Sarcoma[JOURNAL]
Sun
200 papers
Adult Primary Bone Sarcoma and Time to Treatment Initiation: An Analysis of the National Cancer Database.
Lawrenz JM, Curtis GL, Styron JF … +7 more , George J, Anderson PM, Zahler S, Shepard DR, Rubin BP, Nystrom LM, Mesko NW
Sarcoma · 2018 · PMID 30533997 · Full text
OBJECTIVE: The time to treatment interval (TTI), defined as the period from diagnosis to first definitive treatment, has very limited descriptions toward understanding delays in primary bone sarcoma (PBS) care. Our prima...
OBJECTIVE: The time to treatment interval (TTI), defined as the period from diagnosis to first definitive treatment, has very limited descriptions toward understanding delays in primary bone sarcoma (PBS) care. Our primary goal was to determine the national standard for time to treatment initiation (TTI) in PBS in adults and to identify characteristics associated with TTI variability. METHODS: An analysis of the National Cancer Database identified 15,083 adult patients with PBS diagnosed from 2004 to 2013. Kruskal-Wallis analysis identified differences between covariates regarding TTI and regression modeling identified covariates that independently influenced TTI. RESULTS: The median TTI was 22 days. Approximately 60% of patients were definitively treated in the same center where the index diagnosis was made. Increased TTI was correlated with a transition in care institution (incidence rate ratio (IRR) = 1.89; < 0.001), being uninsured (IRR = 1.36; < 0.001), primary tumor site in the pelvis (IRR = 1.26; < 0.001), Medicaid insurance status (IRR = 1.22; < 0.001), care at an academic center (IRR = 1.14; < 0.001), non-white race (IRR = 1.12; =0.002), and Medicare insurance status (IRR = 1.08; =0.017). Decreased TTI was correlated with a diagnosis of chondrosarcoma (IRR = 0.85; < 0.001), having surgery as the index treatment (IRR = 0.88; < 0.001), a primary tumor site of the lower (IRR = 0.91; =0.001) or upper extremity (IRR = 0.92; =0.023), and stage II or stage III disease (IRR = 0.91; =0.010). CONCLUSIONS: TTI is associated with tumor, treatment, and socioeconomic and healthcare system characteristics. Transitions in care between institutions are responsible for the greatest increase in TTI. As TTI is more commonly used as a quality metric, physicians need to be aware of the causes for prolonged TTI, as we work to improve national delays in diagnosis and treatment initiation.
Myxoid Liposarcoma: Treatment Outcomes from Chemotherapy and Radiation Therapy.
Chowdhry V, Goldberg S, DeLaney TF … +5 more , Cote GM, Chebib I, Kim J, Lozano-Calderón SA, De Amorim Bernstein K
Sarcoma · 2018 · PMID 30515050 · Full text
INTRODUCTION: Myxoid liposarcoma (MLS) is a subtype of liposarcoma characterized morphologically by lipomatous differentiation with a myxoid stroma. The purpose of this study was to review clinical and pathological infor...
INTRODUCTION: Myxoid liposarcoma (MLS) is a subtype of liposarcoma characterized morphologically by lipomatous differentiation with a myxoid stroma. The purpose of this study was to review clinical and pathological information for patients treated for MLS at our institution to better understand neoadjuvant and adjuvant therapy. MATERIALS AND METHODS: An institutional database of sarcomas was queried for patients who were treated for MLS at our institution between 1992 and 2013. Survival curves were constructed using Kaplan-Meier analysis, and univariate and multivariate statistics were performed using the Cox-proportional hazards model and using linear regression. RESULTS: A total of 85 patients with myxoid liposarcoma were identified. The mean and median histologic response rate to treatment for patients who received preoperative radiation therapy was 77.6%. Five-year disease-free survival, distant metastasis-free survival, local recurrence-free survival, and overall survival were 78.6% (95% CI: 67.8-86.1), 84.7% (95% CI: 74.5-91.0), 95.6% (95% CI: 86.9-98.6), and 87.5% (95% CI: 77.2-93.3) respectively. On univariate analysis, there was a trend towards higher necrosis or treatment response rates in patients who received concurrent chemotherapy, 84.7% (95% CI: 75.9-93.4) and 69.5% (95% CI: 55.1-83.8), =0.061. Tumor size was associated with inferior disease-free and overall survival. Hazard ratio for disease-free survival is 1.08 (per cm) (95% CI: 1.01-1.16), =0.019. CONCLUSIONS: Myxoid liposarcoma exhibits histological response to chemotherapy and radiation therapy. Tumor size appears to be greatest predictor of long-term disease control and overall survival. We were not able to show that chemotherapy provides a clinical benefit with regard to local control, disease-free survival, or overall survival. However, it is important to note that the selected usage of chemotherapy in the highest risk patients confounds this analysis. Further investigation is needed to help better determine the optimal use of chemotherapy in this group of patients.
