Sarcomas generally metastasize to the lung, while extra-pulmonary metastases are rare. However, they may occur more frequently in certain histological sub-types. Bone metastases from bone and soft tissue sarcomas account for a significant number of extra-pulmonary disease. Resection of lung metastases is widely accepted as therapeutic option to improve the survival of oligometastatic patients but there is currently no literature supporting curative surgical management of sarcoma bone metastases. Most are treated on a case-by-case basis, following multidisciplinary tumour boards recommendations. One study reported some success in controlling bone metastases using radiofrequency ablation. Our goal was to assess the impact of curative resection of bone metastases from soft tissue and bone sarcomas on oncologic outcomes. Extensive review of literature was done to evaluate epidemiological and outcomes of bone metastases in sarcoma. We examined our prospective database for all cases of bone metastases from sarcoma treated with surgical resection between 1990 and 2016. Epidemiology, pathology, metastatic status upon diagnosis, type of secondary relapses and their treatments were recorded. Overall survival and disease-free survival were calculated and compared to literature. Thirty-five patients were included (18 men, 17 women) with a mean age of 46 years. Fifteen were soft tissue (STS) and 20 were bone (BS) sarcomas. Most STS were fibrosarcomas, leiomyosarcomas or UPS while chondrosarcomas and osteosarcomas were the most frequent BS. Nine (60%) STS were grade 3, 4 (27%) grade 2 and one grade 1 (3%). Eight (23%) were metastatic upon diagnosis (6 lungs, 3 bone). Treatment of the primary tumour included wide excision with reconstruction and (neo)-adjuvant therapies as required. Margins were negative in 32 cases and micro-positive in 3 cases. Amputation occurred in 6 (17%) cases. Primary lung metastases were treated by thoracotomy and primary bone metastases by wide excision. First relapse occurred in bone in 19 cases (54%), lungs and bone in 7 cases, 5 in lungs and 4 in soft-tissues. Lung metastases were treated by thoracotomy and chemotherapy in 3 cases, chemotherapy alone in the remaining cases. Bone metastases were treated by wide resection-reconstruction in 24 cases, extensive curettage in 4. Soft tissue relapses were re-excised in 4 patients. Two amputations were required. All margins were negative except for the 4 treated by curettage. Fourteen second relapses occurred in bone, 7 were radically-excised and 2 curetted. At last follow-up, 6 patients were alive (overall survival of 17%), with a mean survival of 57 months, a median overall survival of 42.5 months and a median disease-free survival (DFS) of 17 months. Overall survival was 17%, compared to an 11% 10-year survival previously reported in metastatic sarcomas. Median disease-free survival was better in this study, compared to 10 months in literature, so as median OS (42.5 months vs 15). Three patients were alive with no evidence of disease. DFS, OS and median survival seemed to be improved by bone metastases wide excision and even if several recurrences occur, curative surgery with adjuvant therapies should be considered

Background. Myxofibrosarcomas are malignant soft tissue tumours that often present as painless slowly growing masses in the extremities of older males. Locally infiltrative growth means risks of local recurrence is high. Management emphasises negative surgical margins and adjuvant therapy. The aim of this retrospective case series was to review our experience of this tumour, and make recommendations about a minimum resection margin and how best to utilise the expertise of the multidisciplinary team. Methods. A computerised database identified patients with myxofibrosarcoma surgically treated in our centre between 1997 and 2011. Clinical records were reviewed. Margins were positive if tumour was at or within 1mm of the resection plane. Results. 43 patients (median age 68.6 years; 70% male) were identified. 42 underwent surgery: 26 (62%) by orthopaedics; 9 (21%) by plastics, and 7 (17%) jointly. The lower limb was the most common site (30/43, 70%). Mean tumour size was 5.9cm (range 1.5 to 20cm). 53% had grade III tumours. Of 39 tumours with available data, 21 were superficial fascially-based masses and 18 were deep. 23 (55%) had positive margins. Of these, 9 underwent re-excision, 5 of whom had adjuvant radiotherapy. 13 of the remaining 14 patients had adjuvant radiotherapy. Of the fascially-based tumours, there was microscopic spread beyond the macroscopic mass of between 3–25mm. 3 patients (7%) developed local recurrence at a mean of 25 months (14 to 30 months). 12 (29%) had metastases at a mean of 19 months (range 7 to 48 months). Conclusion. Myxofibrosarcoma poses a number of challenges for sarcoma teams; in particular, high positive margin rates, risks of further surgery and local failure. The infiltrative nature makes it suited to management by multidisciplinary teams. Microscopic tumour can present up to 2.5cm from the macroscopic mass for fascially-based tumours, and teams should plan appropriately

Purpose. Evaluate the demographics, stages and outcomes in Myxoid (ML) and Round Cell liposarcoma (RCL). Establish the incidence of local recurrence and metastases. Outline the use and benefits of radiotherapy and chemotherapy. Provide guidelines for future management of these rare tumors. Method. Multicentric retrospective study of 421 cases of MRCLS primarily managed by multidisciplinary sarcoma teams in Canada. Data were collected in each centers through a standardized database and statistically analysed. Results. There were 247 males (59%) Age ranged from 14 to 88 years old (avg: 46 yrs) and the average follow-up was 5.9 yrs (range: 1 mo–21.3 yrs). Tumor volume averaged 745 cc (range: 1.5–14580 cc). The proximal lower limb, including the thigh, the buttock and the inguinal region, was the location in 314 cases (75%). Tumors were deep in 81%. On histology 305 patients were classified as pure myxoid liposarcoma, 87 had mixed myxoid/round cell histology (≥ 5% round cell content)and 19 were pure round cells only. AJCC staging were Ia: 44, Ib: 114, IIa: 115, IIb: 57, IIc: 2, III: 56, IV: 9, unknown: 24. Radiotherapy was given to 310 pts and chemotherapy to 26 pts as part of initial management. 419 underwent surgery (407 limb salvage and 12 amputations) Margins were R0 in 309, R1 in 94 and R2 in 15 patients. Overall 10 yrs local control rate was 92% and no differences were recorded between ML and RCL. Radiotherapy was significant in preventing local relapse (p= 0.03) but did not impact survival. Metastatic disease was recorded in 82 patients (19%). Chemotherapy did not prevent metastatic occurrence and survival was statistically worse for the chemotherapy group (p= 0.01). Location of first metastasis was often multiple (29), followed by lung or soft tissue (14 each), retroperitoneum (12) and bone (10). Thirty-four patients had bone involvement with spine involved in 27. The 5 and 10 yrs metastatic free survival were respectively 84 and 73% for ML and 73 and 49% for RCL (p= 0.003). Latest disease status were: 323 alive with no evidence of disease, 27 alive with disease, 8 alive with unknown status, 52 deadfrom tumor and 11 dead from other causes. Conclusion. Myxoid and Round cell Liposarcoma present different prognoses. Metastatic disease at the time of diagnosis is an unusual event. Local control is good but radiotherapy decreased the incidence of local relapse. The effectiveness of chemotherapy remains to be established for round cell liposarcoma. New staging stategies need to be identified to account for the unusual metastatic pattern of these sarcoma