Care For Soft Tissue Sarcomas Research Articles

Greater travel distance to specialized facilities is associated with higher survival for patients with soft-tissue sarcoma: US nationwide patterns.

PurposeThe survival impact of geographic access to specialized care remains unknown in patients with soft-tissue sarcomas (STS). This study aimed to clarify the association between the patient travel distance and survival outcome and investigate the factors lying behind it.MethodsA total of 34 528 patients with STS registered in the National Cancer Data Base, diagnosed from 2004–2016, were investigated.ResultsTumor stage correlated with travel distance: patients with metastatic disease stayed closer to home. However, the type of facility showed greatest variation: 37.0%, 51.0%, 73.5%, and 75.9% of patients with ≤10 miles, 10.1–50 miles, 50.1–100 miles, and >100 miles, respectively (P<0.001), had a sarcoma care at academic/research centers. On a multivariable analysis, reduced mortality risk was associated with longer (versus short) travel distance (>100 miles: HR = 0.877; P = 0.001) and management at academic/research (versus non-academic/research) centers (HR = 0.857; P<0.001). The greatest divergence was seen in patients traveling very long distance (>100 miles) to an academic/research center, with a 26.9% survival benefit (HR = 0.731; P<0.001), compared with those traveling short distance (≤10 miles; 95.4% living in metropolitan area) to a non-academic/research center. There was no significant correlation between travel distance and survival in patients who had care at academic/research centers, whereas a survival benefit of management at academic/research centers was observed in every group of travel distance, regardless of tumor stage.ConclusionsThis national study demonstrated that increased travel distance was associated with superior survival, attributable to a higher proportion of patients receiving sarcoma care at distant academic/research centers. These data support centralized care for STS. Overcoming referral and travel barriers may enable more patients to be treated at specialized centers and may further improve survival rates for patients with STS, even when it imposes an increased travel burden.

Radiotherapy for Adult Soft Tissue Sarcomas of the Head and Neck.

Surgery followed by postoperative radiation therapy (RT) is the standard of care for soft tissue sarcomas (STS) of the head and neck that are high grade or have close or positive margins. The authors retrospectively reviewed adult patients with head and neck STS treated with RT at a single institution between 1981 and 2017. All patients who were 19 years and older with STS of the head and neck-excluding rhabdomyosarcoma, angiosarcoma, and Ewing tumors-were included in this study. Toxicity was graded using Common Terminology Criteria for Adverse Events (CTCAE), version 4. Among 34 patients with head and neck STS treated with postoperative RT (33) or primary RT (1) who met the inclusion criteria, the median age at diagnosis was 45 years (range, 20 to 83). Overall, 37% had T1 tumors, 50% had high-grade histology (grade 3), and 26% had microscopically positive margins. The median RT dose was 65 Gy to the primary site; 29% received elective nodal irradiation. The median follow-up for living patients was 16.6 years (range, 0.6 to 30). At 5 and 10 years, the local control rates were 88% and 80%, the regional control rates were 97% and 97%, the freedom from distant metastases rates were 100% and 100%, the cause-specific survival rates were 88% and 81%, and the overall survival rates were 85% and 69%. Two patients (6%) developed late grade 3+ complications. Our study demonstrates that surgery and radiotherapy for STS of the head and neck have excellent disease outcomes.

Update on Systemic Therapy for Advanced Soft Tissue Sarcoma

Background: Soft tissue sarcoma (STS) is a rare group of mesenchymal neoplasms which contains over 50 heterogenous subtypes. There have been great efforts to increase the understanding of treatment of advanced STS (unresectable or metastatic disease). We wished to determine if outcomes for patients with advanced STS have improved over time, and to assess the current evidence for systemic therapy. Methods: We performed a scoping review to evaluate the contemporary evidence for systemic treatment of advanced soft tissue in adults (&gt;18 years old). Phase I, II, and III studies of systemic therapy for advanced STS published in the English language were included. After abstract and full text review of 77 studies, 62 trials met inclusion criteria. Results: The number of clinical trials conducted and published in advanced STS has increased over the last 30 years. Although median OS has increased, attempts at improving first line therapy through dose intensification, doublet chemotherapy or alternative backbones have not been successful. The optimal therapy beyond anthracyclines remains a challenge, especially given the inherent heterogeneity of grouping multiple STS subtypes within clinical trials. However, increasing numbers of agents are being studied and several studies had shown isolated PFS or OS benefit. Conclusions: First line anthracycline systemic therapy remains the standard of care for advanced STS. However, choice of subsequent therapy beyond anthracyclines remains challenging. Novel systemic therapies, using molecular diagnostics to direct therapy, subtype specific trials and learning from real world retrospective data are all important in improving outcomes for patients with advanced STS.