SARC018_SPORE02: Phase II Study of Mocetinostat Administered with Gemcitabine for Patients with Metastatic Leiomyosarcoma with Progression or Relapse following Prior Treatment with Gemcitabine-Containing Therapy.
Choy E, Ballman K, Chen J … +8 more , Dickson MA, Chugh R, George S, Okuno S, Pollock R, Patel RM, Hoering A, Patel S
Sarcoma · 2018 · PMID 30473623 · Full text
Histone deacetylase inhibitors (HDACi) can reverse chemoresistance, enhance chemotherapy-induced cytotoxicity, and reduce sarcoma proliferation in cell lines and animal models. We sought to determine the safety and toxic...
Histone deacetylase inhibitors (HDACi) can reverse chemoresistance, enhance chemotherapy-induced cytotoxicity, and reduce sarcoma proliferation in cell lines and animal models. We sought to determine the safety and toxicity of mocetinostat and its ability to reverse chemoresistance when administered with gemcitabine in patients with metastatic leiomyosarcoma resistant to prior gemcitabine-containing therapy. Participants with metastatic leiomyosarcoma received mocetinostat orally, 70 mg per day, three days per week, increasing to 90 mg after three weeks if well tolerated. Gemcitabine was administered at 1,000 mg/m intravenously at 10 mg/m/minute on days five and 12 of every 21-day cycle. Disease response was evaluated with CT or MRI. Twenty participants with leiomyosarcoma were evaluated for toxicity. Median time to disease progression was 2.0 months (95% CI 1.54-3.12). Eighteen participants were evaluated for radiologic response by RECIST 1.1. Best responses included one PR and 12 SD. Tumor size reduced in 3 patients. Most common toxicities were fatigue, thrombocytopenia, anemia, nausea, and anorexia. One patient experienced a significant pericardial adverse event. No study-related deaths were observed. Rechallenging with gemcitabine by adding mocetinostat was feasible and demonstrated modest activity in patients with leiomyosarcoma. Further studies are needed to better define the role of HDAC inhibitors in patients with metastatic leiomyosarcoma.
Periosteal Osteosarcoma: A Single-Institutional Study of Factors Related to Oncologic Outcomes.
Chan CM, Lindsay AD, Spiguel ARV … +2 more , Gibbs CP, Scarborough MT
Sarcoma · 2018 · PMID 30363676 · Full text
BACKGROUND: Periosteal osteosarcoma is a rare surface-based variant with a lower propensity to metastasis and better prognosis than conventional osteosarcoma. The literature supporting survival benefit with adjuvant chem...
BACKGROUND: Periosteal osteosarcoma is a rare surface-based variant with a lower propensity to metastasis and better prognosis than conventional osteosarcoma. The literature supporting survival benefit with adjuvant chemotherapy is lacking. Our institutional practice is for chemotherapy to be offered to patients with high-grade disease. METHODS: We conducted a retrospective cohort study of patients managed for periosteal osteosarcoma from 1970 to 2015 analyzing the survival outcomes and assessing for any relationship of survival to patient- or treatment-related factors. 18 patients were included. The study population presented at a mean of 20.8 years and was followed for a mean of 10.7 years. Factors assessed for an association with survival included age, size of tumor, use of chemotherapy, presence of medullary involvement, presence of high-grade disease, local recurrence, and site of disease. Kaplan-Meier survival analysis and Cox proportional hazard regression were performed to calculate the survival rates and to assess for the effect of any factor on survival. RESULTS: 10-year overall survival rate was 77.1%, and 10-year event-free survival rate was 66.4%. No factor was found to have an association with overall or event-free survival. CONCLUSION: These findings add to the available evidence which has failed to find any survival benefit from chemotherapy; patients with this rare disease and their families should be counselled regarding the unclear role of chemotherapy in this rare subtype of osteosarcoma.
Health Care Resource Utilization and Costs among Adult Patients with Advanced Soft Tissue Sarcoma: A Retrospective Medical Record Review in the United Kingdom, Spain, Germany, and France.
Mytelka DS, Nagar SP, D'yachkova Y … +6 more , La EM, Kaye JA, Candrilli SD, Kasper B, Lopez-Martin JA, Lorenzo M
Sarcoma · 2018 · PMID 30297977 · Full text
OBJECTIVE: To describe health care resource utilization and costs for patients with advanced soft tissue sarcoma (STS) in the United Kingdom (UK), Spain, Germany, and France. METHODS: Physicians abstracted data for adult...
