Recurrent Sarcoma Research Articles

Genomic Alterations Found in Recurrent Uterine Sarcomas of African-American Women [18A

INTRODUCTION: To describe the frequency of genomic alterations found in tumors from a population of African-American women with recurrent uterine sarcomas METHODS: Medical records were analyzed for all African American women with recurrent uterine sarcomas who underwent next generation sequencing of their tumors. Demographics were collected as well as all tumor genomic alterations. Data was evaluated by SPSS to see if any consistent genomic alterations were found. RESULTS: Twenty-four African-American women had next-generation sequencing on their recurrent uterine sarcomas with 12 having high-grade leiomyosarcomas (LMS), 11 having carcinosarcomas (MMMT), and one having an undifferentiated uterine sarcoma. The mean age of the patients was 54 years (median 52 years, 95%CI 51.2-57.7). The mean mutation burden/Mb was 6.4 mutations/Mb (median 4.5, 95%CI 1.7-11.1). Nineteen tumors had 1-5 mutations/Mb, 4 had 6-10 mutations/Mb and 1 had >10 mutations/Mb. p53 mutations occurred in the majority of tumors (LMS 66.7%, MMMT 63.6%; respectively). The gene with the second most alterations was PIK3CA (LMS 8.3%, MMMT 45.5%). BRCA2 mutations were the only other genomic alterations to occur in >10% of recurrent uterine sarcomas (LMS 16.7%, MMMT 27.3%). CONCLUSION: In this population of African-American women with recurrent uterine sarcomas, the 3 genes found to most commonly harbor genomic alterations were p53, PIK3CA, and BRCA2.

Recurrence of Ewing sarcoma: Is detection by imaging follow-up protocol associated with survival advantage?

The Cooperative Ewing Sarcoma Study and the Late Effects Surveillance System of the Society for Paediatric Oncology and Haematology recommend a structured follow-up imaging protocol (FUIP) for patients with Ewing sarcoma (EwS) with decreasing frequency of imaging over the first 5 years. The present study aims to assess the effectiveness of the FUIP for EwS patients regarding survival after relapse. A retrospective multicenter analysis on 160 eligible patients with EwS recurrence was performed. Potential survival differences following recurrence diagnosis between patients with protocol-detected and symptomatic relapse were investigated using the Kaplan-Meier method. Additional subgroup analyses were performed on the relapse type. Overall survival (OS) was calculated from diagnosis of relapse to last follow-up or death. In the multicenter analysis, recurrence was detected by FUIP in 77 of 160 patients (48%) and due to symptoms in 83 patients (52%). Regarding the entire study population, OS was significantly superior in patients with protocol-detected relapse compared to patients with symptomatic relapse (median, 2.4 vs. 1.2 years; P<0.001). In the subgroup analyses, patients whose lung recurrences were detected by the FUIP experienced longer survival after recurrence than those whose recurrences were detected symptomatically (P=0.023). In the 83 symptomatic patients, pain was the most prevalent symptom of relapse (72%). FUIP may benefit survival in EwS relapse, especially in lung recurrence. Pain was the leading symptom of relapse.

Possibility of predicting the efficiency of soft tissue sarcoma treatment on the basis of features of their immunological microenvironment

Цель. Изучить возможность использования оценки факторов локального клеточного иммунитета для прогнозирования эффективности лечения сарком мягких тканей.&#x0D; Методы. В исследование вошли 38 пациентов с саркомой мягких тканей: 22 с первичными опухолями и 16 с рецидивными, находившихся на хирургическом лечении в 2014-2016 гг. У всех пациентов с целью оценки показателей локального иммунитета исследовали полученные интраоперационно ткани опухоли, перитуморальной зоны и условно здоровые ткани, соответствующие линиям резекции (всего 114 образцов). Образцы тканей гомогенизировали, субпопуляции лимфоцитов определяли на проточном цитометре.&#x0D; Результаты. Исследованы характеристики лимфоцитарного микроокружения сарком мягких тканей в связи с клиническим эффектом комплексного лечения больных. При первичных саркомах развитию у больных бессобытийной выживаемости более 12 мес после операции соответствовало более высокое содержание NK-клеток в ткани опухоли и более высокое соотношение уровней NKT-лимфоцитов в перитуморальной зоне и опухоли, а также более низкий уровень Tregs, чем у больных с бессобытийной выживаемостью менее 12 мес. При рецидивных саркомах короткая бессобытийная выживаемость была связана с высокими показателями уровня DN-клеток в опухоли и соотношения доли CD3+-лимфоцитов в перитуморальной зоне и опухоли. Все указанные различия статистически значимы (р &lt;0,05).&#x0D; Вывод. Клинический эффект комплексного лечения сарком мягких тканей связан с исходным состоянием иммунологического микроокружения опухоли; ряд показателей локального клеточного иммунитета можно рассматривать в качестве прогностических факторов, различных для первичных и рецидивных сарком.