Prise en charge des tumeurs des parties molles au membre supérieur — à propos d'un cas et revue de la littérature

Soft tissue sarcomas are rare in the hand and wrist. The goals of treatment primary to get an oncological cure, and secondary the maintenance of maximum function and appearance. Traditional treatment has focused on amputation as it provides adequate local control, however it sacrifices function. Recently, limb salvage has become the standard of care for soft tissue sarcomas of the extremities. We present the case of a patient with a leiomyosarcoma of the wrist with a literature review of the current treatment of soft tissue sarcomas of the upper extremity. A 40-year-old woman presented with a giant mass of a one-year history of growing on the right wrist and forearm. The patient presented a loss of extension of all the hand digits, pain but with no neurovascular related symptoms. On MRI, a mass with intermediate signal intensity on T1 and T2 weighted images was seen, involvment of the posterior tendons and osteolysis of the distal part of the forearm bones. A metastatic work-up was negative. A biopsy with histological examination showed a leiomyosarcoma. A neoadjuvant chemotherapy was started but with no response and a permanent growing tumor volum. An amputation above the elbow was performed. All margins were free of tumor. The patient did well after surgery and after 4 years is disease free. Although limb salvage with chemotherapy and radiation is now considered the standard of care, there are still some instances where amputation is considered as the best method for cure — Limb larger and more invasive tumors which was the case of our patient, the presence of neurovascular structures involvement, anticipated poor functional outcomes, and patient preferences. Some cases would result in improved functional outcomes with amputation and prosthesis or a simple amputation without efforts at complex reconstruction may provide the least morbidity and the fastest return to desired functional levels. In terms of oncologic cure, the rate of local recurrence was found higher in patients who had limb salvage over amputation, however survival was equivalent. These developments resulted in increased use of limb salvage with increasing reliance on adjuvant therapy. Overall, limb salvage often provides good functional outcome. Local control with limited surgery is dependent on surgical resection. Amputation has been superseded by limb salvage in most cases, although it can occasionally be the only option. A variety of adjuvant therapies have been described, including radiation or brachytherapy, chemotherapy, and regional hyperthermia.

Soft tissue sarcoma in Asia.

Sarcoma is an uncommon and heterogeneous group of malignancies linked by their mesenchymal origin. They are rare and account for 1% of adult cancers, and 10-20% of adolescent and young adult (AYA) cancers. While there is good published literature on the incidence and distribution of sarcoma subtypes in the western populations, there is a paucity of data from Asia, particularly on the epidemiology, treatment and outcomes of STS in Asia. Formalized prospective national registries in soft tissue sarcoma (STS) are lacking and little is known about how sarcomas are treated and managed throughout the Asia-Pacific region. Due to geographical and varying affluence levels across over 50 countries, no single uniform guideline exists across Asia to inform of chemotherapeutic options. Any existent guidelines tend to be country-specific, reflecting resource availability and geographical limitation. To understand and improve STS care in Asia, one must appreciate the macroeconomics and healthcare structures in place and to work within the limitations imposed by them. Concurrently, there exists an urgency to develop strong sarcoma centres of excellence (SCE) across Asia to deliver state-of-art care to our patients. Equally important is the need to facilitate sarcoma education to patients and healthcare workers. Development of such centres is vital in improving clinical care as they provide high-quality sub-specialized care to patients within that geographical location, serve as reference centres for knowledge and education as well as nerve centres for care-coordination in a hub-and-spoke model. Key to these centres of excellence is the sarcoma multi-disciplinary team (MDT). Studies have consistently shown that patients managed in high-volume sarcoma centres, by an expert sarcoma MDT, achieve better clinical outcomes. This report serves to highlight the challenges and opportunities of sarcoma care in Asia, map out a vision for the development of SCE across Asia and highlight the areas of potential collaboration between centres to advance the science of sarcoma.

Pathologic femur fractures following limb-salvage surgery and radiotherapy for soft tissue sarcomas: They don't heal!

