Testicular, Spermatic Cord, and Scrotal Soft Tissue Sarcomas: Treatment Outcomes and Patterns of FailureRead the full article
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Prognostic and Therapeutic Utility of Variably Expressed Cell Surface Receptors in Osteosarcoma
Background. Six cell surface receptors, human epidermal growth factor receptor-2 (Her-2), platelet-derived growth factor receptor-β (PDGFR-β), insulin-like growth factor-1 receptor (IGF-1R), insulin receptor (IR), c-Met, and vascular endothelial growth factor receptor-3 (VEGFR-3), previously demonstrated variable expression across varying patient-derived and standard osteosarcoma (OS) cell lines. The current study sought to validate previous expression patterns and evaluate whether these receptors offer prognostic and/or therapeutic value. Methods. Patient-derived OS cell lines (n = 52) were labeled with antibodies to Her-2, PDGFR-β, IGF-1R, IR, c-Met, and VEGFR-3. Expression was characterized using flow cytometry. The difference in geometric mean fluorescent intensity (geoMFIdiff = geoMFIpositive − geoMFInegative) was calculated for each receptor across all cell lines. Receptor expression was categorized as low (Q1), intermediate (Q2, Q3), or high (Q4). The event-free survival (EFS) and overall survival for the six cell surface receptors were estimated by the Kaplan–Meier method. Differences in hazard for EFS event and overall survival event for patients in each of the three expression levels in each of the six cell surface receptors were assessed using the log-rank test. Results. All 6 receptors were variably expressed in the majority of cell lines. IR and PDGFR-β expressions were found to be significant predictors for EFS amongst patients with nonmetastatic disease ( and 0.01, respectively). The hazard ratio for EFS was significantly higher between high IR and intermediate IR expression (HR = 2.66, ), as well as between high PDGFR-β and intermediate PDGFR-β expression (HR = 5.68, ). Her-2, c-Met, IGF-1R, and VEGFR-3 were not found to be significant predictors for either EFS or overall survival. Conclusion. The six cell surface receptors demonstrated variable expression across the majority of patient-derived OS cell lines tested. Limited prognostic value was offered by IR and PDGFR-β expression within nonmetastatic patients. The remaining receptors do not provide clear prognostic utility. Nevertheless, their consistent, albeit variable, surface expression across a large panel of patient-derived OS cell lines maintains their potential use as future therapeutic targets.
Sarcoma of the Breast: Clinical Characteristics and Outcomes of 991 Patients from the National Cancer Database
Background. Sarcoma of the breast is a rare malignancy with heterogeneous histology. Angiosarcoma, including secondary angiosarcoma from previous radiation, is the most common type of sarcoma of the breast. Other types of sarcomas of the breast have limited clinical and survival information. Methods. We obtained clinicopathological data and survival outcomes from the patients with sarcoma of the breast, excluding angiosarcoma, that were registered in the National Cancer Database (NCDB) from 2004 to 2016. The treatment patterns and prognostic factors were analyzed. Results. A total of 991 patients had sarcoma of the breast other than angiosarcoma. The most common histology was spindle cell sarcoma (13.4%), followed by leiomyosarcoma (11.7%) and giant cell sarcoma (10.1%). Surgical resection was performed in 894 out of 991 patients (90.2%), including R0 resection achieved in 781 (87.4%). The patients who received surgery showed better survival than the patients without surgery regardless of radiation therapy. When radiation was added to the surgical management, the OS (overall survival) benefit was marginally significant (hazard ratio 1.30 (CI 1.01–1.67), ). Adding chemotherapy did not improve OS. Conclusions. Surgical resection seems to be the most important treatment modality in sarcoma of the breast from the analysis of a large database. Radiation therapy added a minor survival benefit to the patients who received surgical resection. Systemic chemotherapy did not play a clear role in sarcoma of the breast.
Clear Cell Chondrosarcoma: Clinical Characteristics and Outcomes in 15 Patients
Background. Clear cell chondrosarcoma (CCC) represents less than 6% of all chondrosarcomas, and thus, our understanding of this rare entity is limited. Analyzing clinical characteristics and treatment patterns, thus increasing our knowledge, may improve treatment strategy. We review our institutional experience with 15 patients, including one case with dedifferentiation. Methods. A retrospective review was conducted in CCC patients treated at our institution from 1996 to 2015, with at least 2-year follow-up. Descriptive statistics and Kaplan–Meier survival analyses were performed. Results. Of 19 patients identified, 15 patients had at least 2-year follow-up and were included. The median age at diagnosis was 43 years. 80% were male. The most common presenting signs were pain (12 patients; 80%) and fracture (2 patients; 13.3%). The most common site was proximal femur (8 patients; 53%). All patients had MSTS Stage I disease. Primary treatment included wide resection in 10 patients (67%) and intralesional or marginal resection in 5 patients (33%). Three patients died of disease during the study period, 1 with dedifferentiation of recurrent CCC. The median time to death from disease was 15.3 years (95% CI: (14.2; NA)). The median time to either recurrence or death was 7.73 years for patients who had intralesional/marginal resection and 16.44 years for patients with wide resection (HR (wide vs. intralesional/marginal) = 0.21, 95% CI: (0.04; 1.18), ). The median time to recurrence or death was significantly shorter for patients not initially treated at a sarcoma center (). Conclusions. CCC is a rare entity, and our understanding of it is still evolving. We observed a higher recurrence rate for intralesional or marginal resection, and wide resection alone remains the mainstay of treatment. Better outcomes were observed in patients initially treated by trained musculoskeletal oncologists. Due to the propensity of CCC to recur decades after initial resection, lifelong surveillance is recommended.
