Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumor in Patients with Agenesis of the Dorsal Pancreas

Background: This study aimed to clarify the feasibility and justification of pancreatic head sparing (PHS) enucleation for patients with agenesis of the dorsal pancreas (ADP) associated with a solid pseudopapillary tumor (SPT).Methods: Data of the SPT patients with and without ADP, including clinical presentations, surgical options, and surgical and survival outcomes, were recruited for comparison.Results: A total of 31 patients with SPTs were included, 3 of whom displayed ADP and underwent PHS enucleation. Surgical complications were comparable between the groups. Overall, the 5-year and 10-year disease-free survival rates were 100% and 90%, respectively. The 20-year and 25-year overall survival rates were 100% and 66.7%, respectively. Only one patient (3.2%) developed tumor recurrence 7.3 years after pancreatectomy for an SPT with lymph node involvement, and the patient survived 24.5 years after the initial operation. No tumor recurrence occurred in any patient with ADP after PHS enucleation.Conclusion: The association between tumorigenesis and ADP suggests that every congenital ADP patient should be observed for the early detection of pancreatic neoplasia. PHS enucleation seems to be feasible and justifiable for SPT patients with ADP in terms of surgical and survival outcomes, and this approach could be recommended to avoid pancreatic insufficiency.

A case of curative surgical treatment of the rare pancreatic tumor in a child

Pancreatic tumors are very rare problem in children that mostly in literature presented just with case reports. Solid-pseudopapillary tumors of the pancreas are a little part, just about 1–2%, from all nonendocrine tumors of the pancreas. About 90% of all patients with this tumor are female teenagers and young women. Solid-pseudopapillary tumors of the pancreas are innocent, and mostly all of them are incidental findings. However, we can see clinical findings if patient has big size of such tumor, which compress nearest anatomical structures. In this article we present a case of incidental finding of solid- pseudopapillary tumor of body and tale of the pancreas in 11 years old girl, who was treated with distal resection of pancreas.

The Landscape of Genetic Alterations Stratified Prognosis in Oriental Pancreatic Cancer Patients

BackgroundPancreatic cancer is a life-threatening malignant disease with significant diversity among geographic regions and races leading to distinct carcinogenesis and prognosis. Previous studies mainly focused on Western patients, while the genomic landscape of Oriental patients, especially Chinese, remained less investigated.MethodsA total of 408 pancreatic cancer patients were enrolled. A panel containing 436 cancer-related genes was used to detect genetic alterations in tumor samples.ResultsWe profiled the genomic alteration landscape of pancreatic duct adenocarcinoma (PDAC), intraductal papillary mucinous neoplasm (IPMN), periampullary carcinoma (PVC), and solid-pseudopapillary tumor (SPT). Comparison with a public database revealed specific gene mutations in Oriental PDAC patients including higher mutation rates of DNA damage repair-related genes. Analysis of mutational signatures showed potential heterogenous carcinogenic factors caused by diabetes mellitus. KRAS mutation, especially KRAS G12D mutation, was associated with poor survival, while patients not harboring the 17 significant copy number variations (CNVs) had a better prognosis. We further identified multiple correlations between clinicopathologic variables and genetic mutations, as well as CNVs. Finally, by network-based stratification, three classes of PDAC patients were robustly clustered. Among these, class 1 (characterized by the Fanconi anemia pathway) achieved the best outcome, while class 2 (involved in the platinum drug resistance pathway) suffered from the worst prognosis.ConclusionsIn this study, we reported for the first time the genetic alteration landscape of Oriental PDAC patients identifying many Oriental-specific alterations. The relationship between genetic alterations and clinicopathological factors as well as prognosis demonstrated important genomic impact on tumor biology. This study will help to optimize clinical treatment of Oriental PDAC patients and improve their survival.