OBJECTIVE: To describe health care resource utilization and costs for patients with advanced soft tissue sarcoma (STS) in the United Kingdom (UK), Spain, Germany, and France. METHODS: Physicians abstracted data for adult patients with a diagnosis of advanced STS (other than Kaposi's sarcoma or gastrointestinal stromal tumor) who received ≥1 lines of systemic therapy. Health care resource utilization related to advanced STS treatment was recorded; associated costs were estimated by applying unit costs. RESULTS: A total of 130 physicians provided data for 807 patients (UK: 199; Spain: 203; Germany: 204; and France: 201). The site of care during active treatment varied based on differences in the health care systems of these four countries. Total mean per-patient health care cost in the UK was £19,457; in Spain, €26,814; in Germany, €20,468; and in France, €24,368. Advanced STS-related systemic treatment costs were driven primarily by drug acquisition and administration costs. Treatment-related costs increased during later lines of therapy for all countries except France, where they decreased after first-line therapy. Pain control and antiemetics were the most common supportive care medications. CONCLUSIONS: This study provides real-world data on resource utilization and estimated costs in advanced STS and could inform policymakers about treatment burden.
Benign Smooth Muscle Tumors (Leiomyomas) of Deep Somatic Soft Tissue.
McCarthy AJ, Chetty R
Sarcoma · 2018 · PMID 30271265 · Full text
Leiomyomas of deep soft tissue are extremely rare and should only be diagnosed following adherence to stringent histological criteria, namely, the absence of nuclear atypia and of coagulative tumor necrosis. Whether extr...
Leiomyomas of deep soft tissue are extremely rare and should only be diagnosed following adherence to stringent histological criteria, namely, the absence of nuclear atypia and of coagulative tumor necrosis. Whether extremely low counts of, or even any, mitotic activity are acceptable when making a diagnosis of leiomyoma in deep soft tissue sites is controversial. The morphology and immunophenotype of smooth muscle tumors in deep soft tissue are similar to their counterparts irrespective of topography. It is interesting to note that leiomyomas of deep soft tissue (extremity and retroperitoneum) are often hyalinized/sclerosed and calcified. However, the prediction of their behavior and correct codification is dependent on thorough, meticulous search for mitoses and necrosis. Leiomyomas of deep soft tissue in the extremity should be devoid of mitoses and "significant" cytological atypia. An occasional larger, slightly pleomorphic cell in the midst of bland spindle cells, can be regarded as insignificant atypia. If any mitotic activity and several atypical cells are encountered in smooth muscle tumors of deep soft tissue of the extremity, it would be prudent to invoke the appellation of smooth muscle tumor of uncertain malignant potential and advocate wide local excision and follow-up.
Addressing the Adult Soft Tissue Sarcoma Microenvironment with Intratumoral Immunotherapy.
Raj S, Miller LD, Triozzi PL
Sarcoma · 2018 · PMID 30158830 · Full text
Sarcoma is comprised of a heterogeneous group of tumors originating from the mesenchyme. Sarcoma is also the first tumor that responded to immunotherapeutic agents often termed as "Coley's toxins." However, immunotherapy...
Sarcoma is comprised of a heterogeneous group of tumors originating from the mesenchyme. Sarcoma is also the first tumor that responded to immunotherapeutic agents often termed as "Coley's toxins." However, immunotherapy is yet to establish its presence in sarcomas. Complex interactions between tumor and immune cells in the tumor microenvironment play a crucial role in response to immunotherapy. There is a dynamic equilibrium created by the immune cells infiltrating the tumor, and this forms the basis of tumor evasion. Manipulating the intratumoral microenvironment will help overcome tumor evasion.
Comparison of Epidemiology, Clinical Features, and Outcomes of Patients with Reported Ewing Sarcoma and PNET over 40 Years Justifies Current WHO Classification and Treatment Approaches.
Campbell K, Shulman D, Janeway KA … +1 more , DuBois SG
Sarcoma · 2018 · PMID 30158829 · Full text
BACKGROUND: As of 2013, the WHO has classified peripheral primitive neuroectodermal tumors (PNETs) within the umbrella of Ewing sarcoma family of tumors (ESFTs) given their shared biology. Histologic features differ betw...
BACKGROUND: As of 2013, the WHO has classified peripheral primitive neuroectodermal tumors (PNETs) within the umbrella of Ewing sarcoma family of tumors (ESFTs) given their shared biology. Histologic features differ between PNET and Ewing sarcoma (ES), and potential clinical differences between PNET and ES have not been fully elucidated. METHODS: Through the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database, we identified 3,575 patients identified with histologic diagnosis of ES or PNET from 1973 to 2014. We used Fisher's exact tests to compare patient and tumor characteristics between groups. Kaplan-Meier methods were used to estimate overall survival. RESULTS: Patients with ES were more likely to be male, ≤18 years old at diagnosis, white, and hispanic compared to patients with PNET (=0.016 for sex; < 0.001 for all other variables). Patients with PNET were more likely to have soft tissue primary tumors ( < 0.001), and among those with bone tumors, a lower rate of axial or pelvic tumors ( < 0.001). Patients with PNET had significantly worse 5-year survival compared to ES patients, though the absolute difference was small (51.3% versus 55.5%; < 0.001). Survival of patients with PNET diagnosed in the 1990s or later more closely approximated patients with ES, while patients with PNET diagnosed in the 1980's and earlier had inferior outcomes. CONCLUSIONS: Despite shared underlying biology, patients with PNET and ES show differences in clinical presentation and overall survival, with the latter differences largely mitigated in more recent decades.