Introduction Combined limb-sparing surgery and radiation therapy are considered the standard of care for soft tissue sarcomas (STS) of the extremities. The correlation between radiation therapy and the risk of post radiation fracture is known but underestimated and can end up in serious long-term complications. Case description We reviewed the records of 3 patients with pathological femur fracture years after wide local excision of a STS of the proximal lower extremity with postoperative radiation therapy. All patients received more than 50 Gray to the entire femur circumference. No one received perioperative chemotherapy. During surgery, all patients had bone exposure, whereas only one patient had the periosteum stripped. Results and Conclusions Two patients were female and one male. The median time from surgery/radiation to fracture was 116 months (range, 84 to 156 months). The median age at the time of diagnosis was 66 years (range, 54 to 79 years). All fractures occurred within the radiation treatment field. Two fractures occurred after minimal or no trauma, one fracture occurred after a mountain bike fall. All three fractures 3/3 (100%) developed a non-union. One patient died due to uncontrolled pulmonary metastasis and local recurrent disease. In the second case we had to perform an exarticulation at hip level due to an uncontrolled infected non-union with soft tissue defect despite several surgical revisions. The third patient is still under treatment of his non-union. Take home message Local control rates after combined therapy for the treatment of soft-tissue sarcomas are high. However, pathologic fractures after radiation therapy pose an extreme challenge in their treatment and may be associated with long-term complications that can cause physical disability and impairment of the quality of life.

Myxofibrosarcoma primary cultures: molecular and pharmacological profile.

Background:Myxofibrosarcoma (MFS), formerly considered as a myxoid variant of malignant fibrous histiocytoma, is the most common sarcoma of the extremities in adults and is characterized by a high frequency of local recurrence. The clinical behavior of MFS is unpredictable and the efficacy of chemotherapy is still not well documented. Furthermore, given the relatively recent recognition of MFS as a distinct pathologic entity its cellular and molecular biology has still not been extensively studied in patient-derived preclinical models. We examined the molecular biology and treatment outcomes of high-grade, patient-derived MFS primary cultures.Methods:A total of three patient-derived MFS primary cultures were analyzed. We evaluated the role of CD109 expression and also looked for a correlation between transforming growth factor-beta (TGF-β) expression and sensitivity of the primary cultures to different drugs.Results:CD109 was a promising marker for the identification of more aggressive high-grade MFS and a potential therapeutic target. The results also highlighted the potential role of TGF-β in chemoresistance. Pharmacological analysis confirmed the sensitivity of the cultures to chemotherapy. The most active treatments were epirubicin alone and epirubicin in combination with ifosfamide, the latter representing the current standard of care for soft tissue sarcomas (STSs), including MFS.Conclusions:Our results provide a starting point for further research aimed at improving the management of MFS patients undergoing chemotherapy.

Adherence to Guidelines for Adult (Non-GIST) Soft Tissue Sarcoma in the Netherlands: A Plea for Dedicated Sarcoma Centers

IntroductionOptimal management of soft tissue sarcoma (STS) remains a challenge. A nationwide survey assessed the quality of STS care in the Netherlands, thereby aiming to identify potentialities for improvement through more centralized disease management.MethodsFrom the Netherlands Cancer Registry (NCR), data were obtained on 3317 adult STS patients (excluding gastrointestinal stromal tumor, GIST) diagnosed in 2006–2011. Logistic regression models were employed to compare outcomes on selected clinical indicators reflecting prevailing STS guidelines between high-volume (≥10 resections annually) and low-volume (<10 resections) hospitals, between academic and general hospitals, and between sarcoma research centers and other hospitals, adjusted for case mix. Analyses were performed on imputed datasets (m = 50), generated through multiple imputations by chained equations.ResultsOverall, 89% of patients underwent surgical resection. Resection status remained unknown in 24% (excluding those with metastasized disease), and grade was not documented for one-third of tumors. Microscopic residual disease was detected in 20% with an increased risk for older patients, larger and deeply located tumors, and those located in the (retro)peritoneum or upper extremity. Almost half of patients with an R1 resection received adjuvant radiotherapy. Following adjustment for case mix factors, patients treated in high-volume hospitals less often had macroscopic residual disease (R2 resection; adjusted odds ratio: 0.54). A strongly skewed distribution of surgical volumes was observed.ConclusionsThese survey results indicate a potential for improving Dutch STS care. More centralized sarcoma management should improve definitive pathology reporting on tumor characteristics, adherence to treatment guidelines and overall disease outcome.