Resection arthrodesis using distraction osteogenesis then plating as a hybrid surgical technique for the management of bone sarcomas of the distal tibia.

We report the oncological and functional results of limb salvage for bone sarcomas involving the distal tibia using hybrid surgical technique of resection arthrodesis by bone transport then plating. Five patients (mean age 18.6years) with primary distal tibial sarcomas (two Ewing's sarcomas and three osteosarcomas) were treated by this method. The average duration of follow-up is 53months. All patients accepted distraction osteogenesis with a standard technique using external fixator after wide (four cases) or marginal (one case) resection in the first operation. They were re-admitted for the second surgical treatment (plate insertion and removal of the external fixator) one to two months after they achieved the necessary limb length and desired alignment. Solid union of the lengthening site and sound fusion of the ankle were achieved in all five patients with full and unassisted weight bearing. The mean lengthening was 11.8cm (range 8-14cm) and the external fixation index (EFI) was 29.3days/cm (range 22.8-36.3days/cm). The mean functional score according to the rating system of the Musculoskeletal Tumour Society was 88% (83-90%). One patient showed poor response to chemotherapy, had local recurrence of sarcoma one year after plating, and was treated with above-knee amputation. In carefully selected patients with primary distal tibial sarcomas, this hybrid method can effectively eliminate tumor lesion, reconstruct function, and shorten the length of wearing an external fixator by a meticulous conversion to internal fixator.

Efficacy and safety of apatinib monotherapy in advanced bone and soft tissue sarcoma: An observational study

ABSTRACT Sarcomas are rare but malignant tumors with high risks of local recurrence and distant metastasis. Anti-angiogenic therapy is a potential strategy against un-controlled and not-organized tumor angiogenesis. We aimed to assess the safety and efficacy of apatinib, an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor-2, in patients with advanced sarcoma. Thirty-one patients who received initial apatinib between September 2015 and August 2016 were retrospectively reviewed. Among them, 19 (61.3%) patients were heavily pretreated with two or more lines of cytotoxic chemotherapy. Apatinib was given at a start-dose of 425 mg qd. During therapy, 9 (29.0%) patients required dose interruption and 7 (22.6%) needed dose reduction, and the mean dosage of apatinib was 372.9 ± 68.4 mg/day. In the study cohort, one patient was treated as adjunctive therapy and 6 patients stopped treatment before radiographic response assessment. Thus, 24 patients were eligible for tumor response evaluation. The objective response rate was 33.3% and clinical benefit rate was as high as 75.0%. The progression free survival was 4.25 (95% confidence interval [CI], 2.22–5.11) months, whereas the overall survival was 9.43 (95% CI, 6.64–18.72) months. Compared with other histological subtypes, leiomyosarcoma did not show significant survival benefits. Most of the adverse events (AEs) were at grade 1 or 2. The main grade 3 AEs were hypertension (6.5%), hand foot skin reaction (6.5%), and diarrhea (3.2%). In conclusion, apatinib showed promising efficacy and acceptable safety profile in metastatic or recurrent sarcoma, giving rationale clinical evidence to conduct clinical trials.

局所再発をきたした乳腺periductal stromal sarcomaの1例

局所再発をきたした乳腺periductal stromal sarcomaの1例

Pulmonary Metastasectomy – Analysis of Survival and Prognostic Factors in 243 Patients

