Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1–WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs. The treatment of advanced and recurrent disease with tyrosine kinase inhibitors, such as pazopanib, sunitinib, and mTOR inhibitors was evaluated by small trials. Recent studies using comprehensive molecular profiling of DSRCT identified potential therapeutic targets. In this review, we aim to describe the current studies conducted to better understand DSRCT biology and to explore the new therapeutic strategies under investigation in preclinical models and in early phase clinical trials.
Background
There are limited data on surgical complications for patients that have delayed surgery after severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection. We aimed to analyze the surgical outcomes of patients submitted to surgery after recovery from SARS‐CoV‐2 infection.
Methods
Asymptomatic patients that had surgery delayed after preoperative reverse‐transcription polymerase chain reaction (RT‐PCR) for SARS‐CoV‐2 were matched in a 1:2 ratio for age, type of surgery and American Society of Anesthesiologists to patients with negative RT‐PCR for SARS‐CoV‐2.
Results
About 1253 patients underwent surgical procedures and were subjected to screening for SARS‐CoV‐2. Forty‐nine cases with a delayed surgery were included in the coronavirus disease (COVID) recovery (COVID‐rec) group and were matched to 98 patients included in the COVID negative (COVID‐neg) group. Overall, 22 (15%) patients had 30‐days postoperative complications, but there was no statistically difference between groups –16.3% for COVID‐rec and 14.3% for COVID‐neg, respectively (odds ratio [OR] 1.17:95% confidence interval [CI] 0.45–3.0; p = .74). Moreover, we did not find difference regarding grades more than or equal to 3 complication rates – 8.2% for COVID‐rec and 6.1% for COVID‐neg (OR 1.36:95%CI 0.36‐5.0; p = .64). There were no pulmonary complications or SARS‐CoV‐2 related infection and no deaths within the 30‐days after surgery.
Conclusions
Our study suggests that patients with delayed elective surgeries due to asymptomatic preoperative positive SARS‐CoV‐2 test are not at higher risk of postoperative complications.
Sao Paulo countryside or other state 5 (12.2) Costs coverage Public health system (SUS) 8 (19.5) Insurance/private 33 (80.5) Primary cancer Genitourinary 15 36.6) Gastrointestinal 10 (24.4) Breast 5 (12.2) Head and neck 5 (12.2) Skin 3 (7.3) Others 3 (7.3) Surgical department a Urology 8 (19.5) Gynecology 7 (17.1) Vascular surgery 7 (17.1) Breast 5 (12.2) Head and neck 4 (9.8) Skin cancer 4 (9.8) Others 6 (14.6) Abbreviation: COVID-19, coronavirus disease 2019. a Department responsible for the current surgical procedure.
BackgroundRelapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1).MethodsSeventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included. The treating physicians defined the modality of local control based on the recommendations of the coordinating center and the patient and tumor characteristics. Possible associations of local control modality with local failure (LF), disease-free survival (DFS), event-free survival (EFS), overall survival (OS), and clinical characteristics were analyzed.ResultsMean patient age was 12.8 years (range, 2 to 25 years) and median follow-up time was 4.5 years (range, 2.3 to 6.7 years). Forty-seven patients underwent surgery, 13 received radiotherapy, and 13 received both. The 5-year EFS, OS, and DFS for all patients was 62.1%, 63.3%, and 73.1%, respectively. The 5-year cumulative incidence (CI) of LF was 7.6% for surgery, 11.1% for radiotherapy, and 0% for postoperative radiotherapy (PORT) (p = 0.61). The 5-year EFS was 71.7% for surgery, 30.8% for radiotherapy, and 64.1% for PORT (p = 0.009).ConclusionsThere was a significant effect of local control modality on EFS and OS in the study. Surgery and PORT modalities yielded very close results. The group treated with radiotherapy alone had considerably worse outcomes. This may be confounded by greater risk factors in these patients. There was no significant effect of local control modality on the CI of LF and DFS.
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