EL2 - Standards of care for Soft Tissue Sarcoma

The mainstay of care for unresectable, metastatic, and relapsed sarcoma is chemotherapy, and the principle treatment aims are disease palliation and control. Historically, all soft tissue sarcoma (STS) subtypes have been treated similarly, because of the lack of information about the outcome differences for conventional chemotherapy. The treatment strategies for sarcomas need to be adapted considering the histological subtype and tumor characteristics. However the rarity of STS makes it difficult to develop personalized treatment for advanced STS based on the phenotype or genotype.Doxorubicin is the most frequently used agent in first-line treatment of metastatic STS, and results in response rates of 10–20%. Multiple phase III clinical trials of doxorubicin-containing chemotherapy have been conducted in study groups in the US and Europe, but no treatment has shown superior activity considering the survival benefit compared to doxorubicin monotherapy. The combination of doxorubicin and ifosfamide is used as one of the options for situations in which tumor shrinkage is required, such as symptom palliation or when a critical structure is threatened.Currently, the clinical development of targeted therapy for STS has been initiated. For STSs, the multi-targeted TKI pazopanib, which has activity against VEGFR 1–3, PDGFRA and PDGFRB, and KIT, was approved in Japan 3 years ago. Potential drug targets in STS have been reported, such as the ALK fusion gene in inflammatory myofibroblastic tumors, activation of PDGFRB in dermatofibrosarcoma protuberans, and amplification of MDM2 and CDK4 in well-differentiated and de-differentiated liposarcoma. Other targets have also been reported, and the clinical utility of targeted therapy for specific histological subtypes harboring such target genes has been indicated in some cases. Thus, histology- or target-based therapies for advanced STS are expected to be introduced in clinical practice in the future. The mainstay of care for unresectable, metastatic, and relapsed sarcoma is chemotherapy, and the principle treatment aims are disease palliation and control. Historically, all soft tissue sarcoma (STS) subtypes have been treated similarly, because of the lack of information about the outcome differences for conventional chemotherapy. The treatment strategies for sarcomas need to be adapted considering the histological subtype and tumor characteristics. However the rarity of STS makes it difficult to develop personalized treatment for advanced STS based on the phenotype or genotype. Doxorubicin is the most frequently used agent in first-line treatment of metastatic STS, and results in response rates of 10–20%. Multiple phase III clinical trials of doxorubicin-containing chemotherapy have been conducted in study groups in the US and Europe, but no treatment has shown superior activity considering the survival benefit compared to doxorubicin monotherapy. The combination of doxorubicin and ifosfamide is used as one of the options for situations in which tumor shrinkage is required, such as symptom palliation or when a critical structure is threatened. Currently, the clinical development of targeted therapy for STS has been initiated. For STSs, the multi-targeted TKI pazopanib, which has activity against VEGFR 1–3, PDGFRA and PDGFRB, and KIT, was approved in Japan 3 years ago. Potential drug targets in STS have been reported, such as the ALK fusion gene in inflammatory myofibroblastic tumors, activation of PDGFRB in dermatofibrosarcoma protuberans, and amplification of MDM2 and CDK4 in well-differentiated and de-differentiated liposarcoma. Other targets have also been reported, and the clinical utility of targeted therapy for specific histological subtypes harboring such target genes has been indicated in some cases. Thus, histology- or target-based therapies for advanced STS are expected to be introduced in clinical practice in the future.

The challenge of access to care for soft tissue sarcomas bridging pediatric and adult age: the Italian pediatric oncology view

Synovial sarcoma and rhabdomyosarcoma are two high-grade soft tissue sarcoma subtypes that occur in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, partly because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition. In this review, the doubts concerning how best to manage soft tissue sarcomas in patients between pediatric and adult ages lead up to a more general discussion of the issue of access to optimal cancer services for adolescents and young adults – a subset of patients acknowledged as being under-represented in clinical trials on therapies that may improve their outcome. The situation in Italy is described, along with action taken in an effort to bridge the gap and implement specific programs tailored to these patients.

Influence of Specialty and Clinical Experience on Treatment Sequencing in the Multimodal Management of Soft Tissue Extremity Sarcoma

