Sarcoma[JOURNAL]
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200 papers
Psychosocial Distress Scores and Needs among Newly Diagnosed Sarcoma Patients: A Provincial Experience.
Srikanthan A, Leung B, Shokoohi A … +3 more , Smrke A, Bates A, Ho C
Sarcoma · 2019 · PMID 31354383 · Full text
INTRODUCTION: Information on the psychosocial distress and needs of sarcoma patients at diagnosis is sparse. The Canadian Problem Checklist (CPC) and Psychosocial Screen for Cancer-Revised (PSSCAN-R) are validated tools...
INTRODUCTION: Information on the psychosocial distress and needs of sarcoma patients at diagnosis is sparse. The Canadian Problem Checklist (CPC) and Psychosocial Screen for Cancer-Revised (PSSCAN-R) are validated tools to identify cancer patients' distress and are administered to all new patients referred to BC Cancer prior to their consultation. We used the CPC and PSSCAN-R to understand sarcoma patients' needs at the initial oncology consultation in British Columbia, Canada. MATERIALS AND METHODS: All sarcoma patients who completed the CPC and PSSCAN-R within 6 months of diagnosis between 2011 and 2016 were included. The retrospective chart review identified baseline demographics: age, performance status, disease location, resectability, and histology. Analysis was conducted using descriptive statistics, chi-squared test, Fisher's exact test, and Kaplan-Meier method. RESULTS: 413 sarcoma patients were identified. The majority of patients were over the age of 40 (83.3%) with ECOG performance status 0-1 (82.6%) and lower extremity tumors (55.4%). The most common diagnoses were liposarcoma 21.3%, undifferentiated pleomorphic sarcoma 12.1%, and myxofibrosarcoma 11.1%. At the initial consultation, 42.6% of patients were deemed resectable, 8.5% unresectable/metastatic, and 48.9% required further staging investigations. The top three patient-reported distress symptoms were feeling tense and unable to relax (50%), feeling nervous and shaky (48%), and experiencing repetitive and scary thoughts (42%). 38% of patients had subclinical/clinical anxiety symptoms, and 21% of patients had subclinical/clinical depression symptoms. 5% of patients expressed suicidal ideation. The top three concerns/needs were understanding of illness/treatment (45.5%), fear/worries (45.3%), and worry about family (23%). No differences in overall survival were identified for patients displaying symptoms of depression or anxiety versus no symptoms. DISCUSSION: Up to 45% of sarcoma patients experience some form of psychological distress at disease presentation. Patients desire information about their diagnosis and treatment. Tailored interventions to individual psychological comorbidity and improved patient education resources would be beneficial.
How Effective Are Noninvasive Tests for Diagnosing Malignant Peripheral Nerve Sheath Tumors in Patients with Neurofibromatosis Type 1? Diagnosing MPNST in NF1 Patients.
Schwabe M, Spiridonov S, Yanik EL … +6 more , Jennings JW, Hillen T, Ponisio M, McDonald DJ, Dehdashti F, Cipriano CA
Sarcoma · 2019 · PMID 31354382 · Full text
BACKGROUND: Distinguishing between benign and malignant peripheral nerve sheath tumors (MPNSTs) in neurofibromatosis 1 (NF1) patients prior to excision can be challenging. How can MPNST be most accurately diagnosed using...
BACKGROUND: Distinguishing between benign and malignant peripheral nerve sheath tumors (MPNSTs) in neurofibromatosis 1 (NF1) patients prior to excision can be challenging. How can MPNST be most accurately diagnosed using clinical symptoms, magnetic resonance imaging (MRI) findings (tumor size, depth, and necrosis), positron emission tomography (PET) measures (SUV, SUV, SUV/SUV, and qualitative scale), and combinations of the above? . All NF1 patients who underwent PET imaging at our institution (January 1, 2007-December 31, 2016) were included. Medical records were reviewed for clinical findings; MR images and PET images were interpreted by two fellowship-trained musculoskeletal and nuclear medicine radiologists, respectively. Receiver operating characteristic (ROC) curves were created for each PET measurement; the area under the curve (AUC) and thresholds for diagnosing malignancy were calculated. Logistic regression determined significant predictors of malignancy. RESULTS: Our population of 41 patients contained 34 benign and 36 malignant tumors. Clinical findings did not reliably predict MPNST. Tumor depth below fascia was highly sensitive; larger tumors were more likely to be malignant but without a useful cutoff for diagnosis. Necrosis on MRI was highly accurate and was the only significant variable in the regression model. PET measures were highly accurate, with AUCs comparable and cutoff points consistent with prior studies. A diagnostic algorithm was created using MRI and PET findings. CONCLUSIONS: MRI and PET were more effective at diagnosing MPNST than clinical features. We created an algorithm for preoperative evaluation of peripheral nerve sheath tumors in NF1 patients, for which additional validation will be indicated.
Predictors of Disease-Free and Overall Survival in Retroperitoneal Sarcomas: A Modern 16-Year Multi-Institutional Study from the United States Sarcoma Collaboration (USSC).