BACKGROUND Pulmonary metastases are a sign of advanced malignant disease. Interdisciplinary management of metastatic cancer mandates the consideration of all treatment options, and in selected patients pulmonary metastasectomy can be performed with curative intent. This study aims to analyze the prognostic factors associated with survival and optimize the selection of surgical candidates. The sarcoma subset analysis aims to examine the role of multiple repeat resections for pulmonary metastatic recurrence. METHODS A total of 243 patients were analyzed in this retrospective cohort study. Overall survival was estimated using Kaplan-Meier analysis. Univariate analyses with log-rank tests and multivariate analysis with Cox proportional hazards model were undertaken to determine the independent prognostic factors for survival. RESULTS Multivariate analyses identified germ cell cancer (P = 0.01) and a disease-free interval of >36 months (P = 0.006) as significant independent prognostic factors for improved survival, whilst synchronous metastases (P = 0.04), multiple metastases (P = 0.005) and incomplete resection (P < 0.001) were identified as significantly poor prognostic factors. Subset analyses identified that ≥2 repeat resections within the sarcoma cohort was associated with an increased median survival of 63.5 months (P = 0.04). CONCLUSION In selected patients, pulmonary metastasectomy can be performed with curative intent and have associated long-term survival benefits. Patients presenting with recurrent sarcoma pulmonary metastases should be considered for repeat metastasectomy.

Computed tomography-guided preoperative localization of musculoskeletal lesions using the ROLL technique.

ObjectiveTo describe the preoperative localization of musculoskeletal lesions with the radioguided occult lesion localization (ROLL) technique.Materials and MethodsIn all cases, computed tomography-guided injection of technetium-99m sulfur colloid was performed, directly into or near the suspicious lesion, up to 36 hours before the surgical procedure. Lesions were detected intraoperatively with a gamma probe.ResultsWe report the cases of six patients submitted to radioguided surgery, including three patients with bone lesions suspicious for metastasis, two patients suspected of recurrent sarcoma, and one patient with no previous diagnosis who had a nodular lesion on the left leg. Patients tolerated the procedure well, and no complications were associated with the puncture. All marked lesions were easily identified intraoperatively and were excised with clear margins.ConclusionThe ROLL technique was effective in the intraoperative localization of occult musculoskeletal lesions, demonstrating that it is a feasible and promising technique for the surgical exploration of selected cases.

Management of Locally Recurrent Sarcoma in the Extremity

Extremity sarcomas are a heterogeneous group of malignant tumors with a varied propensity for local recurrence. This review focuses on factors associated with local recurrence and survival, diagnostic workup, management, outcomes, and surveillance. Local recurrence is more common in patients with previous local recurrence, positive margins, high-grade histology, and deep tumors. In the absence of metastases, the mainstay of treatment is limb-sparing surgery, with radiation to improve local control. Modalities such as brachytherapy or proton therapy may be valuable in the setting of previous irradiation. Systemic chemotherapy is typically limited to the treatment of distant disease, although chemotherapy can be delivered locally via limb perfusion or infusion for locally advanced or recurrent disease. Amputation is used if local control cannot be achieved while preserving adequate limb function or as a palliative option for pain, bleeding, or fungating tumors. Prognostic factors associated with poor survival include tumors that recur with high-grade histology, with a large size (&gt; 5 cm), or within a short interval (&lt; 16 months). Reports of 5-year overall survival in patients with locally recurrent sarcomas vary from 36 to 65%. Surveillance includes physical examination, cross-sectional imaging, and chest x-ray. Genetic profiling and intratumoral injections provide novel therapeutic targets. This review contains 1 figure, 4 tables and 40 references. Key words: chemotherapy, hyperthermic isolated limb perfusion, intratumoral injection, isolated limb perfusion, local recurrence, margin status, radiation, soft tissue sarcoma, wide local excision

Management of Locally Recurrent Sarcoma in the Extremity

Extremity sarcomas are a heterogeneous group of malignant tumors with a varied propensity for local recurrence. This review focuses on factors associated with local recurrence and survival, diagnostic workup, management, outcomes, and surveillance. Local recurrence is more common in patients with previous local recurrence, positive margins, high-grade histology, and deep tumors. In the absence of metastases, the mainstay of treatment is limb-sparing surgery, with radiation to improve local control. Modalities such as brachytherapy or proton therapy may be valuable in the setting of previous irradiation. Systemic chemotherapy is typically limited to the treatment of distant disease, although chemotherapy can be delivered locally via limb perfusion or infusion for locally advanced or recurrent disease. Amputation is used if local control cannot be achieved while preserving adequate limb function or as a palliative option for pain, bleeding, or fungating tumors. Prognostic factors associated with poor survival include tumors that recur with high-grade histology, with a large size (&gt; 5 cm), or within a short interval (&lt; 16 months). Reports of 5-year overall survival in patients with locally recurrent sarcomas vary from 36 to 65%. Surveillance includes physical examination, cross-sectional imaging, and chest x-ray. Genetic profiling and intratumoral injections provide novel therapeutic targets. This review contains 1 figure, 4 tables and 40 references. Key words: chemotherapy, hyperthermic isolated limb perfusion, intratumoral injection, isolated limb perfusion, local recurrence, margin status, radiation, soft tissue sarcoma, wide local excision

Elevated Alkaline Phosphatase in a Cancer Patient: Think You Know the Source?

