Sarcoma最新文献

Background: Synovial sarcoma can present morphologically in multiple forms, including biphasic and monophasic subtypes. As a result, the histological diagnosis can sometimes be challenging. Transducin-Like Enhancer 1 (TLE1) is a transcriptional corepressor that normally is involved in embryogenesis and hematopoiesis but is also expressed in certain tumors. This systematic review examines the potential role of TLE1 as a diagnostic biomarker for the synovial sarcoma. Materials and Methods. A literature review and meta-analysis were conducted using the electronic databases Pubmed, the Cochrane Library, and Google Scholar. Thirteen studies met our eligibility criteria and were selected for in-depth analysis.

Results: The mean sensitivity and specificity of TLE1 in detecting synovial sarcoma were 94% (95% CI 91%-97%) and 81% (95% CI 72%-91%), respectively, when all studies were aggregated together. The mean positive predictive value (PPV) of TLE1 was 75% (95% CI 62%-87%), whereas the negative predictive value (NPV) was 96% (95% CI 93%-98%).

Conclusion: TLE1 is a sensitive and specific marker for synovial sarcoma that can aid in its diagnosis. Due to its involvement in several relevant signaling pathways, TLE1 might have direct relevance to the pathophysiology of the disease.

Does reimplantation of sterilized tumor bone for reconstruction provide outcome benefits in intercalary osteosarcoma based on the potential immunogenic effect of reimplanted sterilized tumor tissue? Of 720 cases of surgically treated high-grade osteosarcoma patients treated at our institute from 2006 to 2013, 61 had predominantly diaphyseal disease. All patients were nonmetastatic at presentation. Patient and tumor characteristics, treatment details, and local recurrence-free, metastasis-free, and overall survival were compared for 24 patients who had reconstruction with sterilized tumor bone reimplantation vs 37 who did not. Both the groups were well matched in terms of baseline characteristics. Means were compared with the t-test, proportions with the chi-square test, and survival with the log-rank test. The Kaplan-Meier method was used to construct time to event curves. Cox proportional hazard regression modeling was employed for multivariate time to event analysis. Twenty-two had extracorporeal radiation and reimplantation (ECRT) with or without the vascularised fibula. Fifty-gray single dose was used in all cases. Two had pasteurization and reimplantation. Thirty seven had non-reimplantation reconstructions (including intercalary or osteoarticular endoprosthesis, pedicled bone grafts, rotation-plasty, and amputations). Five-year local recurrence-free survival was 85% for reimplantation and 97% for non-reimplantation groups (p=0.17). Five-year metastasis-free survival was 63% and 54%, respectively (p=0.44). Five-year overall survival was 70% and 58%, respectively (p=0.39). The data from this study did not demonstrate significantly better local recurrence-free, distant relapse-free, or overall survival benefit in the tumor bone reimplantation group.

Distinguishing lipoma from liposarcoma is challenging on conventional MRI examination. In case of uncertain diagnosis following MRI, further invasive procedure (percutaneous biopsy or surgery) is often required to allow for diagnosis based on histopathological examination. Radiomics and machine learning allow for several types of pathologies encountered on radiological images to be automatically and reliably distinguished. The aim of the study was to assess the contribution of radiomics and machine learning in the differentiation between soft-tissue lipoma and liposarcoma on preoperative MRI and to assess the diagnostic accuracy of a machine-learning model compared to musculoskeletal radiologists. 86 radiomics features were retrospectively extracted from volume-of-interest on T1-weighted spin-echo 1.5 and 3.0 Tesla MRI of 38 soft-tissue tumors (24 lipomas and 14 liposarcomas, based on histopathological diagnosis). These radiomics features were then used to train a machine-learning classifier to distinguish lipoma and liposarcoma. The generalization performance of the machine-learning model was assessed using Monte-Carlo cross-validation and receiver operating characteristic curve analysis (ROC-AUC). Finally, the performance of the machine-learning model was compared to the accuracy of three specialized musculoskeletal radiologists using the McNemar test. Machine-learning classifier accurately distinguished lipoma and liposarcoma, with a ROC-AUC of 0.926. Notably, it performed better than the three specialized musculoskeletal radiologists reviewing the same patients, who achieved ROC-AUC of 0.685, 0.805, and 0.785. Despite being developed on few cases, the trained machine-learning classifier accurately distinguishes lipoma and liposarcoma on preoperative MRI, with better performance than specialized musculoskeletal radiologists.

Soft tissue sarcomas (STS) constitute a heterogeneous group of rare solid tumors associated with significant morbidity and mortality. The evaluation and treatment of STS require a multidisciplinary team with extensive experience in the management of these types of tumors. National and international clinical practice guidelines for STS do not always provide answers to a great many situations that specialists have to contend with in their everyday practice. This consensus provides a series of specific recommendations based on available scientific evidence and the experience of a group of experts to assist in decision-making by all the specialists involved in the management of STS.

Background: Dedifferentiated chondrosarcomas (DDCSs) are highly malignant tumors with a dismal prognosis and present a significant challenge in clinical management.

Methods: In an IRB approved retrospective protocol, we identified 72 patients with DDCS treated at our institution between 1993 and 2017 and reviewed clinicopathological characteristics, treatment modalities, and outcomes to analyze prognostic factors.