Schwartz PB, Vande Walle K, Winslow ER … +15 more , Ethun CG, Tran TB, Poultsides G, Tseng J, Roggin K, Grignol V, Howard JH, Krasnick BA, Fields RC, Mogal H, Clarke CN, Senehi R, Votanopoulos K, Cardona K, Abbott DE
Sarcoma · 2019 · PMID 31281208 · Full text
BACKGROUND: Retroperitoneal sarcomas (RPS) comprise approximately 15% of all soft-tissue sarcomas and frequently associated with significant morbidity and as little as 30% 5-year survival. Here, we provide a large, conte...
BACKGROUND: Retroperitoneal sarcomas (RPS) comprise approximately 15% of all soft-tissue sarcomas and frequently associated with significant morbidity and as little as 30% 5-year survival. Here, we provide a large, contemporary, and multi-institutional experience to determine which tumor, patient, and treatment characteristics are associated with long-term outcomes in RPS. METHODS: 571 patients with primary RPS were identified from the United States Sarcoma Collaboration (USSC). RPS patients who underwent resection from January 2000 to April 2016 were included with patient, tumor, and treatment-specific variables investigated as independent predictors of survival. Survival analyses for disease-free and overall survival were conducted using Kaplan-Meier and Cox proportional hazards model methods. RESULTS: The study cohort was 55% female, with a median age of 58.9 years (IQR: 48.6-70.0). The most common tumor histiotypes were liposarcoma (34%) and leiomyosarcoma (28%). Median follow-up was 30.6 months (IQR: 11.2-60.4). Median disease-free survival was 35.3 months (95% CI: 27.6-43.0), with multivariate predictors of poorer disease-free survival including higher grade tumors, nodal-positive disease, and multivisceral resection. Median overall survival was 81.6 months (95% CI: 66.3-96.8). Multivariate predictors of shorter overall survival included higher grade tumors, nodal-positive and multifocal disease, systemic chemotherapy, and grossly positive margins (2) following resection. CONCLUSIONS: The strongest predictors of disease-free and overall survival are tumor-specific characteristics, while surgical factors are less impactful. Nonsurgical therapies are not associated with improved outcomes despite persistent interest and utilization. Complete macroscopic resection (0/1) remains a persistent potentially modifiable risk factor associated with improved overall survival in patients with retroperitoneal sarcomas.
Survival and Cost-Effectiveness of Trabectedin Compared to Ifosfamide Monotherapy in Advanced Soft Tissue Sarcoma Patients.
Verboom MC, Gelderblom H, Kerst JM … +5 more , Steeghs N, Reyners AKL, Sleijfer S, van der Graaf WTA, van den Hout WB
Sarcoma · 2019 · PMID 31281207 · Full text
Trabectedin and ifosfamide are among the few cytostatic agents active in advanced soft tissue sarcomas (STSs). Trabectedin is most potent against so-called L-sarcomas (leiomyosarcoma and liposarcoma). The survival gain a...
Trabectedin and ifosfamide are among the few cytostatic agents active in advanced soft tissue sarcomas (STSs). Trabectedin is most potent against so-called L-sarcomas (leiomyosarcoma and liposarcoma). The survival gain and cost-effectiveness of these agents in a second-line setting were analysed in the setting of advanced STS after failure of anthracyclines. A prospective observational trial had previously been performed to assess the use of trabectedin in a Dutch real-world setting. Data on ifosfamide monotherapy were acquired from previous studies, and an indirect comparison of survival was made. A state-transition economic model was constructed, in which patients could be in mutually exclusive states of being preprogression, postprogression, or deceased. The costs and quality-adjusted life years (QALYs) for both treatments were assessed from a Dutch health-care perspective. Separate analyses for the group of L-sarcomas and non-L-sarcomas were performed. Trabectedin treatment resulted in a median progression-free survival of 5.2 months for L-sarcoma patients, 2.0 months for non-L-sarcoma patients, and a median overall survival of 11.8 and 6.0 months, respectively. For L-sarcoma patients, trabectedin offered an increase of 0.368 life years and 0.251 QALYs compared to ifosfamide and €20,082 in additional costs, for an incremental cost-effectiveness ratio (ICER) of €80,000 per QALY gained. In the non-L-sarcoma patients, trabectedin resulted in 0.413 less life years and 0.266 less QALYs, at the increased cost of €4,698. The difference in survival between drugs and the acquisition costs of trabectedin were the main influences in these models. Trabectedin was shown to have antitumour efficacy in advanced L-sarcoma. From a health economics perspective, the costs per QALY gained compared to ifosfamide monotherapy that may be acceptable, considering what is currently regarded as acceptable in the Netherlands.
Patient and Tumour Characteristics of Adult Head and Neck Soft Tissue Sarcomas: A Systematic Review and Meta-Analysis.
Andersen S, Mann H, Krarup-Hansen A … +2 more , Lajer CB, Grønhøj C
Sarcoma · 2019 · PMID 31263380 · Full text
BACKGROUND: Head and neck soft tissue sarcomas (HNSTS) constitute a rare and heterogeneous cancer entity. Management remains a challenge due to the rarity and varied biological behaviour among various subtypes. This syst...