A 45-year-old male with recurrent metastatic Ewing sarcoma presented to Memorial Sloan Kettering Cancer Center for potential enrollment in a clinical trial. Initial laboratory results were notable for increased alkaline phosphatase levels [(ALP2, 495 U/L; (reference interval: 33–97 U/L); Abbott Architect C8000] and γ-glutamyl transferase levels [GGT, 393 U/L (reference interval: 0–73 U/L); Abbott Architect C8000] but were otherwise unremarkable, including normal levels of aspartate aminotransferase (AST) and alanine transaminase (ALT). Elevations in ALP and GGT levels suggested liver disease, although the patient had no previous history of liver abnormalities. Given the patient's history of metastatic bone disease, it was suspected that the observed elevation in ALP was due to increased bone ALP isoenzyme. However, it was important to document the origin of the increased ALP as the patient was to be enrolled in an investigational trial that would define drug dosage and side effects. Elevations in ALP over the course of the trial that were attributable to liver-specific isoenzymes would be reported as a dose-limiting toxicity and could delay treatment or potentially exclude the patient from the clinical trial. Conversely, elevations in ALP originating from bone and secondary to disease would not be reported as toxicity. The laboratory was consulted regarding testing options to identify the specific isoenzyme(s) responsible …

Soft tissue sarcoma: how can posttreatment alterations be distinguished from recurrences?

The recognition of recurrent soft tissue sarcomas and the differentiation from post-treatment alterations is complex. This article aims to assist the clinical radiologist in the systematic evaluation of local follow-up imaging in soft tissue sarcoma patients. Soft tissue sarcomas encompass multiple entities with different recurrence rates and follow-up intervals. Approved and up to date recommendations are provided, including imaging techniques. The past medical history of the patient, the clinical situation and previous therapies should be known in detail, including surgery, radiation therapy and chemotherapy. Previous imaging results should be consulted, if available. This article describes the time-dependent imaging spectrum of local post-therapeutic as well as local treatment-related complications. These include early complications, such as seromas, hematomas and infections, as well as late complications, including edema, fibrosis and joint stiffness, and also inflammatory pseudotumors, which may occur after variable time intervals. The imaging appearance of local recurrent and radiation-associated sarcoma are elucidated. In particular, magnetic resonance imaging (MRI) criteria are provided, which may help in differentiating post-therapeutic alterations from recurrent soft tissue sarcomas.

Cost-Effectiveness of Surveillance for Distant Recurrence in Extremity Soft Tissue Sarcoma.

Optimal distant recurrence (DR) surveillance strategies for extremity soft tissue sarcoma (STS) are unknown. We performed a cost-effectiveness analysis of different imaging modalities performed at guideline-specified intervals. We developed a Markov model simulating lifetime outcomes for 54-year-old patients after definitive treatment for American Joint Committee on Cancer stage II-III extremity STS using four surveillance strategies: watchful waiting (WW), chest X-ray (CXR), chest computed tomography (CCT), and positron emission tomography-computed tomography (PET/CT). Probabilities, utilities, and costs were extracted from the literature and Medicare claims to determine incremental cost-effectiveness ratios (ICER). CCT was the most effective and most costly strategy with CXR the most cost-effective strategy at a societal willing-to-pay (WTP) of $100,000/quality-adjusted life year (QALY). The ICER was $12,113/QALY for CXR versus $104,366/QALY for CCT while PET/CT was never cost-effective. Sensitivity analyses demonstrated CCT becomes the preferred imaging modality as the lifetime risk of DR increases beyond 33% or as the WTP increases beyond $120,000/QALY. Optimal DR surveillance imaging for stage II-III extremity STS should be individualized based on patients' risks for DR. These results suggest CXR, or CCT performed at more protracted intervals, may be preferred for lower-risk patients (i.e., DR risk <33%), whereas CCT may be preferred for higher-risk patients (i.e., DR risk >33%). Further study of optimal strategies is needed. In the interim, these findings may help to refine guidelines to reduce resource overutilization during routine surveillance of lower-risk sarcoma patients.

Neem leaf glycoprotein generates superior tumor specific central memory CD8+ T cells than cyclophosphamide that averts post-surgery solid sarcoma recurrence.