The Volume-Outcome Relationship in Retroperitoneal Soft Tissue Sarcoma: Evidence of Improved Short- and Long-Term Outcomes at High-Volume Institutions.
Bagaria SP, Neville M, Gray RJ … +4 more , Gabriel E, Ashman JB, Attia S, Wasif N
Sarcoma · 2018 · PMID 30140165 · Full text
BACKGROUND: We sought to study the association between RPS case volume and outcomes. Although a relationship has been demonstrated between case volume and patient outcomes in some cancers, such a relationship has not bee...
BACKGROUND: We sought to study the association between RPS case volume and outcomes. Although a relationship has been demonstrated between case volume and patient outcomes in some cancers, such a relationship has not been established for retroperitoneal sarcomas (RPSs). STUDY DESIGN: The National Cancer Database (NCDB) was queried for patients undergoing treatment for primary RPS diagnosed between 2004 and 2013. Mean annual patient volume for RPS resection was calculated for all hospitals and divided into low volume (<5 cases/year), medium volume (5-10 cases/year), and high volume (>10 cases/year). Risk-adjusted regression analyses were performed to identify predictors of 30-day surgical mortality, 0 margin status, and overall survival (OS). RESULTS: Our study population consisted of 5,407 patients with a median age of 61 years, of whom 47% were male and 3,803 (70%) underwent surgical resection. Absolute 30-day surgical mortality and 0 margin rate following surgery for low-, medium-, and high-volume institutions were 2.4%, 1.3%, and 0.5% (=0.027) and 68%, 65%, and 82%, ( < 0.001), respectively. Five-year overall survival rates for low, medium, and high-volume institutions were 56%, 57%, and 66%, respectively ( < 0.001). Patients treated at low-volume institutions had a significantly higher risk of 30-day mortality (adjusted OR = 4.66, 95% CI 2.26-9.63) and long-term mortality (adjusted HR = 1.56, 95% CI 1.16-2.11) compared to high-volume institutions. CONCLUSION: We demonstrate the existence of a hospital sarcoma service line volume-oncologic outcome relationship for RPS at the national level and provide benchmark data for cancer care delivery systems and policy makers.
A Longitudinal Study of Functional Outcomes in Patients with Limb Salvage Surgery for Soft Tissue Sarcoma.
Oh E, Seo SW, Han KJ
Sarcoma · 2018 · PMID 30123044 · Full text
BACKGROUND: Many studies have reported on the surgical outcomes of soft tissue sarcoma. However, there was no longitudinal cohort study. Because time is the most valuable factor for functional recovery, adjusting time va...
BACKGROUND: Many studies have reported on the surgical outcomes of soft tissue sarcoma. However, there was no longitudinal cohort study. Because time is the most valuable factor for functional recovery, adjusting time value was the key for finding the causal relationship between other risk factors and postoperative function. Therefore, existing cross-sectional studies can neither fully explain the causal relationship between the risk factors and the functional score nor predict functional recovery. The aim of this study was to determine important predictive factors that affect postoperative functional outcomes and longitudinal changes in functional outcomes in patients who had undergone limb-sparing surgery (LSS) for soft tissue sarcoma (STS). METHODS: Between January 2008 and December 2014, we retrospectively enrolled 150 patients who had undergone LSS for STS and had been assessed for postoperative functional outcomes with questionnaires. To evaluate functional outcomes, we used the Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score (TESS). Multivariate generalized estimating equation (GEE) analysis was used to identify the predictive factors, including size, stage, and anatomic location of tumor, bone resection, flap reconstruction, age, and time after surgery. Each continuous variable such as age and time after surgery was explored for statistically significant cutoff points using the Wilcoxon rank sum test. RESULTS: Functional scores significantly improved until the second year after surgery and plateaued for the rest of the 5-year period. Age ( < 0.0001), bone resection (=0.0004), and time after surgery ( < 0.0001) were identified as significant predictive factors. The functional score was significantly higher in patients younger than 47 years old. CONCLUSIONS: Functional outcomes can improve until the second year after surgery. Patients who were older than 47 and underwent bone resection may have poor final functional outcomes.