Although multimodal management of extremity soft tissue sarcoma (STS) is the standard of care, considerable variation exists in the sequencing of radiotherapy (RT) or chemotherapy (CT). Our goal was to identify factors responsible for this variation. Members of specialty societies with an interest in STS were emailed a questionnaire about multimodal treatment of STS. Survey responses were scored on a 5-point Likert scale (1 = always preoperative and 5 = always postoperative) and analyzed by specialty, years in practice, and percentage of practice consisting of STS. The questionnaire was completed by 320 (65%) of 490 physicians, including medical oncologists (18%), radiation oncologists (8%), orthopedic oncologists (22%), surgical oncologists (45%), and others (7%). Respondents were evenly split on the use of neoadjuvant RT (mean 3.03 ± 0.06) and showed a slight preference for neoadjuvant CT (2.89 ± 0.06). Radiation oncologists (2.52 ± 0.18), physicians with a >75% STS practice (2.58 ± 0.17), and those in practice <5 years (2.79 ± 0.12) preferred neoadjuvant RT. Neoadjuvant CT was preferred by orthopedic oncologists (2.62 ± 0.12) and physicians with >75% STS practice (2.51 ± 0.16). Factors influencing the choice for neoadjuvant RT were well-defined treatment volume, increased acute morbidity, and decreased late morbidity, while for CT, they were in-situ disease monitoring and early treatment of micrometastases. Treatment sequencing in STS is influenced by specialty and clinical experience, with no clear consensus. These patterns may reflect the recent trend toward regionalization of STS care.

A phase II study of a new formulation of nonpegylated liposomal doxorubicin (doxorubicin GP-pharm) as first-line treatment in patients with advanced soft-tissue sarcomas (STS) who are age 65 or older: A GEIS trial.

10072 Background: Doxorubicin remains the standard of care for STS. Its use has been limited due to bone marrow and cardiac toxicity, specially in elderly patients (pts). Doxorubicin GP-Pharm (Sarcodoxome)(DX-GP) is a non-pegylated liposomal formulation containing lipochromane-6. Previous Phase I study suggested activity and a low cardiac toxicity (CTOS 2008) Methods: Multicenter multinational Phase II prospective trial in advanced or metastatic STS pts aged ≥64 yr, to asses efficacy and safety of DX-GP in first line setting. Eligibility: ECOG ≤ 2, measurable disease by RECIST and acceptable hematologic, liver, renal and cardiac function. DX-GP 80 mg/m2 was administered IV over 60 minutes on day 1 every 3 weeks for a maximum of 6 cycles. Troponin, BNP, ECG and MUGA were performed every 2 cycles to evaluate cardiac function. An amendment (2009) expanded inclusion criteria to pts over than 45 years (thus only 4 pts <65 years old have been recruited). Results: From February 2007 to January 2010, 37 pts (13 male and 24 female) were included (12 retroperitoneum/trunk, 8 extremities, 4 uterine, 4 visceral). Median age 74 years (51-87). Non-resectable locally advanced 9, metastatic 28 pts. Nr of cycles: 116, mean 3(1-6). Grade 3/4 toxicities: Neutropenia 23 (62%), Leukopenia 10 (27%), febrile neutropenia 6 (16%), thrombopenia 4 (10%), anemia 2(5%) pts. Non-hematological toxicities were grade 3 asthenia 4 (10%) pts and grade II stomatitis and alopecia among others. 2 pts presented cardiac toxicity grade 1 and grade 5 respectively. Non cumulative cardiac toxicity. One treatment-related death. Efficacy (PR or SD) was seen in 8/26 (30%) evaluable pts. Median PFS was 3 (CI95% 1.9-7.4) months. Median survival time 14.4 (CI95% 6.3-27.1) months. Conclusions: DX-GP is a new liposomal DX that shows acceptable toxicity profile and efficacy at 80 mg/m2 in elderly pts with STS. Studies are needed in younger pts.

Impact of subspecialty, clinical volume, and years of practice in selection of treatment sequencing in the management of localized extremity soft tissue sarcoma.

10085 Background: Although the treatment of localized extremity soft tissue sarcoma (STS) is multimodality in nature, considerable variation exists in the use and timing of radiation (RT) and chemotherapy in relation to surgical resection. Our objective was to identify factors responsible for this variation. Methods: A questionnaire was emailed to active members of ASCO who specialize in the management of STS. Questions pertained to the multimodality management of STS. Survey responses were scored on a five-point Likert scale. Means were compared using independent samples ANOVA with post-hoc Bonferroni correction as indicated. Results: The questionnaire was viewed by 490 potential respondents and completed by 320 (65%). Respondents self-identified by specialty: Medical Oncology (18%), Radiation Oncology (8%), Surgeons (67%), and Other (7%). Sarcoma volume in relation to overall clinical practice comprised: >75% (12%), 25-75% (39%), and <25% (49%). Respondents were evenly distributed on the use of preoperative versus postoperative radiation (mean 3.03 ± 0.06), a decision not influenced by specialty. However, physicians with a >75% STS practice preferred preoperative RT compared to those with <25% (2.58 ± 0.17 vs. 3.19 ± 0.08, p 0.003). Similarly, physicians in practice <5 years preferred preoperative RT compared to those in practice >15 years (2.79 ± 0.12 vs. 3.21 ± 0.09, p 0.025). Factors identified as being important in receipt of RT included tumor size >10cm, high grade, positive margins and proximity to neurovascular bundle. Overall, respondents preferred preoperative chemotherapy (mean 2.89 ± 0.06). No significant difference existed by specialty or years in practice. Physicians with >75% STS practice preferred preoperative chemotherapy compared to those with <25% (2.51 ± 0.16 vs. 3.22 ± 0.07, p <0.001). Factors important for chemotherapy were tumor size >10cm, high grade, age <50, and synovial sarcoma histology. Conclusions: Treatment sequencing in the management of STS is influenced by clinical volume and years in practice but not by physician specialty. This may reflect the recent trend towards regionalization of multimodality STS care. No significant financial relationships to disclose.

