posterior synovectomy. Radiotherapy was applied in 2 patients, radiosynoviorthesis in 27 patients. RESULTS: In histopathology, the diffuse type was seen in 66 (54%) lesions. Two patients were lost during follow-up. At a median follow-up time of 71 months (range: 13-238), 22 (18%) lesions recurred within a median of 18 months, >90% in the first 3 years. Out of those 22 recurrences, 9 (11%) were seen in primary disease and 13 (34%) were a second recurrence. After renewed resection, 6 (5%) out of the 120 resections had persistent tumour at the end of follow-up. Based on the number of patients with complete follow-up (n = 103), this represents 5.8%. CONCLUSION: In diffuse-type pigmented villonodular synovitis, total synovectomy might be difficult to achieve. As shown in our results and also in the literature, meticulous open resection, especially in difficult to approach areas such as the popliteal space, reduces local recurrence rates. External beam radiation is an option in prevention of otherwise non-operable local recurrences or in non-operable disease.

External Beam Radiation Therapy for Resectable Soft Tissue Sarcoma: A Systematic Review and Meta-Analysis.

PURPOSE: The aim of this study was to evaluate the role of preoperative and postoperative external beam radiation therapy (EBRT) in the treatment of resectable soft tissue sarcomas (STSs) of different tumor locations. METHODS: A systematic literature search was performed to identify studies investigating the effects of EBRT (versus no EBRT) on local recurrence (LR) and overall survival (OS) or comparing different EBRT sequences. Random effects meta-analyses were calculated and presented as cumulative odds ratios (ORs). RESULTS: Sixteen studies (n = 3958 patients) comparing EBRT versus no EBRT, including one randomized controlled trial (RCT) in extremity sarcoma, were analyzed. EBRT appeared to reduce LR in both retroperitoneal tumors (OR 0.47, p < 0.0001) and other locations (OR 0.49, p = 0.001). OS was improved by EBRT in retroperitoneal STSs (OR 0.37, p < 0.0001) but not in other tumor locations. Eleven studies (n = 2140), including one RCT, compared preoperative and postoperative radiotherapy. LR was less frequent following preoperative EBRT in retroperitoneal STSs (OR 0.03, p = 0.02), as well as in other tumor locations (OR 0.67, p = 0.01), while wound complications in extremity sarcoma were more frequent following preoperative EBRT (OR 2.92, p < 0.0001). Several studies included in this meta-analysis bear a high risk of bias and no RCT has been published for retroperitoneal STS. CONCLUSIONS: This meta-analysis supports the use of EBRT for local tumor control in patients with resectable STSs. Based on a small number of non-randomized studies, a positive effect on OS may exist in the subgroup of retroperitoneal STSs.

Intraoperative Electron Radiation Therapy Combined with External Beam Radiation Therapy after Gross Total Resection in Extremity Soft Tissue Sarcoma: A European Pooled Analysis.

INTRODUCTION: We report a pooled analysis evaluating the combination of gross complete limb-sparing surgery, intraoperative electron radiation therapy (IOERT), and external beam radiation therapy (EBRT) in patients with extremity soft tissue sarcoma (STS). METHODS: Individual data of 259 patients (median follow-up 63 months) with extremity STS from three European expert centers were pooled. Median age was 55 years and median tumor size was 8 cm. Eighty percent of patients presented with primary disease, mainly located in the lower limb (81%). Union for International Cancer Control 7th edition stage at presentation was as follows: stage I: 9%; stage II: 47%; stage III: 39%; stage IV: 5%. Most patients showed high-grade lesions (91%), predominantly liposarcoma (31%). Median IOERT dose was 12 Gy, preceeded (17%) or followed (83%) by EBRT, with a median dose of 45 Gy. RESULTS: Surgery resulted in R0 resections in 71% of patients and R1 resections in 29% of patients. The 5-year local control (LC) rate was 86%, and significant factors in univariate analysis were disease status and resection margin. Only margin remained significant in multivariate analysis. The 5-year distant control rate was 69%, and significant factors in univariate analysis were histology, grading, resection margin, and metastases prior to/at IOERT. Only grading and metastases remained significant in multivariate analysis. Actuarial 5-year rates of freedom from treatment failure and OS were 61% and 78%, respectively. Significant factors for OS were grading and metastases prior to/at IOERT (univariate, multivariate). Limb preservation and good functional outcome were achieved in 95% and 81% of patients. CONCLUSIONS: Our pooled analysis confirmed prior reports of encouraging LC and survival, with excellent rates of preserved limb function with this treatment approach. Resection margin remained the most important factor for LC, while grading and metastases prior to/at IOERT mainly predicted survival.