Outcome of Nonsurgical Management of Extra-Abdominal, Trunk, and Abdominal Wall Desmoid-Type Fibromatosis: A Population-Based Study in the Netherlands.
van Broekhoven DLM, Verschoor AJ, van Dalen T … +16 more , Grünhagen DJ, den Bakker MA, Gelderblom H, Bovee JVMG, Haas RLM, Bonenkamp HJ, van Coevorden F, Ten Oever D, van der Graaf WTA, Flucke UE, Pras E, Reyners AKL, Westermann AM, Oldenburger F, Verhoef C, Steeghs N
Sarcoma · 2018 · PMID 30034268 · Full text
INTRODUCTION: Nonsurgical management of patients with desmoid-type fibromatosis (DF) is increasing. This study tries to provide insight on type, usage, and outcome of first-line nonsurgical management strategies. PATIENT...
INTRODUCTION: Nonsurgical management of patients with desmoid-type fibromatosis (DF) is increasing. This study tries to provide insight on type, usage, and outcome of first-line nonsurgical management strategies. PATIENTS AND METHODS: From the Dutch Pathology Registry (PALGA), patients with extra-abdominal or trunk/abdominal wall DF, diagnosed between 1993 and 2013, were identified. First-line treatment was analyzed. Best response (BR) using RECIST criteria from start of treatment/surveillance until change of treatment or last follow-up was analyzed. RESULTS: Ninety-one of the 1141 identified patients had first-line nonsurgical management. The percentage of patients treated nonsurgically increased from 0.6% in 1993-1998 to 12.8% in 2009-2013. Thirty-seven patients had surveillance (41%), 35 radiotherapy (38%), and 19 systemic treatment (21%). BR for surveillance was complete response (CR) in 2/37, partial response (PR) in 4/37, stable disease (SD) in 21/37, progressive disease (PD) in 5/37, and unknown in 5/37 patients. BR for radiotherapy was CR in 4/35, PR in 11/35, SD in 16/35, and unknown in 4/35. BR for systemic treatment was CR in 1/19, PR in 1/19, SD in 10/19, PD in 2/19, and unknown in 5/19. Totally, 91% of patients did not progress. DISCUSSION: Given the low percentage (9%) of PD of nonsurgical management, these data can be used in shared decision making with the patient regarding optimal treatment.
Safety and Accuracy of Core Needle Biopsy for Soft Tissue Masses in an Ambulatory Setting.
Walker JB, Stockwell E, Worhacz K … +2 more , Kang P, Decomas A
Sarcoma · 2018 · PMID 30008580 · Full text
BACKGROUND: Percutaneous needle biopsy has been found to be a safe and accurate method for the initial investigation of soft tissue masses. The notion exists that needle biopsies should be performed in specialized sarcom...
BACKGROUND: Percutaneous needle biopsy has been found to be a safe and accurate method for the initial investigation of soft tissue masses. The notion exists that needle biopsies should be performed in specialized sarcoma centers, which can place a financial burden on patients without a sarcoma center near their place of residence. There is no consensus in the current literature regarding the diagnostic accuracy and clinical utility of clinic-based percutaneous core needle biopsy performed by community orthopedic surgeons with fellowship training in musculoskeletal oncology. QUESTIONS/PURPOSES: Our primary goal was to determine if office-based core needle biopsy of soft tissue masses could safely yield accurate diagnoses when performed by a community orthopedic surgeon with fellowship training in musculoskeletal oncology. PATIENTS AND METHODS: We retrospectively reviewed the charts of 105 patients who underwent percutaneous core needle biopsy of soft tissue masses in a community clinic. All procedures were performed by one fellowship-trained musculoskeletal oncologist. Accuracy of the initial clinic-based needle biopsy was determined through comparison to the results of pathological analysis of the surgically excised masses. Final data analysis included 69 patients who underwent both clinic-based biopsy and subsequent surgical excision of their masses. RESULTS: We found clinic-based biopsies to be 87.0% accurate for exact diagnosis and 94.2% accurate in determining whether the mass was benign or malignant ( < 0.0001). Minor complications related to the clinic-based biopsy occurred in 5.80% of cases, with no documentation of major complications. CONCLUSIONS: Our results provide evidence that office-based percutaneous biopsy can be administered safely and yield accurate, clinically useful results when performed by a fellowship-trained musculoskeletal oncologist.
Treatment Patterns and Survival among Adult Patients with Advanced Soft Tissue Sarcoma: A Retrospective Medical Record Review in the United Kingdom, Spain, Germany, and France.
Nagar SP, Mytelka DS, Candrilli SD … +5 more , D'yachkova Y, Lorenzo M, Kasper B, Lopez-Martin JA, Kaye JA
Sarcoma · 2018 · PMID 29977133 · Full text
OBJECTIVE: To describe real-world treatment patterns and outcomes for patients with advanced soft tissue sarcoma (STS) not amenable to surgery or radiotherapy in the United Kingdom, Spain, Germany, and France. METHODS: P...