BACKGROUND: Head and neck soft tissue sarcomas (HNSTS) constitute a rare and heterogeneous cancer entity. Management remains a challenge due to the rarity and varied biological behaviour among various subtypes. This systematic review examines the characteristics of tumours and patients with HNSTS. MATERIALS AND METHODS: A systematic literature review and meta-analysis were performed using the electronic databases PubMed and Embase. Eight eligible studies were identified, and 13 variables were extracted from each study including 5-year overall survival (OS) rate and 5-year disease-free survival (DFS) rate. RESULTS: We identified eight studies ( = 1,120 patients; 739 males (66%)) from six different countries). In total, 24 histological subtypes were found, and 20% of the sarcomas ( = 215) could not be subclassified. 607 sarcomas (57%) were <5 cm in diameter, and 945 sarcomas (84%) were grade 3. 1,059 patients (90%) underwent surgery. Estimated 5-year OS was 74% (95% CI; 0.63-0.84%) and 5-year DFS was 56% (95% CI; 38-74%). CONCLUSION: HNSTS holds a relative poor prognosis possibly explained by the heterogeneity of the disease. Treatment of HNSTS has shown to be highly diverse, underlining the importance of uniformed treatment guidelines in order to achieve improved survival outcomes.
Use of Healthcare Services Two Years before Diagnosis in Danish Sarcoma Patients, 2000-2013.
Rædkjær M, Maretty-Kongstad K, Baad-Hansen T … +4 more , Safwat A, Petersen MM, Keller J, Vedsted P
Sarcoma · 2019 · PMID 31205448 · Full text
BACKGROUND: Sarcoma is a rare type of cancer with nonspecific symptoms and uncertain aetiology. Thus, timely diagnosis of sarcomas is a clinical challenge. The aim of this study was to investigate the use of healthcare s...
BACKGROUND: Sarcoma is a rare type of cancer with nonspecific symptoms and uncertain aetiology. Thus, timely diagnosis of sarcomas is a clinical challenge. The aim of this study was to investigate the use of healthcare services 24 months preceding a sarcoma diagnosis compared to a matched cohort. MATERIALS AND METHODS: The study was a retrospective, population-based, matched cohort registry-study. Patients with sarcoma in Denmark in 2000-2013 were identified in the Danish Sarcoma Registry ( = 2167) and matched 1 : 10 on gender, age, and listed general practice. Using a binomial regression model, incidence rate ratios were calculated for face-to-face contacts in general practice, inpatient and outpatient visits, surgery, paraclinical examinations, and diagnostic imaging. Analyses were stratified for sarcoma subtypes, grade, stage, gender, and presence of comorbidity. RESULTS: The sarcoma patients had significantly increased incidence rate ratios in use of healthcare services compared to the matched cohort a year before their diagnoses. An increase in consultation rates was seen 11 months before diagnosis for inpatient visits, 9 months before diagnosis in general practice and outpatient visits, 8 months before diagnosis for paraclinical examinations, and 4 and 3 months before diagnosis for diagnostic imaging and surgery, respectively. There were no clinical significant differences in length of increased consultation rates between sarcoma type, stage, and grade. Sarcoma patients with comorbidity had persistently higher consultation rates compared to patients without comorbidity. CONCLUSIONS: The use of healthcare services among sarcoma patients increased several months before diagnosis in all healthcare sectors. The results reveal a diagnostic time window and a potential to refer, diagnose, and treat sarcoma patients in a timelier manner.
Role of Radiation Therapy in Adult Extraskeletal Ewing's Sarcoma Patients Treated with Chemotherapy and Surgery.
Saiz AM, Gingrich AA, Canter RJ … +4 more , Kirane AR, Monjazeb AM, Randall RL, Thorpe SW
Sarcoma · 2019 · PMID 31178655 · Full text
Radiation therapy (RT) is advocated in the multimodal treatment of high-grade soft tissue sarcoma (STS), but its role may be less clear in chemotherapy-sensitive STS such as extraskeletal Ewing sarcoma (EES). The purpose...
Radiation therapy (RT) is advocated in the multimodal treatment of high-grade soft tissue sarcoma (STS), but its role may be less clear in chemotherapy-sensitive STS such as extraskeletal Ewing sarcoma (EES). The purpose of this study was to determine the role of RT on overall survival (OS) in localized EES adult patients treated with chemotherapy and surgery. Adult patients diagnosed with EES and reported to the National Cancer Database from 2004 to 2014 were evaluated. All patients were treated with surgical resection. Patient demographics, tumor characteristics, treatments received, resection margins, and survival were examined for the 232 patients identified. Using multivariate analysis and Cox proportional hazard analysis, predictors of OS were determined. In the overall cohort, 40 percent of patients received RT and 78 percent received chemotherapy, with 31 percent receiving both. The addition of RT to the patients receiving surgery + chemotherapy did not improve OS ( < 0.05). Twenty-four percent of patients who achieved R0 resection after surgery still received RT without any improvement in OS. Patients treated at community cancer centers were more likely to receive additional RT compared with Comprehensive Cancer Centers ( < 0.05). In adult EES patients with localized disease treated with chemotherapy and surgery, the addition of RT does not improve overall survival.
