RESUMO
Pancreatic ductal adenocarcinoma is the most prevalent form of pancreatic cancer, originating in the duct lining of the pancreas. The simultaneous occurrence with a solitary fibrous tumor (SFT) represents an unexpected finding. We present a case involving a 64-year-old female with synchronous pancreatic cancer and SFT. The patient initially experienced severe abdominal pain, visible jaundice, and itching. Diagnostic imaging revealed a mass in the head of the pancreas and a soft tissue mass in the right hemipelvis. Further investigations included histological examination, immunohistochemistry, and genetic testing. Subsequently, the patient underwent appropriate management, which involved the excision of both masses and radiochemotherapy. The discussion focuses on the genetic linkages in this rare presentation, aiming to identify treatment connections for both tumors. Throughout this case report, our aim is to contribute to enriching the limited literature with new insights and underscore the importance of identifying genetic linkages between both tumors which may lead to more effective management strategies and better treatment outcomes.
RESUMO
PURPOSE: This study addresses the effectiveness of oral everolimus in treating various malignancies associated with Neurofibromatosis Type 1 (NF1). The purpose is to determine whether everolimus reduces lesion size in NF1 patients, considering the controversial findings from previous clinical trials. The scientific hypotheses and questions involve evaluating the impact of everolimus on NF1-associated lesions and understanding the variability in treatment outcomes. METHODS: A systematic review and meta-analysis were conducted following PRISMA and Cochrane Collaboration guidelines. The study included four-phase II, single-arm, nonrandomized trials investigating the effect of oral everolimus on NF1-associated lesion size. The search covered multiple databases, and data extraction involved evaluating studies for inclusion criteria and assessing quality using the Cochrane Collaboration's Risk of Bias in Nonrandomized Studies tool. Statistical analysis utilized Open Meta(Analyst). FINDINGS: The search yielded 388 studies, with 10 selected for full-text review and four included in the final analysis. The quality of the studies ranged from low to moderate. The meta-analysis indicated no observed heterogeneity (I^2 = 0%), and the overall estimate suggested no significant reduction in NF1-associated lesion size with everolimus (P = 0.069). IMPLICATIONS: The findings reveal a varied and inconsistent picture of everolimus efficacy in NF1 treatment. The study highlights the need for personalized approaches, considering individual genetic and clinical differences. The limitations, including small sample sizes and nonrandomized trials, call for larger, more standardized research efforts. The study emphasizes ongoing trials and the importance of future research in understanding predictors of everolimus response and optimizing treatment strategies for NF1 patients. CONCLUSION: While everolimus shows promise in reducing lesion size in a subset of NF1 patients, the study cannot draw conclusive results due to limitations in the included studies. Ongoing, adequately powered trials are crucial for advancing the evidence base and informing the potential role of everolimus in NF1 treatment. OTHERS: There was no funding for this review and no conflicts of interest.