The success of cancer vaccines is limited as most of them induce corrupted CD8+ T cell memory populations. We reported earlier that a natural immunomodulator, neem leaf glycoprotein (NLGP), therapeutically restricts tumor growth in a CD8+ T cell-dependent manner. Here, our objective is to study whether memory CD8+ T cell population is generated in sarcoma hosts after therapeutic NLGP treatment and their role in prevention of post-surgery tumor recurrence, in comparison to the immunostimulatory metronomic cyclophosphamide (CTX) treatment. We found that therapeutic NLGP and CTX treatment generates central memory CD8+ T (TCM) cells with characteristic CD44+CD62LhighCCR7highIL-2high phenotypes. But these TCM cells are functionally impaired to prevent re-appearance of tumors along with compromised proliferative, IL-2 secretive and cytotoxic status. This might be due to the presence of tumor load, even a small one in the host, which serves as a persistent source of tumor antigens thereby corrupting the TCM cells so generated. Surgical removal of the persisting tumors from the host restored the functional characteristics of memory CD8+ T cells, preventing tumor recurrence after surgery till end of the experiment. Moreover, we observed that generation of superior TCM cells in NLGP treated surgically removed tumor hosts is related to the activation of Wnt signalling in memory CD8+ T cells with concomitant inhibition of GSK-3β and stabilisation of β-catenin, which ultimately activates transcription of Wnt target genes, like, eomesodermin, a signature molecule of CD8+ TCM cells.

Cost effectiveness of surveillance for distant recurrence in extremity soft tissue sarcoma.

11021 Background: Optimal surveillance strategies for extremity soft tissue sarcoma (STS) are unknown. We performed a cost-effectiveness analysis of competing imaging modalities performed at National Cancer Comprehensive Network guideline-specified intervals. Methods: We developed a Markov model simulating lifetime outcomes for 54-year-old patients after definitive treatment for Stage II-III extremity STS using four surveillance strategies: watchful waiting (WW), chest x-ray (CXR), chest computed tomography (CCT) and positron emission tomography-computed tomography (PET/CT) performed every 3-6 months for the first 3 years, every 6 months until year 5, and then annually. We used probabilities, utilities and costs extracted from the literature and Medicare claims to determine incremental cost-effectiveness ratios (ICER). Results: While the model showed that CCT is the most effective strategy, CXR is the most cost-effective strategy at a societal willing-to-pay (WTP) of $100,000/quality-adjusted life year (QALY). The ICER is $14,306/QALY for CXR versus $117,683/QALY for CCT while PET/CT is never cost effective (Table). Sensitivity analyses demonstrated CCT becomes the preferred imaging modality as the lifetime risk of DR increases beyond 38% or as the societal WTP increases beyond $130,000/QALY. Conclusions: Optimal DR surveillance imaging for Stage II-III extremity STS should be individualized based on patients’ risks for DR. CXR, or CCT at more protracted intervals, may be preferred for lower risk patients (i.e. DR risk less than 38%), whereas CCT may be preferred for higher risk patients (i.e. DR risk greater than 38%). These findings can help refine guidelines to reduce resource overutilization during surveillance of sarcoma patients. [Table: see text]

BLOOD SUPPLY FEATURES OF SOFT TISSUE SARCOMAS OF THE EXTREMITIES

Surgical removal of tumor remains the only one radical treatment for patients with soft tissue sarcomas of the extremities. However, the parameters of total and disease-free survival after this type of treatment cannot be considered satisfactory. Currently the active search and introduction into clinical practice of additional impact methods that can improve the immediate and long-term results of treatment of such patients is being conducted. In the article there are presented results of the analysis of preoperative angiographic studies of 94 patients with primary and recurrent sarcomas of soft tissues of the extremities. The features of blood supply and internal angioarchitectonics of tumor were revealed depending on the size, histological type and localization. The features of blood supply of tumors in patients with primary and recurrent sarcomas of soft tissues of extremities were described in detail. The results of further surgical treatment of patients included in the study showed that information on the number and size of blood supply sources of the tumor zone, supplemented by selective embolization of vessels, made it possible to select the optimal plan and minimize trauma, reduce intraoperative hemorrhage, and perform organ-preserving and functionally beneficial operations.

Neem leaf glycoprotein prevents post-surgical sarcoma recurrence in Swiss mice by differentially regulating cytotoxic T and myeloid-derived suppressor cells.