Medulloblastoma in adults : A retrospective single institution analysis.

PURPOSE: Adult medulloblastoma is a rare disease treated according to the current pediatric treatment guidelines. This retrospective analysis investigated the clinical outcomes and prognostic factors of adult medulloblastoma patients, who received multimodal therapy at our institution. METHODS: Treatment charts of all patients over the age of 15 years of age with de novo medulloblastoma, who had been treated at our institution between 2001 and 2014, were retrospectively analyzed. Patients' demographic parameters, initial symptoms, treatment modalities, toxicities, and survival outcomes were investigated. RESULTS: In all, 21 patients with a median age of 30.2 years were identified. The most frequent histologies were desmoplastic and classic, and the most common molecular subtype was sonic hedgehog (SHH). After tumor resection, all patients received craniospinal irradiation (median dose 35.2 Gy) and a boost to the posterior fossa (median dose 19.8 Gy). Simultaneous chemotherapy with vincristine was given to 20 patients and sequential chemotherapy to 15 patients. The most common side effects were hematological toxicities. Median overall survival (OS) has not been reached after a median follow-up of 92 months. Estimated 5­ and 10-year OS was 89 and 80%, respectively. Estimated 5­ and 10-year progression-free survival (PFS) was 89 and 81%, respectively. In univariate analysis, a shorter interval between tumor resection and end of irradiation was significantly associated with improved OS and PFS, anaplastic histology with worse OS and PFS. CONCLUSIONS: The combined modality treatment showed a good outcome in adults with medulloblastoma. Treatment time was revealed to be prognostic and should be kept as short as possible.

Neoadjuvant chemoradiation for esophageal cancer : Surgery improves locoregional control while response based on FDG-PET/CT predicts survival.

INTRODUCTION: To retrospectively analyze the outcome of patients with esophageal cancer treated with neoadjuvant chemoradiation. METHODS: A total of 41 patients received neoadjuvant intent chemoradiation for esophageal cancer. Most patients had a locally advanced disease (T3/4: 82%, N+: 83%, M0: 100%) and squamous cell carcinoma (83%). All patients received concurrent chemotherapy with cisplatin/5-fluorouracil or mitomycin/5-fluorouracil. Median radiation dose was 50.4 Gy in the 25 patients who proceeded to surgery and 57.4 Gy in 16 patients who did not undergo surgery. FDG-PET/CT was used for treatment planning in 24 patients. A second FDG-PET/CT was available for response evaluation in 18 patients. RESULTS: Median follow-up was 16 months in all patients and 30 months in survivors. Radiotherapy was completed without interruptions >3 days in 90% of patients, and chemotherapy was carried out to >80% in 85% of patients. The 2­year locoregional control rate was 60%, distant control rate 54% and overall survival rate 50%. Hematological toxicity grade 3/4 was observed in 34%/10% of patients and non-hematological toxicity grade 3/4 in 46%/2% of patients. Perioperative 30-day mortality was 4%. Subgroup analyses revealed that surgery significantly improved locoregional control (74% vs. 39%, p = 0.034), but not the 2­year survival rate (54% vs. 43%, p = 0.246). In contrast, response based on FDG-PET/CT prior and after chemoradiation significantly predicted improved overall survival (2-year overall survival 61% vs. 40%, p = 0.048). CONCLUSION: Outcomes of our cohort were comparable to other series using similar treatments. Surgery significantly improved locoregional control but not survival. Response based on FDG-PET/CT predicted survival and might be used for treatment stratification.