OBJECTIVE: To describe real-world treatment patterns and outcomes for patients with advanced soft tissue sarcoma (STS) not amenable to surgery or radiotherapy in the United Kingdom, Spain, Germany, and France. METHODS: Physicians completed a web-based medical record abstraction for adult patients with advanced STS (other than Kaposi's sarcoma or gastrointestinal stromal tumor) who received ≥1 line of systemic therapy. Clinical characteristics, treatments, tumor responses, and mortality data were recorded. RESULTS: A total of 130 physicians provided data for 807 patients. Patients' mean age at advanced STS diagnosis was 57.1 (±12.3) years; 59% were male. The most commonly identified histologic categories were leiomyosarcoma (28%), liposarcoma (13%), and rhabdomyosarcoma (11%). Overall, 57% of patients received only 1 line of therapy, 32% received 2 lines of therapy, and 11% received ≥3 lines of therapy. The most common first-line regimens were doxorubicin alone (41%), doxorubicin plus ifosfamide (19%), docetaxel plus gemcitabine (9%), paclitaxel alone (4%), and ifosfamide (4%). Median overall survival from start of treatment was estimated to be 17.6 months (95% confidence interval, 15.6-19.0 months). CONCLUSIONS: In real-world clinical practice, advanced STS is most commonly treated with older therapies in the United Kingdom, Spain, Germany, and France. New therapies that improve overall survival in advanced STS are needed.
Role of High-Dose Chemotherapy and Autologous Hematopoietic Cell Transplantation for Children and Young Adults with Relapsed Ewing's Sarcoma: A Systematic Review.
Tenneti P, Zahid U, Iftikhar A … +4 more , Yun S, Sohail A, Warraich Z, Anwer F
Sarcoma · 2018 · PMID 29973774 · Full text
BACKGROUND: Relapsed Ewing's sarcoma (RES) is an aggressive malignancy with poor survival. Although high-dose chemotherapy (HDCT) with autologous stem cell transplantation (ASCT) given after conventional chemotherapy (CC...
BACKGROUND: Relapsed Ewing's sarcoma (RES) is an aggressive malignancy with poor survival. Although high-dose chemotherapy (HDCT) with autologous stem cell transplantation (ASCT) given after conventional chemotherapy (CC) has shown survival benefits, it is not generally used in the United States for RES. We performed a systemic review to evaluate the benefits of HDCT for RES. METHODS: Literature search involved Medline, Embase, and Cochrane database. We included studies with RES patients treated with HDCT/ASCT. RESULTS: Twenty-four studies with total of 345 reported RES patients that got HDCT were included in final analysis. Seventeen studies had patients with multiple malignancies including RES, while seven had only RES patients. At 2 and 3-5 years, event-free survival (EFS) in studies with only RES patients ranged 42-47% and 20-61% and overall survival (OS) ranged 50-66% and 33-77%, respectively. In studies with combined patients that reported outcomes of RES separately, the EFS at 1-3 and 4 years was 36-66% and 17-50%, respectively. The OS at 1-2 and 3-4 years was 40-60% and 50-70%. CONCLUSIONS: Most studies using HDCT/ASCT as consolidation regimen showed improved survival benefits compared to CC. Randomized controlled studies are needed to determine true clinical benefits of HDCT followed by ASCT in patients with RES.
Sister Chromatid Exchange and Genomic Instability in Soft Tissue Sarcomas: Potential Implications for Response to DNA-Damaging Treatments.
Salawu A, Wright K, Al-Kathiri A … +3 more , Wyld L, Reed M, Sisley K
Sarcoma · 2018 · PMID 29853780 · Full text
Sarcomas are rare heterogeneous malignancies of mesenchymal origin characterised by complex karyotypes but no specific abnormalities. Recurrence is common, and metastatic disease carries poor survival despite standard DN...
Sarcomas are rare heterogeneous malignancies of mesenchymal origin characterised by complex karyotypes but no specific abnormalities. Recurrence is common, and metastatic disease carries poor survival despite standard DNA-damaging radiotherapy or chemotherapy. DNA double-strand breaks (DSBs) are either repaired by mechanisms such as homologous recombination (HR) or result in cell death by apoptosis. Endogenous H2AX formation and SCE formation are early and late events, respectively, and their levels are considered surrogate measures of genomic instability. Combined H2AX and SCE analysis was used to evaluate endogenous DNA DSB levels (and their subsequent repair) in 9 primary sarcoma cell lines and compared with well-established commercial lines. All the sarcoma cell lines had elevated H2AX and SCE levels, but there was no correlation between the DNA DSB frequency and subsequent SCE. Typically, radioresistant osteosarcoma cells had relatively low H2AX frequency but high SCE counts suggestive of efficient DNA repair. Conversely, liposarcoma cells derived from a radiosensitive tumour had high H2AX but relatively lower SCE levels that may imply inefficient DNA DSB repair. To our knowledge, this is the first report that correlates H2AX and SCE levels in primary sarcoma cell lines and may provide insight into potential response to DNA-damaging treatments.