Reconstructive Challenges of Proximal Ulnar Bone Tumors: Our Experience with Biological Osteoarticular Reconstruction Using Extracorporeal Irradiation and Reimplantation.
Gundavda MK, Agarwal MG, Reddy R
Sarcoma · 2019 · PMID 31110466 · Full text
INTRODUCTION: Limb salvage surgery following proximal ulna resection poses a challenge in reconstruction of the complex elbow anatomy. Various reconstruction methods described offer inadequate restoration of function and...
INTRODUCTION: Limb salvage surgery following proximal ulna resection poses a challenge in reconstruction of the complex elbow anatomy. Various reconstruction methods described offer inadequate restoration of function and stability. Following resection of proximal ulna tumors, we aimed to restore the joint using the resected osteochondral segment of proximal ulna treated with extracorporeal irradiation and reimplantation. QUESTIONS/PURPOSES: (1) Does irradiated osteoarticular autograft reconstruction for proximal ulna allow anatomical joint restoration and what are the oncological and functional outcomes? (2) Is there evidence of graft-related complications or osteoarthritis at a minimum of 2 years follow-up with irradiated osteoarticular autografts for the proximal ulna? (3) How does our method of reconstruction fare as compared to reported reconstruction options in the literature? . 3 patients with primary bone tumors involving the proximal ulna underwent limb salvage surgery with en bloc resection and reconstruction using the resected bone after treating it with extracorporeal irradiation of 50 Gy. Minimum follow-up of 2 years was considered for assessment of final outcomes. Radiographs were assessed for bony recurrence, union across osteotomy junction, and signs of joint arthritis. Functional outcome measures included range of movement, muscle power testing, and functional and disability scores. RESULTS: 2 complete and 1 partial olecranon involving proximal ulnar resections were performed for three cases of Ewing's sarcoma in 2 males and 1 female. Follow-up ranged from 28 to 42 months, and all patients continue to remain disease free. All 3 patients have achieved full range of flexion-extension and pronosupination movement in comparison to the contralateral side. Muscle power for flexion and extension was 5/5. MSTS score: 100% (30/30); MEPS score: 100; and DASH score: zero were achieved for all patients. Union across osteotomy junctions at median follow-up of 8 months without need for intervention/bone grafting was achieved. No complications related to joint laxity/subluxation, infection, graft fracture, or implant failure was noted. None of the patients have clinical or radiological signs of joint arthritis across the irradiated articulation. CONCLUSION: Biological restoration of elbow anatomy using osteoarticular irradiated graft for proximal ulna reconstruction offers great joint stability and functional outcomes. Although the potential risks of infection and graft failure need to be considered, reconstruction with the size-matched radiated autograft eliminates donor site morbidity, offers a low-cost alternative to endoprosthesis, and provides outcomes superior to any other methods of reconstruction as analyzed from the literature.
Patient Perceptions of the Impact of Treatment (Surgery and Radiotherapy) for Soft Tissue Sarcoma.
Hewitt L, Powell R, Zenginer K … +4 more , Coyle C, Murray H, Cooper L, Gregory J
Sarcoma · 2019 · PMID 31049021 · Full text
BACKGROUND AND OBJECTIVES: Treatment for soft tissue sarcoma (STS) is challenging for patients. This study aimed to gain an in-depth understanding of patients' experiences of STS treatment, including whether the sequence...
BACKGROUND AND OBJECTIVES: Treatment for soft tissue sarcoma (STS) is challenging for patients. This study aimed to gain an in-depth understanding of patients' experiences of STS treatment, including whether the sequence of treatment (preoperative or postoperative radiotherapy) influences patient perceptions. METHODS: Face-to-face semi-structured interviews were conducted with nineteen patients who had been treated for STS with surgery and radiotherapy between 2011 and 2016. Topics discussed included perceptions of treatment, social support, and coping mechanisms. Qualitative, inductive, thematic analysis was conducted and structured using the Framework approach. RESULTS: Treatment sequence itself did not appear to cause concern, but uncertainty regarding treatment and side effects could negatively impact participants. Social relationships and individual coping strategies influenced participants' experiences of treatment. CONCLUSIONS: Participants' perceptions of the treatment process varied; the experience was highly individual. It is important to ensure individual psychosocial and information needs are met. In particular, the removal of uncertainty regarding treatment is important in supporting patients undergoing treatment for soft tissue sarcoma.
and Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review.
Bode-Lesniewska B, Fritz C, Exner GU … +2 more , Wagner U, Fuchs B
Sarcoma · 2019 · PMID 31049020 · Full text
The spectrum of mesenchymal tumors associated with rearrangements of the gene has been growing in recent years due to progress in molecular detection techniques. Originally identified as the gene involved in the pathoge...