Myxoid liposarcoma: local relapse and metastatic pattern in 43 patients.

BACKGROUND: Liposarcomas are the second most common type of soft tissue sarcomas, 30-50% of these are of myxoid subtype. The aim of this retrospective study was to analyze the local control rate, the metastatic pattern and survival of patients in a consecutive single-institution series. METHODS: From 1983 to 2015, 43 patients with myxoid liposarcoma of the extremities and trunk wall underwent resections. The margin was defined as R0 (wide) or R1 (marginal). Patients were followed for evidence of local recurrence or distant metastasis. Overall and recurrence-free survival was calculated. RESULTS: The mean age was 48.6 years. The lower extremity was involved in 40 cases, the mean tumour size was 12 cm. In 31 cases a wide and in 12 cases a marginal resection was performed. Grading was G1 in 14, G2 in 25 and G3 in 4 cases. Nine patient died in follow-up, 4 of them with metastatic disease, all nonpulmonary. 5-year local recurrence (LR) free survival was 82%. 4 (9.3%) patients developed LR (all R1). Overall survival (OS) was 81% after 5 and 72% after 10 years. In multivariate analysis age and Grading proved to be significant on OS. According to univariate analysis, only age over 48 years and distant metastasis had a significant impact on overall survival. CONCLUSIONS: Patients with myxoid liposarcomas have a good prognosis. Myxoid liposarcoma has a distinct pattern of nonpulmonary metastatic disease. Therefore, patients with high-risk extremity myxoid liposarcoma should undergo imaging studies of the chest, abdomen, spine and pelvis as part of their staging and follow-up examinations preferably with whole body MRI, or CT scans and MRI of the spine and pelvic region for detection of suspected metastatic disease.

Survival and prognostic factors in conventional central chondrosarcoma.

BACKGROUND: Chondrosarcoma is the second most frequent primary malignant bone tumor. Treatment is mainly based on surgery. In general, wide resection is advocated at least in G2 and G3 tumors. But which margins should be achieved? Does localization as for example in the pelvis have a higher impact on survival than surgical margins themselves? METHODS: From 1982 to 2014, 87 consecutive patients were treated by resection. The margin was defined as R0 (wide resection), R1 (marginal resection) or, R2 if the tumor was left intentionally. All patients were followed for evidence of local recurrence or distant metastasis. Overall and recurrence-free survival were calculated, significance analysis was performed. RESULTS: In 54 (62%) cases a R0 resection, in 31 (36%) a R1 and in 2 (2%) patients a R2-resection was achieved. Histology proved to be G1 in 37 patients (43%), G2 in 41 (47%) and G3 in 9 cases (10%). 5-year local recurrence-free survival (LRFS) was 75%. Local recurrence-free survival showed a significant association with the margin status and the localization of the tumor with pelvic lesions doing worst. Metastatic disease was initially seen in 4 patients (4.6%), 19 others developed metastatic disease during follow-up. Overall survival of the entire group at 5 and 10 years were 79 and 75%, respectively. The quality of surgical margins and the presence of local recurrence did not influence overall survival in a multivariate analysis. Pelvic lesions had a worse prognosis as did higher grades of the tumor, metastatic disease and age. CONCLUSIONS: The mainstay of therapy in Chondrosarcoma remains surgery. Risk factors as grading, metastatic disease, age and location significantly influence overall survival. Margin status (R0 vs. R1) did influence local recurrence-free survival but not overall survival. Chondrosarcomas of the pelvis have a higher risk of local recurrence and should be treated more aggressively.