Results: Femur (44.4%), pelvis (22.2%), and humerus (12.5%) were most commonly involved sites. Twenty-three patients (31.9%) presented with distant metastasis, and 3 (4.2%) of them also had regional lymph node involvement. The median overall survival (OS) was 13.9 months. On multivariate analysis, pathological fracture, larger tumor size, lymph node involvement, metastasis at diagnosis, extraosseous extension, and undifferentiated pleomorphic sarcoma component correlated with worse OS, whereas surgical resection and chemotherapy were associated with improved OS. For progression-free survival (PFS), pathological fracture and metastasis at diagnosis showed increased risk, while chemotherapy was associated with decreased risk. Among patients who received chemotherapy, doxorubicin and cisplatin were significantly associated with improved PFS but not OS. Among patients without metastasis at diagnosis, 17 (34.7%) developed local recurrence. Thirty-one (63.3%) developed distant metastases at a median interval of 18.1 months. On multivariate analysis, R1/R2 resection was related with local recurrence, while macroscopic dedifferentiated component was associated with distant metastasis.

Conclusions: The prognosis of DDCS is poor. Complete resection remains a significant prognostic factor for local control. Chemotherapy with doxorubicin and cisplatin seems to have better PFS. More prognostic, multicenter trials are warranted to further explore the effectiveness of chemotherapy in selected DDCS patients.

In today's era, limb salvage surgery is the procedure of choice and current standard of care in appropriately selected patients of bone sarcomas. For adequate oncologic clearance, preoperative evaluation of the extent of tumor is mandatory. The present study was done to compare measurements of bone sarcomas (osteosarcoma, Ewing's sarcoma, and chondrosarcoma) as determined by magnetic resonance imaging (MRI) with the histopathological extent seen on resected specimens. We prospectively evaluated 100 consecutive patients with a diagnosis of bone sarcoma who underwent limb salvage surgery between May 2014 and December 2014. The maximum longitudinal (cranio-caudal) dimension of tumor on the noncontrast T1-WI sequence of MRI (irrespective of whether it was pre/postchemotherapy) was compared with the gross dimensions of the tumor on histopathology. The arithmetic mean difference, Wilcoxon signed-rank test, and Spearman's correlation analysis were used to test the differences and correlation between groups. Mean tumor size on MRI based on the largest extent on MRI was 12.1 ± 4.85 cm (mean ± standard deviation), while it was 10.77 ± 4.6 cm (mean ± standard deviation) on histopathology. In 79 cases, MRI overestimated the extent of disease; the mean was 1.79 cm with a standard deviation of 1.56 cm. When the disease extent was underestimated on MRI (13 cases), the mean was 0.58 cm with a standard deviation of 0.43 cm. In 8 cases (osteosarcoma (7), Ewing's sarcoma (1)), MRI measurement was equal to histopathology. The Spearman correlation analysis showed a high correlation of tumor length on histopathology with the MRI for all patients (R = 0.948, P < 0.0001). We thus conclude that MRI is accurate in delineating the extent of bone sarcomas. A margin of 2 cm from the maximum tumor extent is adequate to ensure appropriate surgical resection.

Objective: To evaluate the efficacy and safety of nanosomal docetaxel lipid suspension (NDLS, DoceAqualip) based chemotherapy in patients with sarcoma.

Methods: In this retrospective, multicenter (6 centers), observational study, we analyzed the medical charts of adult patients of either sex, who were treated with NDLS (75 mg/m² in 3-weekly cycles) based chemotherapy for the treatment of sarcoma. The efficacy outcomes were overall response rate (ORR: complete response (CR) + partial response (PR)) and disease control rate (DCR: CR + PR + stable disease (SD)) in patients who received NDLS-based chemotherapy in neoadjuvant and metastatic settings. Overall survival (OS) and safety were evaluated for all settings.

Results: Of 11 patients (neoadjuvant: 1, adjuvant: 3, and metastatic: 7) in this study, majority had leiomyosarcoma (63.6%, 7/11) followed by extraskeletal myxoid chondrosarcoma (EMC), high grade pleomorphic sarcoma of mandible, malignant fibrous histiocytoma of right thigh, and osteosarcoma of femur (9.1% each, 1/11 each). NDLS plus gemcitabine combination was used in 10 patients (90.9%), and NDLS plus cyclophosphamide was used in one patient with EMC (9.1%). Efficacy evaluation was performed for 7 patients (neoadjuvant: 1/1; metastatic: 6/7). Complete response was reported in one patient (soft tissue sarcoma of mandible) treated in neoadjuvant setting. In metastatic setting, ORR was 50% and DCR was 66.7% (CR: 16.7% (1/6), PR: 33.3% (2/6), SD: 16.7% (1/6)). At a median follow-up of 6.5 months (range: 0.06-20.2 months), median OS was not reached in neoadjuvant and adjuvant settings, but it was 15.8 months in metastatic setting. At least 1 AE was reported in 7 (63.6%) patients. Neutropenia, thrombocytopenia, lymphopenia, and anemia were the hematological AEs, whereas nausea, vomiting, and diarrhea were the most common nonhematological AEs. NDLS treatment was well tolerated without any new safety concerns.

Conclusion: Nanosomal docetaxel lipid suspension-based chemotherapy was efficacious and well tolerated in the treatment of sarcoma. Further prospective trials are needed to confirm the data.