The spectrum of mesenchymal tumors associated with rearrangements of the gene has been growing in recent years due to progress in molecular detection techniques. Originally identified as the gene involved in the pathogenesis of Ewing sarcoma, the gene is now known to be rearranged in diverse clinical and histopathological entities. The gene is one of the many translocation partners of in gene fusions in a morphologically typical, albeit rare, subgroup of mesenchymal tumors. Little is known about the clinical characteristics of tumors containing gene rearrangements since most of the few reports published describe molecular rather than clinical aspects. In the current study, we report three patients with tumors carrying the gene translocation, including one rare primary tumor of soft tissues. Another patient with a benign-appearing bone tumor with a unique gene translocation is described. In various mesenchymal tumors (e.g., myxoid/round cell liposarcoma, low-grade fibromyxoid sarcoma, or angiomatoid fibrous histiocytoma), the gene, as a member of the TET family, may be alternatively rearranged instead of the gene without any noticeable influence on the microscopical appearance or clinical outcome. This fact seems not to apply to mesenchymal tumors with the involvement of the gene because both in our experience and according to the extensive literature review, they have different properties on the morphological and molecular level. Both and fusion-carrying tumors do not show microscopical or clinical features of Ewing sarcoma.
Primary Cardiac Sarcoma: A Rare, Aggressive Malignancy with a High Propensity for Brain Metastases.
Siontis BL, Zhao L, Leja M … +5 more , McHugh JB, Shango MM, Baker LH, Schuetze SM, Chugh R
Sarcoma · 2019 · PMID 30962762 · Full text
INTRODUCTION: Primary cardiac sarcoma (PCS) has a poor prognosis compared to other sarcomas due to late presentation, challenging resection, incidence of metastases, and limited efficacy of systemic therapies. METHODS: A...
INTRODUCTION: Primary cardiac sarcoma (PCS) has a poor prognosis compared to other sarcomas due to late presentation, challenging resection, incidence of metastases, and limited efficacy of systemic therapies. METHODS: A medical record search engine was queried to identify patients diagnosed with PCS from 1992 to 2017 at the University of Michigan. RESULTS: Thirty-nine patients with PCS had a median age of 41 years (range 2-77). Common histologies were angiosarcoma (AS, 14), high-grade undifferentiated pleomorphic sarcoma (UPS, 10), and leiomyosarcoma (LMS, 5). Sites of origin were left atrium (18), right atrium (16), and pericardium (5). AS was the most common right-sided tumor; UPS was more common on the left. Eighteen patients presented with metastases involving lung (10), bone (7), liver (5), and brain (4). Twenty-five patients underwent resection, achieving 3 R resections. Patients received a median of 2 (1-6) systemic therapies. Median overall survival (OS) was 12.1 months (range 0-79). Median OS was 14.0 months and 8.2 months in patients who did or did not undergo resection, respectively (=0.018). Brain metastases occurred in 12 (31%) patients, 9 (75%) of whom had left heart tumors, at a median of 8.5 months (range 0-75) from diagnosis. Median OS was 5.6 months (range 0-30) after the diagnosis of brain metastases. CONCLUSIONS: PCS portends a poor prognosis, because of difficulty in obtaining complete resection of sarcoma, advanced stage at diagnosis, and high risk of brain metastases. Providers should be aware of the increased risk of brain metastases and consider brain imaging at diagnosis and follow-up.
Evaluation of Planned versus Unplanned Soft-Tissue Sarcoma Resection Using PROMIS Measures.
Wilke BK, Cooper AR, Aratani AK … +3 more , Scarborough MT, Gibbs CP, Spiguel A
Sarcoma · 2019 · PMID 30956533 · Full text
BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) is a tool developed by the National Institutes of Health that allows comparisons across conditions or even the United States (U.S.) genera...
BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) is a tool developed by the National Institutes of Health that allows comparisons across conditions or even the United States (U.S.) general population. OBJECTIVES: Our purpose was to compare PROMIS outcomes between patients who underwent a planned resection to those who underwent an initial unplanned excision of their sarcoma followed by a definitive oncologic resection. We then compared these groups to the U.S. general population. METHODS: Eighty-five patients were included and were divided into those who underwent an initial planned resection (67) and unplanned excision (18). These patients were then further categorized based on the length of follow-up since their last surgery, either early (<12 months) or late (>12 months). RESULTS: We evaluated seven PROMIS domains and found no differences between patients who underwent planned resection versus those who underwent an initial unplanned excision followed by a wide resection of the previous wound bed. When compared to the U.S. population, both cohorts demonstrated significantly improved scores in several emotional health domains. CONCLUSIONS: Patients who undergo an unplanned excision followed by a definitive oncologic procedure have similar PROMIS scores compared to patients who undergo an initial planned resection.
A Critical Review of the Impact of Sarcoma on Psychosocial Wellbeing.
Storey L, Fern LA, Martins A … +8 more , Wells M, Bennister L, Gerrand C, Onasanya M, Whelan JS, Windsor R, Woodford J, Taylor RM
Sarcoma · 2019 · PMID 30911268 · Full text
BACKGROUND: Previous reviews of outcomes in specific sarcoma populations suggest patients have poor quality of life. In most of these reviews, there is a predominant focus on physical function rather than psychosocial ou...