Assessment of Resection Margins in Bone Tumor Surgery
Limb salvage surgery is now the preferred procedure for bone tumor surgery. To decrease the risk of local recurrence, it is crucial to obtain adequate resection margins. The obtained margins must be evaluated postoperatively because they influence what treatment is given subsequently when margins are not adequate (e.g., surgical revision and radiotherapy). The study aims to evaluate margin assessment of tumor specimen by MRI compared to conventional histology (to establish the viability of using MRI) and assess the accuracy of a patient-specific instrument when narrow margins were aimed. The resection margins in 12 consecutive patients that were operated on for bone tumor resection were prospectively analyzed using three methods: MRI of the resection specimen, macroscopic evaluation of specimen slices, and microscopic pathological evaluation. The assessments were qualitative (R0, R1, and R2) and quantitative (distance in mm). MRI, macroscopic, and microscopic margins generated similar results for both the qualitative (all resections were R0) and quantitative assessments. The median error in safe margins was 2 mm with a surgical guide (PSI) and 5 mm without a surgical guide. Local recurrences were not detected after a mean follow-up period of 3.7 years (range, 2.1–5 years); however, four patients died during the study. In conclusion, MRI is a valuable tool for assessing safe margins. When specimens are not available for pathological assessment (e.g., extracorporeally irradiated autograft or autoclaved autograft), MRI could be used to evaluate margins. In particular, when tumor volume is high, MRI could also help to focus the pathological examination on areas of concern.
Survey of Paediatric Oncologists and Pathologists regarding Their Views and Experiences with Variant Translocations in Ewing and Ewing-Like Sarcoma: A Report of the Children’s Oncology Group
Advances in molecular diagnostics have identified subsets of Ewing and Ewing-like sarcomas driven by variant translocations with unique biology. It is likely that patients with these tumours will have different clinical features and therapeutic outcomes. Nevertheless, the management of these patients both locally and within cooperative group trials depends on the local pathological diagnosis. It is not known what molecular diagnostic approaches are employed by local pathologists or if the exact translocation is commonly determined. In addition, it is not known what therapeutic approaches are employed for these patients or what cooperative trials are deemed appropriate for these patients by expert consensus. To answer these questions, we performed an international survey of oncologists and pathologists to better understand the diagnostic approaches used to identify variant translocations and the influence the findings have on therapy and clinical trial eligibility. An online survey was distributed to oncologists and pathologists primarily in North America. A total of 141 surveys were completed, representing a 28% response rate. The majority of respondents considered EWSR1-ETS gene family translocations (range 61–96%) to be Ewing sarcoma and would include them on the primary arm of a Ewing sarcoma clinical trial. There was a lack of consensus on how to classify and stratify BCOR-CCNB3, CIC-DUX4, and EWSR1+ with non-ETS partner fusions. Most respondents were either unsure how their institution tested, or their institution did not perform the test. In cases with atypical Ewing morphology, most respondents favoured additional fusion transcript testing. There is a lack of consensus regarding the classification and stratification of rare molecular subtypes in Ewing sarcoma. It is not clear how these alternative translocations have impacted outcomes for past clinical studies. This suggests a need for molecular confirmation of diagnoses and centralized or minimum standardization of testing for future trial enrolment.
Potential Association between Kaposi Sarcoma and Gout: An Exploratory Observational Study
Background. Kaposi sarcoma is a rare vascular mesenchymal neoplasm, associated with Human Herpes Virus 8 (HHV8). Gout is a condition clinically characterized by recurrent flares of arthritis and hyperuricemia. Following our clinical impression that patients with classical Kaposi sarcoma (CKS) have a high rate of gout, we explored this in a retrospective manner. Methods. All consecutive patients diagnosed with sarcoma or carcinosarcoma within a single tertiary center between 1/2012–12/2017 were identified through the pathology department database. A cohort of CKS patients was compared with the non-Kaposi sarcoma and carcinosarcoma cohort. Data were extracted from patients’ electronic medical records. Patients younger than 18 and patients without clinical data available were excluded. Association between diagnosis of gout and CKS was assessed and adjusted for risk factors. Results. Three hundred and sixty-one patients were eligible for this analysis, 61 were diagnosed with CKS and 300 with other types of sarcoma. We found a higher incidence of gout in CKS patients, 11/61 (18%) patients, compared with 8/300 (2.6%) with other types of sarcoma, odds ratio (OR) 8.0 (). This association persisted when adjusted for age >39 years (OR = 6.7, ), age and male sex (OR = 4.97, ), and when adjusting for multiple confounding factors and medical comorbidities. Conclusions. We have demonstrated a statistically significant association between gout and CKS. As risk factors for gout were accounted for, this association may be explained by HHV8 immune-related effects. This should be further explored in vitro and in population-based studies.