The role of surgical margins in atypical Lipomatous Tumours of the extremities.

BACKGROUND: Atypical lipomatous tumours (ALT) are common adipocytic tumours. Due to their large size and deep-seated location, wide resection might result in severe functional deficits. The question which margins should be aimed is hence discussed controversially. METHODS: Forty consecutive patients underwent limb-sparing resections. Margins were defined as R0 (wide resection), R1 (marginal resection) or R2 if tumour was left. All patients were followed for evidence of local recurrence or remote metastases. Overall and recurrence-free survival was calculated. RESULTS: The mean age at the time of surgery was 61.9 years. The mean tumour diameter was 17 cm with no patient having metastatic disease. In 8 cases a wide (R0) resection, in 31 cases a marginal (R1) and in one patient a R2-resection was performed. The median follow-up time was 40 months. Four patients died due to causes that were not tumour-related. 3 (7.5%) patients (all R1) developed local recurrences. Two of our 3 recurrences in this series occurred in 6 already recurring cases. We observed no dedifferentiation of tumours and no metastatic disease. CONCLUSIONS: ALT represents a comparatively common diagnosis in large deep-seated lesions of the extremities, especially in patients over 60 years. Marginal resection shows an acceptable rate of local recurrence. The risk of dedifferentiation as proven also in a metaanalysis of the English literature of the last 30 years is close to 1%, metastatic disease is exceedingly rare.

Desmoid Tumours of the extremity and trunk. A retrospective study of 44 patients.

BACKGROUND: Desmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy. METHODS: From 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/- radiation therapy, NSAIDs or chemotherapy. In 4 cases a conservative approach was chosen. Thirty seven patients had primary, 17 recurrent disease. Endpoint was either local recurrence (LR), progression of residual disease or rare non-metastatic secondary lesions at the same extremity. RESULTS: The mean age was 39,4 years. In 17 cases a R0, in 27 a R1 and in 6 cases a R2 resection was achieved. Four patients were treated conservatively. All together in 21 cases radiotherapy, in 5 NSAIDs, in 3 imatinib and in 2 cases each tamoxifen or chemotherapy had been applied. The median follow-up was 119 months. 5-year recurrence free survival after resection was 78%. 10 (20.4%) patients developed LR between 5 and 42 months after therapy. Recurrent disease was a negative factor on LR. Margins, radiotherapy, sex, or size of the tumour had no significant impact on LR. Patients younger than 40 years had a significant higher risk of LR. CONCLUSIONS: Surgical margins are less important than keeping function. Radiotherapy might be an option in unresectable lesions, the role of adjuvant radiotherapy is controversially discussed.

Motion monitoring during a course of lung radiotherapy with anchored electromagnetic transponders : Quantification of inter- and intrafraction motion and variability of relative transponder positions.

PURPOSE: Anchored electromagnetic transponders for tumor motion monitoring during lung radiotherapy were clinically evaluated. First, intrafractional motion patterns were analyzed as well as their interfractional variations. Second, intra- and interfractional changes of the geometric transponder positions were investigated. MATERIALS AND METHODS: Intrafractional motion data from 7 patients with an upper or middle lobe tumor and three implanted transponders each was used to calculate breathing amplitudes, overall motion amount and motion midlines in three mutual perpendicular directions and three-dimensionally (3D) for 162 fractions. For 6 patients intra- and interfractional variations in transponder distances and in the size of the triangle defined by the transponder locations over the treatment course were determined. RESULTS: Mean 3D values of all fractions were up to 4.0, 4.6 and 3.4 mm per patient for amplitude, overall motion amount and midline deviation, respectively. Intrafractional transponder distances varied with standard deviations up to 3.2 mm, while a maximal triangle shrinkage of 36.5% over 39 days was observed. CONCLUSIONS: Electromagnetic real-time motion monitoring was feasible for all patients. Detected respiratory motion was on average modest in this small cohort without lower lobe tumors, but changes in motion midline were of the same size as the amplitudes and greater midline motion can be observed in some fractions. Intra- and interfractional variations of the geometric transponder positions can be large, so for reliable motion management correlation between transponder and tumor motion needs to be evaluated per patient.