136. Extremity Soft Tissue Sarcoma Care in the Elderly: A Population-Based Study

136. Extremity Soft Tissue Sarcoma Care in the Elderly: A Population-Based Study

Rehabilitation and quality-of-life issues in patients with extremity soft tissue sarcoma

The current standard of care for soft tissue sarcoma (STS) is limb salvage surgery and adjuvant radiotherapy, with long-term survival rates of approximately 70%. However, the extensive surgical resection and subsequent reconstruction result in 50% of survivors living with chronic disability. Rehabilitation aims to optimize functional independence and quality of life, and is routinely offered to patients undergoing surgical treatment for STS. Unfortunately, there is a dearth of research related to rehabilitation in this area. We propose a model for assessing disability, for designing treatment interventions and for evaluating rehabilitative outcomes in STS. The World Health Organization's (WHO) international classification of functioning, disability, and health (ICF) is divided into three domains: 1) impairments (related to body structure and function), 2) activity limitations (related to usual self-care activities/activities of daily living), and 3) participation restrictions (related to social roles). A literature review of STS rehabilitation reveals that most studies have focused on disability assessment, with few papers describing or evaluating rehabilitation interventions commonly employed in STS. Clinicians are forced to extrapolate findings from other patient populations in order to evaluate the effectiveness of specific rehabilitation strategies (ie, those used for particular sequelae of STS, such as lymphedema or impaired exercise tolerance). There is strongest support for complex decongestive physiotherapy (targeting lymphedema) and aerobic exercise interventions (aimed at alleviating cancer-related fatigue and psychosocial sequelae). The most poorly researched topic is rehabilitation for genitourinary disability (both incontinence and sexual dysfunction). Most studies related to oncologic rehabilitation are restricted to the impairment level (eg, affecting range of motion, muscle strength) of the ICF, with only a small minority addressing activity limitations (eg, affecting activities of daily living) experienced by patients. A consideration of participation restrictions (eg, fulfillment of vocational roles) is almost wholly absent from the literature. Yet social role reintegration is of fundamental importance to patients. Further research is required in these two domains. The ICF provides a comprehensive framework for future research into rehabilitation interventions for STS.

Processes and Outcomes of Care for Soft Tissue Sarcoma of theExtremities

Purpose: A population-based cohort study of soft tissue sarcoma of the extremities (STSE) in Ontario, Canada was conducted using linked administrative databases. Methods and materials: Electronic administrative databases were linked from the Ontario Cancer Registry, the Canadian Institute for Health Information, and Radiation Oncology Research Unit database of radiation therapy (RT) records. Results: The definitive surgery was amputation for 6.0%, resection for 60.9%, biopsy for 7.5%; the remainder had no surgical record. Adjuvant RT was administered to 40.2% of cases. Among cases initially treated by surgical resection, 2.0% later underwent amputation and 9.5% underwent further resection during follow-up. The adjusted odds ratio (OR) for amputation as definitive surgery was 2.3 (1.19, 4.45) in eastern Ontario relative to Toronto. The likelihood of adjuvant RT among those not registered at a cancer centre within 3 months of diagnosis was decreased (OR = 0.20 (95% CI (0.13, 0.30)) relative to those registered. The adjusted relative risk of amputation at any time following diagnosis was 3.48 (95% CI (1.63, 7.46)) among cases not attending a cancer centre. The adjusted relative risk of death was 1.4; 95% CI (1.1, 1.7) among those not attending a cancer centre. Conclusions: Cases not seen at a multidisciplinary cancer centre within 3 months following diagnosis of STSE have an increased relative risk for amputation at any time, and for death due to any cause. Many hypotheses for further study are suggested by the results of this analysis.