High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature.
Bailey K, Roth M, Weiser D … +1 more , Gill J
Sarcoma · 2018 · PMID 29853779 · Full text
PURPOSE: Desmoplastic small round cell tumor (DSRCT) is a rare cancer that predominantly affects males averaging 21 years of age at the time of diagnosis. We describe four cases from our institution and place them within...
PURPOSE: Desmoplastic small round cell tumor (DSRCT) is a rare cancer that predominantly affects males averaging 21 years of age at the time of diagnosis. We describe four cases from our institution and place them within the context of a comprehensive review of the literature. PATIENTS AND METHODS: Study population included any patient who received treatment at Children's Hospital at Montefiore (CHAM) with histologic diagnosis of DSRCT. A search of the electronic databases PubMed, Cochrane Central Register of Controlled Trials, MEDLINE, and EMBASE for the terms "desmoplastic" AND "small" AND "round" AND "cell" AND "tumor" was performed. RESULTS: One CHAM patient died of disease at 39 months, one patient has relapsed disease at 40 months, and two patients have no evidence of disease at 60 and 91 months. In the literature review, the 3-year OS was 36% and 5-year OS was 13%. There was a statistically significant difference in OS between no transplant and SCT in remission (=0.004); however, there was no difference between no transplant and SCT not in remission (=0.23). CONCLUSION: Given the poor prognosis in DSRCT, this study supports further prospective research into the possible benefit of consolidation of autologous SCT in patients with DSRCT who are in remission, with the alternative inference that these patients in remission may fare well without SCT. Our retrospective review of the literature does not support SCT for patients who are not in remission.
High-Grade Osteosarcoma of the Foot: Presentation, Treatment, Prognostic Factors, and Outcome of 23 Cooperative Osteosarcoma Study Group COSS Patients.
Schuster AJ, Kager L, Reichardt P … +12 more , Baumhoer D, Csóka M, Hecker-Nolting S, Lang S, Lorenzen S, Mayer-Steinacker R, von Kalle T, Kevric M, Werner M, Windhager R, Wirth T, Bielack SS
Sarcoma · 2018 · PMID 29853778 · Full text
Osteosarcoma of the foot is a very rare presentation of a rare tumor entity. In a retrospective analysis, we investigated tumor- and treatment-related variables and outcome of patients registered in the Cooperative Osteo...
Osteosarcoma of the foot is a very rare presentation of a rare tumor entity. In a retrospective analysis, we investigated tumor- and treatment-related variables and outcome of patients registered in the Cooperative Osteosarcoma Study Group (COSS) database between January 1980 and April 2016 who suffered from primary high-grade osteosarcoma of the foot. Among the 23 eligible patients, median age was 32 years (range: 6-58 years), 10 were female, and 13 were male. The tarsus was the most commonly affected site (=16). Three patients had primary metastases. All patients were operated: 5 underwent primary surgery and 18 received surgery following preoperative chemotherapy. In 21 of the 23 patients, complete surgical remission was achieved. In 4 of 17 patients, a poor response to neoadjuvant chemotherapy was observed in the resected primary tumors. Median follow-up was 4.2 years (range: 0.4-18.5). At the last follow-up, 15 of the 23 patients were alive and 8 had died. Five-year overall and event-free survival estimates were 64% (standard error (SE) 12%) and 54% (SE 13%), which is similar to that observed for osteosarcoma in general. Event-free and overall survival correlated with primary metastatic status and completeness of surgery. Our findings show that high-grade osteosarcoma in the foot has a similar outcome as osteosarcoma of other sites.
Outcome for Advanced or Metastatic Soft Tissue Sarcoma of Nonextremities Treated with Doxorubicin-Based Chemotherapy: A Retrospective Study from a Single Cancer Institution.
Marshall S, Nakano K, Sugiura Y … +4 more , Taira S, Ono M, Tomomatsu J, Takahashi S
Sarcoma · 2018 · PMID 29849480 · Full text
BACKGROUND: Doxorubicin is the key drug for treatment of advanced soft tissue sarcoma (STS). The appropriate dosage of doxorubicin, regarding monotherapy or the role of combination therapy, is unclear. METHODS: We retros...