BACKGROUND: Previous reviews of outcomes in specific sarcoma populations suggest patients have poor quality of life. In most of these reviews, there is a predominant focus on physical function rather than psychosocial outcome. The aim of this review was to describe the psychosocial impact of diagnosis and treatment on patients with all types of sarcoma. METHODS: Searches were conducted through six electronic databases for publications of any study design using a validated patient-reported outcome measure reporting the psychosocial impact in this population. RESULTS: Eighty-two studies fulfilled the inclusion criteria. Most (65%) were assessed of being of reasonable quality. The most common aspect of psychosocial wellbeing measured was quality of life (80%). Due to the heterogeneity of methods, outcomes, and populations, it was not possible to make definitive conclusions. It seems there is an improvement in the physical aspects of quality of life over time but not in psychosocial function or mental health. There was no change in mental health scores, but patients reported an improvement in adjusting to normal life. There are no differences according to the type of surgery patients receive, and psychosocial outcomes tend to be poorer than the general population. There is no consistency in identifying the factors that predict/influence psychosocial wellbeing. CONCLUSION: The published literature does not provide a clear understanding of the impact of sarcoma diagnosis and treatment on psychosocial wellbeing. Instead, the review demonstrates a need for well-designed studies in this area and a more consistent approach to the measurement of patient-reported outcomes, which include psychosocial domains. Recommendations for future research have been proposed.
Adjuvant Chemotherapy in Uterine Leiomyosarcoma: Trends and Factors Impacting Usage.
Patel D, Handorf E, von Mehren M … +2 more , Martin L, Movva S
Sarcoma · 2019 · PMID 30881199 · Full text
OBJECTIVES: The benefit of adjuvant chemotherapy in patients with localized uterine leiomyosarcoma (LMS) remains unclear due to a lack of randomized studies and data only from small retrospective series to rely on. We so...
OBJECTIVES: The benefit of adjuvant chemotherapy in patients with localized uterine leiomyosarcoma (LMS) remains unclear due to a lack of randomized studies and data only from small retrospective series to rely on. We sought to identify factors associated with the administration of chemotherapy and to determine the trends in the usage of adjuvant chemotherapy in patients with nonmetastatic uterine LMS. METHODS: Patients diagnosed with nonmetastatic uterine LMS between 2004 and 2014 were identified from the National Cancer Database (NCDB). Multiple regression was used to determine factors with a significant impact on patient receipt of chemotherapy. Kaplan-Meier curves and the Cox model were used to determine the effect of adjuvant chemotherapy on overall survival (OS). RESULTS: 2,732 uterine LMS patients were identified. Patients older than 65 were less likely to receive chemotherapy than their younger counterparts. Patients with stage I or stage II cancer were less likely to receive chemotherapy, whereas individuals with positive regional lymph nodes and those who had received radiation were more likely. In this cohort, adjuvant chemotherapy had no significant impact on OS (HR, 1.04; 95% CI, 0.90-1.22; =0.5768). However, administration of chemotherapy significantly increased from 2004 to 2014 ( < 0.0001). CONCLUSIONS: Expected tumor characteristics such as higher stage of tumor were associated with receipt of chemotherapy. Although adjuvant chemotherapy demonstrated no benefit over observation on OS in patients with nonmetastatic LMS, the number of patients being treated with chemotherapy continued to increase from 2004 to 2014.
Free Gracilis Muscle Flap for Sarcoma Reconstruction: 19 Years of Clinical Experience.
Pedreira R, Calotta NA, Deune EG
Sarcoma · 2019 · PMID 30863198 · Full text
BACKGROUND: Sarcoma treatment necessitates high-dose chemoradiation therapy and wide surgical margins that create wounds that are difficult to reconstruct. Many techniques have been developed to cover these defects, orig...
BACKGROUND: Sarcoma treatment necessitates high-dose chemoradiation therapy and wide surgical margins that create wounds that are difficult to reconstruct. Many techniques have been developed to cover these defects, originating with muscle flaps such as the rectus abdominis and latissimus dorsi. The gracilis flap, which is best known in contemporary practice as a microneurovascular flap for functional reconstructions, is not usually considered a robust option for reconstruction after sarcoma extirpation. METHODS: We reviewed records of 22 patients (9 women) at our institution who underwent reconstructive surgery after sarcoma extirpation using gracilis flaps for soft-tissue coverage from 1998 to 2017. Neurotized gracilis flaps were excluded. The mean patient age was 51 years (range, 18-85 years), and mean length of follow-up was 53 months (range, 9-156 months). Patients had 7 tumor types, with fibrosarcomas and undifferentiated tumors being most common. There were 23 defects (mean size, 118 cm (range, 54-200 cm)). Defects were located most commonly in the foot and leg (=9 each), upper extremity (=4), and head and neck (=1). The primary outcome was the flap success rate. Secondary outcomes were rates of major complications (unplanned reoperations, infections requiring intravenous antibiotics, and amputations); minor complications (superficial infections, partial skin-graft loss, partial flap necrosis, fluid collections treated in the office, and cosmetic reoperations); and sarcoma recurrence. RESULTS: Twenty-one flaps (91%) survived. Six patients (27%) experienced a major complication, and 12 patients (54%) experienced a minor complication. There were 2 amputations, for a limb salvage rate of 91%. CONCLUSIONS: This series shows that the gracilis is well suited to covering large, compromised wounds across diverse anatomic features, which are the hallmark of sarcoma resections. The high rate of limb salvage and minimal donor-site morbidity further support the use of this flap as a first-line option for sarcoma reconstruction.