Post-surgical tumor recurrence is a common problem in cancer treatment. In the present study, the role of neem leaf glycoprotein (NLGP), a novel immunomodulator, in prevention of post-surgical recurrence of solid sarcoma was examined. Data suggest that NLGP prevents tumor recurrence after surgical removal of sarcoma in Swiss mice and increases their tumor-free survival time. In NLGP-treated tumor-free mice, increased cytotoxic CD8+ T cells and a decreased population of suppressor cells, especially myeloid-derived suppressor cells (MDSCs) was observed. NLGP-treated CD8+ T cells showed greater cytotoxicity towards tumor-derived MDSCs and supernatants from the same CD8+ T cell culture caused upregulation of FasR and downregulation of cFLIP in MDSCs. To elucidate the role of CD8+ T cells, specifically in association with the downregulation in MDSCs, CD8+ T cells were depleted in vivo before NLGP immunization in surgically tumor removed mice and tumor recurrence was noted. These mice also exhibited increased MDSCs along with decreased levels of Caspase 3, Caspase 8 and increased cFLIP expression. In conclusion, it can be stated that NLGP, by activating CD8+ T cells, down regulates the proportion of MDSCs. Accordingly, suppressive effects of MDSCs on CD8+ T cells are minimized and optimum immune surveillance in tumor hosts is maintained to eliminate the residual tumor mass appearing during recurrence.

Pediatric Primary Malignant Bone Tumor

: Primary bone tumors are the sixth most common neoplasm occurring in children and constitute approximately 6% of all childhood malignancies with a peak incidence in 15- to 19-year- old individuals. These lesions are the third most Common tumors in adolescents and young adults (exceeded only by leukemia and lymphoma) common complaints are intermittent to constant mild to severe pain, constitutional symptoms (fever, weight loss). Swelling, erythema in the affected extremity; fracture and lesions may be found incidentally on radiographs obtained for other reasons. Radiographic distinction are poorly circumscribed mass with cortical disruption; multiple layers of poorly organized periosteal new bone (eg, and quot; onionskin and quot; appearance of Ewings sarcoma); slow-growing lesions will have moth-eaten lucencies, while faster-growing lesions will have permeative destruction Osteosarcoma and small round cell tumor such as Ewing sarcoma and lymphoma are the most common malignant bone tumors in this age group. Although the overall incidence of osteosarcoma is higher than Ewing sarcoma in adolescents younger than 20 years, Ewing sarcoma is more common in children younger than 10 years of age. MRI or CT to determine extent of skeletal involvement and biopsy is necessary for definitive histologic diagnosis. LDH can be used as a marker in order to detect response to therapy as well as for recurrence in Ewing sarcoma; and elevated serum alkaline phosphatase in osteosarcoma.

Hypoxia-inducible factor 1α predicts recurrence in high-grade soft tissue sarcoma of extremities and trunk wall

Background and aimSarcomas are of mesenchymal origin and typically show abundant tumour stroma and presence of necrosis. In search for novel biomarkers for personalised therapy, we determined the prognostic impact...

Treatment patterns and survival in an exhaustive French cohort of pazopanib-eligible patients with metastatic soft tissue sarcoma (STS)

BackgroundThe French EMS study prospectively collected exhaustive data from STS patients diagnosed in the Rhone-Alpes region from 2005 to 07.MethodsThe database included diagnosis/histology, surgery, radiotherapy, systemic treatments and treatment response. Treatment patterns and outcomes of patients with metastatic disease, excluding adipocytic sarcoma and GIST were analyzed.ResultsOf 888 total patients, 145 were included based on having metastatic disease and appropriate subtypes. All patients received treatment with systemic therapy being most common (74%, n = 107), followed by radiotherapy (30%, n = 44) and surgery (23%, n = 33). Doxorubicin, alone or in combination, was the most common first line systemic therapy (65%, n = 46). Drugs without license in sarcoma were used in 38–83% of treatments depending on treatment line. 24% of frontline patients demonstrated an objective response, decreasing to 11% objective responses in second line but no responses were documented beyond second line, with median PFS declining with each additional line. Median PFS also declined in patients receiving surgery compared to those receiving no surgery (8–15 m vs 5 m). Median OS from metastatic diagnosis for patients receiving systemic therapy was double that of patients without systemic treatment (24 m vs 12 m, p = 0.007).ConclusionsOutcomes in this population were poor and declined with successive treatment. However, results suggest that further anticancer therapies in recurrent sarcoma might be beneficial.