BACKGROUND: Doxorubicin is the key drug for treatment of advanced soft tissue sarcoma (STS). The appropriate dosage of doxorubicin, regarding monotherapy or the role of combination therapy, is unclear. METHODS: We retrospectively reviewed patients with advanced or metastatic STS of nonextremities who were treated with doxorubicin-based chemotherapies in our institution. Time to treatment failure (TTF), overall survival (OS), overall response, and prognostic factors for OS were evaluated. RESULTS: Seventy-five patients were enrolled. The median TTF was 4.7 months, and the median OS was 20.1 months. The overall response rate was 20%. Doses of doxorubicin monotherapy did not show significant difference either in TTF or in OS. There were no significant differences in OS between combination therapy and monotherapy, but the TTF with combination therapy was better than monotherapy. The overall response for combination therapy indicated a better response rate. Less number of involved organs, no bulky mass, and a normal CRP level were independent favorable prognostic factors for OS. CONCLUSIONS: Combination therapy showed better response and TTF than monotherapy but did not show better OS. Possible prognostic factors for OS were indicated. This retrospective study was approved by the institutional review board. This trial is registered with UMIN000028787.
Improving Long-Term Outcomes for Patients with Extra-Abdominal Soft Tissue Sarcoma Regionalization to High-Volume Centers, Improved Compliance with Guidelines or Both?
Bagaria SP, Chang YH, Gray RJ … +3 more , Ashman JB, Attia S, Wasif N
Sarcoma · 2018 · PMID 29849479 · Full text
INTRODUCTION: Optimization of outcomes of extra-abdominal STS is not clearly understood. We sought to determine whether hospital surgical volume and adherence to NCCN guidelines, or both, are associated with outcomes in...
INTRODUCTION: Optimization of outcomes of extra-abdominal STS is not clearly understood. We sought to determine whether hospital surgical volume and adherence to NCCN guidelines, or both, are associated with outcomes in the treatment of extra-abdominal soft tissue sarcoma (STS). METHODS: The National Cancer Database (NCDB) was queried for patients undergoing surgery for extra-abdominal STS diagnosed from 2003 to 2007. Mean annual hospital volume for STS surgery was divided into volume terciles (1T ≤3, 2T 4-10, and 3T ≥11 cases/year). Adherence to NCCN guidelines was determined. Primary outcome was overall survival. RESULTS: Our study population consisted of 13,684 patients with a median age of 56 years. 3T hospitals were more likely to adhere to NCCN guidelines for stage III patients (63% versus 47%; ≤ 0.001) than 1T hospitals. On multivariable analysis, adherence to NCCN guidelines was associated with improved survival (HR = 0.79, CI 0.73-0.87; < 0.001), but hospital volume was not (3T versus 1T: HR = 0.92, CI 0.82-1.02; =0.12). Five-year overall survival was comparable for compliant groups at 1T, 2T, and 3T hospitals (72%, 72.4%, and 72.6%, resp.). 3T hospitals were not associated with a lower risk of 30-day mortality (OR 0.70, 95% CI 0.44-1.11) compared to 1T hospitals but did have a higher R0 resection rate (OR 1.43, 95% CI 1.32-1.54). CONCLUSIONS: Adherence to NCCN guidelines, irrespective of hospital volume, is associated with improved overall survival for patients with extra-abdominal STS. High-volume hospitals more often adhere to guidelines, but low-volume hospitals that follow national guidelines may achieve comparable outcomes.
Epidemiology of Adult Soft-Tissue Sarcomas in Germany.
Saltus CW, Calingaert B, Candrilli S … +5 more , Lorenzo M, D'yachkova Y, Otto T, Wagner U, Kaye JA
Sarcoma · 2018 · PMID 29849478 · Full text
We conducted a retrospective cohort study using data compiled from the regional German cancer registries by the Centre for Cancer Registry Data (ZfKD) at the Robert Koch Institut (RKI) to describe the epidemiology of adu...
We conducted a retrospective cohort study using data compiled from the regional German cancer registries by the Centre for Cancer Registry Data (ZfKD) at the Robert Koch Institut (RKI) to describe the epidemiology of adult soft-tissue sarcomas (STS) in Germany in 2003-2012, focusing on advanced STS. We identified 33,803 incident adult cases of STS (other than the Kaposi sarcoma and gastrointestinal stromal tumors). The incidence of STS was 6.05 (95% confidence interval (CI), 5.82-6.29) per 100,000 in 2012 (4,079 cases). During 2003-2012, the most common histologic categories were leiomyosarcoma (19%), liposarcoma (16%), and STS not otherwise specified (14%). The overall STS-specific mortality rate in 2012 was 2.31 (95% CI, 2.06-2.57) per 100,000, and the median overall survival from initial diagnosis was 5.83 (95% CI, 5.50-6.08) years. Using STS mortality rates as a proxy for incidence of advanced STS in Germany and applying the age- and sex-specific rates to the corresponding German population, we estimated that 1,581 incident adult advanced STS cases occurred in Germany in 2012. Our findings contribute to a refined understanding of the population burden of STS in Germany, including the number of patients with advanced STS who may be candidates for systemic treatment.
About
- Frequency
- Sun
- Papers found
- 200
- RSS feed
- Subscribe