Analysis of the CDK4/6 Cell Cycle Pathway in Leiomyosarcomas as a Potential Target for Inhibition by Palbociclib.
Böhm MJ, Marienfeld R, Jäger D … +11 more , Mellert K, von Witzleben A, Brüderlein S, Wittau M, von Baer A, Schultheiss M, Mayer-Steinacker R, Rücker FG, Möller P, Bullinger L, Barth TFE
Sarcoma · 2019 · PMID 30804704 · Full text
Leiomyosarcoma (LMS) is characterized by high genomic complexity, and to date, no specific targeted therapy is available. In a genome-wide approach, we profiled genomic aberrations in a small cohort of eight primary tumo...
Leiomyosarcoma (LMS) is characterized by high genomic complexity, and to date, no specific targeted therapy is available. In a genome-wide approach, we profiled genomic aberrations in a small cohort of eight primary tumours, two relapses, and eight metastases across nine different patients. We identified CDK4 amplification as a recurrent alteration in 5 out of 18 samples (27.8%). It has been previously shown that the LMS cell line SK-LMS-1 has a defect in the p16 pathway and that this cell line can be inhibited by the CDK4 and CDK6 inhibitor palbociclib. For SK-LMS-1 we confirm and for SK-UT-1 we show that both LMS cell lines express CDK4 and that, in addition, strong CDK6 expression is seen in SK-LMS-1, whereas Rb was expressed in SK-LMS-1 but not in SK-UT-1. We confirm that inhibition of SK-LMS-1 with palbociclib led to a strong decrease in protein levels of Phospho-Rb (Ser780), a decreased cell proliferation, and G/G-phase arrest with decreased S/G fractions. SK-UT-1 did not respond to palbociclib inhibition. To compare these findings with patient tissue samples, a p16, CDK4, CDK6, and p-Rb immunohistochemical staining assay of a large LMS cohort (=99 patients with 159 samples) was performed assigning a potential responder phenotype to each patient, which we identified in 29 out of 99 (29.3%) patients. Taken together, these data show that CDK4/6 inhibitors may offer a new option for targeted therapy in a subset of LMS patients.
An Evaluation of PROMIS Health Domains in Sarcoma Patients Compared to the United States Population.
Wilke B, Cooper A, Scarborough M … +2 more , Gibbs CP, Spiguel A
Sarcoma · 2019 · PMID 30799982 · Full text
BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) is a patient-directed system that allows comparisons across medical conditions. With this tool, comparisons can now be made between rare c...
BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS) is a patient-directed system that allows comparisons across medical conditions. With this tool, comparisons can now be made between rare conditions, such as sarcomas, and more common ailments, of the United States general population. This allows comparisons between rare conditions, such as sarcomas, to more common ailments, or even the United States (US) general population. OBJECTIVES: Our purpose was to use PROMIS to compare outcomes in patients that had undergone resection of a nonmetastatic sarcoma to the US population. METHODS: One hundred thirty-eight patients were included in the analysis. These patients were divided into early (<2 years) and late follow-up (>2 years). RESULTS: We evaluated results from seven health domains and found significantly lower scores in the physical function and depression domains. These differences were present in both the early and late cohorts when compared to the US population. CONCLUSION: While physical function was found to be worse in the sarcoma cohorts, we observed significantly improved levels of depression in these patients when compared to the US population. This finding was maintained over time and is an important reminder that a patient's goals and desires change following a cancer diagnosis and must be taken into consideration when planning treatment and determining a successful outcome.
Using the Spleen as an Systemic Immune Barometer Alongside Osteosarcoma Disease Progression and Immunotherapy with -PD-L1.
Markel JE, Noore J, Emery EJ … +3 more , Bobnar HJ, Kleinerman ES, Lindsey BA
Sarcoma · 2018 · PMID 30651716 · Full text
Indications for immunotherapies are still unclear, and there is a great need for real-time patient immune status monitoring. In this study, we confirmed that the local and systemic immune profiles of an orthotopic osteos...
Indications for immunotherapies are still unclear, and there is a great need for real-time patient immune status monitoring. In this study, we confirmed that the local and systemic immune profiles of an orthotopic osteosarcoma model with or without luciferase transfection were statistically equivalent. Next, we used flow cytometry to describe systemic immune cell populations influenced by osteosarcoma disease progression. When compared to vehicle-inoculated sham mice, it was found that tumor-bearing mice had significant immunophenotype disturbances at approximately 11 weeks after inoculation (at which time 90% of primary tumor-bearing mice have fulminant pulmonary metastases). Percent populations of natural killer cells and T regulatory cells were increased in the spleens of tumor-bearing mice ( < 0.0083) compared to shams. Additionally, T lymphocytes from spleens of tumor-bearing mice showed increased Tim-3/PD-1 exhaustion status ( < 0.0083). There were also increases in the percent populations of myeloid cells and overall M1/M2 macrophage marker expression on tumor-bearing mice spleens versus controls ( < 0.00714). Finally, treatment with 20 g -PD-L1 decreased T-cell exhaustion back to sham status, with a corresponding increase in CTLA-4 expression on cytotoxic T cells in the majority of mice tested. Checkpoint inhibition also increased splenic monocyte maturation and returned macrophage M1/M2 marker expression back to sham status. These data suggest that cancer induces systemic immune dysregulation and that these changes may be elucidated and utilized for treatment purposes by sampling the systemic immune environment via the spleen. In addition, treatment with the checkpoint inhibitor -PD-L1 may neutralize but not overcome the systemic immunological changes induced by a progressing malignancy.