External-beam radiation therapy combined with limb-sparing surgery in elderly patients (>70 years) with primary soft tissue sarcomas of the extremities : A retrospective analysis.

PURPOSE: To report our experience with EBRT combined with limb-sparing surgery in elderly patients (>70 years) with primary extremity soft tissue sarcomas (STS). METHODS: Retrospectively analyzed were 35 patients (m:f 18:17, median 78 years) who all presented in primary situation without nodal/distant metastases (Charlson score 0/1 in 18 patients; ≥2 in 17 patients). Median tumor size was 10 cm, mainly located in lower limb (83%). Stage at presentation (UICC7th) was Ib:3%, 2a:20%, 2b:20%, and 3:57%. Most lesions were high grade (97%), predominantly leiomyosarcoma (26%) and undifferentiated pleomorphic/malignant fibrous histiocytoma (23%). Limb-sparing surgery was preceded (median 50 Gy) or followed (median 66 Gy) by EBRT. RESULTS: Median follow-up was 37 months (range 1-128 months). Margins were free in 26 patients (74%) and microscopically positive in 9 (26%). Actuarial 3­ and 5­year local control rates were 88 and 81% (4 local recurrences). Corresponding rates for distant control, disease-specific survival, and overall survival were 57/52%, 76/60%, and 72/41%. The 30-day mortality was 0%. Severe postoperative complications were scored in 8 patients (23%). Severe acute radiation-related toxicity was observed in 2 patients (6%). Patients with Charlson score ≥2 had a significantly increased risk for severe postoperative complications and acute radiation-related side effects. Severe late toxicities were found in 7 patients (20%), including fractures in 3 (8.6%). Final limb preservation rate was 97%. CONCLUSION: Combination of EBRT and limb-sparing surgery is feasible in elderly patients with acceptable toxicities and encouraging but slightly inferior outcome compared to younger patients. Comorbidity correlated with postoperative complications and acute toxicities. Late fracture risk seems slightly increased.

Isolated pulmonary metastases define a favorable subgroup in metastatic pancreatic cancer.

PURPOSE: Liver metastasis represents the first site of dissemination in >80% of metastatic pancreatic cancer (PC) patients. Pulmonary metastasis as first site of dissemination in PC is a rare event and might define a biologically distinct subgroup in metastatic PC. METHODS: Consecutive PC patients who were diagnosed or treated with isolated pulmonary metastases at our high-volume comprehensive cancer center were included in a prospectively maintained database between 2002 and 2015. Medical records and correlating computed tomography findings (CT) were retrospectively analyzed. RESULTS: A total of 40 PC patients with isolated pulmonary metastases were identified. Pulmonary metastases represented disease recurrence after initial resection of PC in 22 patients and disease progression of locally advanced pancreatic cancer in 5 patients. 14 out of 27 PC patients (56%) had received chemoradiotherapy for localized disease prior to pulmonary metastasis. Data on 1st-line treatment for pulmonary metastases was available for 38 patients: most patients (71%) received a gemcitabine-based chemotherapy regimen, 5 patients (13%) received best supportive care. After a median follow-up of 37.3 months, median survival after diagnosis of pulmonary metastasis was estimated with 25.5 months (95% CI 19.1-31.8); a significantly improved survival after diagnosis of pulmonary metastasis was observed for patients with less than 10 lung metastases (31.3 vs 18.7 months, p = 0.003) and for an unilateral localization of lung involvement (31.3 vs 21.8 months, p = 0.03). CONCLUSIONS: Our results suggest a favorable outcome of PC patients with isolated pulmonary metastases. Further research is warranted to elucidate the specific molecular characteristics of this rare subgroup.