Radiation Therapy for Retroperitoneal Sarcomas: Influences of Histology, Grade, and Size.
Leiting JL, Bergquist JR, Hernandez MC … +6 more , Merrell KW, Folpe AL, Robinson SI, Nagorney DM, Truty MJ, Grotz TE
Sarcoma · 2018 · PMID 30631245 · Full text
Perioperative radiation therapy (RT) has been associated with reduced local recurrence in patients with retroperitoneal sarcomas (RPS); however, selection criteria remain unclear. We hypothesized that perioperative RT wo...
Perioperative radiation therapy (RT) has been associated with reduced local recurrence in patients with retroperitoneal sarcomas (RPS); however, selection criteria remain unclear. We hypothesized that perioperative RT would improve survival in patients with RPS and would be associated with pathological factors. The National Cancer Database (NCDB) from 2004 to 2012 was reviewed for patients with nonmetastatic RPS undergoing curative intent resection. Tumor size was dichotomized at 15 cm based on 8th edition American Joint Committee on Cancer (AJCC) staging. Patients with the highest comorbidity score were excluded. Unadjusted Kaplan-Meier and adjusted Cox proportional hazards modeling analyzed overall survival (OS). Multivariable logistic regression modeled margin positivity. A total of 2,264 patients were included; 727 patients (32.1%) had perioperative radiation in whom 203 (9.0%) had radiation preoperatively. Median (IQR) RPS size was 17.5 [11.0-27.0] cm. Histopathology was high grade in 1048 patients (43.7%). Multivariable analysis revealed that perioperative radiation was independently associated with decreased mortality (HR 0.72, 95% confidence intervals (CIs) 0.62-0.84, < 0.001), and preoperative RT was associated with reduced margin positivity (HR 0.72, 95% CI 0.53-0.97, =0.032). Stratified survival analysis showed that radiation was associated with prolonged median OS for RPS that were high-grade (64.3 vs. 43.6 months, < 0.001), less than 15 cm (104.1 vs. 84.2 months, =0.007), and leiomyosarcomatous (104.8 vs. 61.8 months, < 0.001). Perioperative radiation is independently associated with decreased mortality in patients with high-grade, less than 15 cm, and leiomyosarcomatous tumors. Preoperative radiation is independently associated with margin-negative resection. These data support the selective use of perioperative radiation in the multidisciplinary management of RPS.
A Cadaveric Comparative Study on the Surgical Accuracy of Freehand, Computer Navigation, and Patient-Specific Instruments in Joint-Preserving Bone Tumor Resections.
Bosma SE, Wong KC, Paul L … +2 more , Gerbers JG, Jutte PC
Sarcoma · 2018 · PMID 30538600 · Full text
Orthopedic oncologic surgery requires preservation of a functioning limb at the essence of achieving safe margins. With most bone sarcomas arising from the metaphyseal region, in close proximity to joints, joint-salvage...
Orthopedic oncologic surgery requires preservation of a functioning limb at the essence of achieving safe margins. With most bone sarcomas arising from the metaphyseal region, in close proximity to joints, joint-salvage surgery can be challenging. Intraoperative guidance techniques like computer-assisted surgery (CAS) and patient-specific instrumentation (PSI) could assist in achieving higher surgical accuracy. This study investigates the surgical accuracy of freehand, CAS- and PSI-assisted joint-preserving tumor resections and tests whether integration of CAS with PSI (CAS + PSI) can further improve accuracy. CT scans of 16 simulated tumors around the knee in four human cadavers were performed and imported into engineering software (MIMICS) for 3D planning of multiplanar joint-preserving resections. The planned resections were transferred to the navigation system and/or used for PSI design. Location accuracy (LA), entry and exit points of all 56 planes, and resection time were measured by postprocedural CT. Both CAS + PSI- and PSI-assisted techniques could reproduce planned resections with a mean LA of less than 2 mm. There was no statistical difference in LA between CAS + PSI and PSI resections (=0.92), but both CAS + PSI and PSI showed a significantly higher LA compared to CAS (=0.042 and =0.034, respectively). PSI-assisted resections were faster compared to CAS + PSI ( < 0.001) and CAS ( < 0.001). Adding CAS to PSI did improve the exit points, however not significantly. In conclusion, PSI showed the best overall surgical accuracy and is fastest and easy to use. CAS could be used as an intraoperative quality control tool for PSI, and integration of CAS with PSI is possible but did not improve surgical accuracy. Both CAS and PSI seem complementary in improving surgical accuracy and are not mutually exclusive. Image-based techniques like CAS and PSI are superior over freehand resection. Surgeons should choose the technique most suitable based on the patient and tumor specifics.
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