Adult pancreatoblastoma: A rare malignant tumor of the pancreas.

Pancreatoblastoma (PB) is a rare malignant tumor in adults. It has an overall incidence of 0.004 per 100,000 inhabitants. Its diagnosis with fine-needle aspiration (FNA) is difficult due to multiple differentiation lines present on PB that overlap with other tumors. A 76-year-old male patient presented with jaundice, weight loss of 10 kg in 6 months, and appetite loss. Abdominal computed tomography scan showed a tumor in the pancreas head. Transendoscopic ultrasound with FNA biopsy revealed a malignant epithelial neoplasia compatible with PB with immunohistochemistry CK19 (+), P63(+), synaptophysin (-), and Ki67 50%. The patient underwent surgical resection and adjuvant chemotherapy. Currently, the patient is in follow-up with the latest imaging showing no evidence of disease at 32 months after his initial diagnosis. PB is an uncommon malignant neoplasia with an aggressive behavior. Its diagnostic and therapeutic protocols are unclear. Its preoperative diagnosis may be difficult since its imaging features and serological markers are non-specific. However, FNA may be useful in some situations. Surgical resection is currently the recommended treatment that is associated with the best long-term survival.

Defining Benchmark Outcomes for Pancreatoduodenectomy With Portomesenteric Venous Resection.

OBJECTIVE: The aim of this study was to establish clinically relevant outcome benchmark values using criteria for pancreatoduodenectomy (PD) with portomesenteric venous resection (PVR) from a low-risk cohort managed in high-volume centers. SUMMARY BACKGROUND DATA: PD with PVR is regarded as the standard of care in patients with cancer involvement of the portomesenteric venous axis. There are, however, no benchmark outcome indicators for this population which hampers comparisons of patients undergoing PD with and without PVR resection. METHODS: This multicenter study analyzed patients undergoing PD with any type of PVR in 23 high-volume centers from 2009 to 2018. Nineteen outcome benchmarks were established in low-risk patients, defined as the 75th percentile of the median outcome values of the centers (NCT04053998). RESULTS: Out of 1462 patients with PD and PVR, 840 (58%) formed the benchmark cohort, with a mean age was 64 (SD11) years, 413 (49%) were females. Benchmark cutoffs, among others, were calculated as follows: Clinically relevant pancreatic fistula rate (International Study Group of Pancreatic Surgery): ≤14%; in-hospital mortality rate: ≤4%; major complication rate Grade≥3 and the CCI up to 6 months postoperatively: ≤36% and ≤26, respectively; portal vein thrombosis rate: ≤14% and 5-year survival for patients with pancreatic ductal adenocarcinoma: ≥9%. CONCLUSION: These novel benchmark cutoffs targeting surgical performance, morbidity, mortality, and oncological parameters show relatively inferior results in patients undergoing vascular resection because of involvement of the portomesenteric venous axis. These benchmark values however can be used to conclusively assess the results of different centers or surgeons operating on this high-risk group.

Appendiceal carcinoma associated with microsatellite instability.

Approximately 15% of colorectal cancer (CRC) cases exhibit microsatellite instability (MSI), which appears to be associated with unique biological behavior. The present study presents a case of appendiceal carcinoma associated with MSI that responded well to adjuvant chemotherapy. Clinical, pathological and immunohistochemical (IHC) characteristics have been described. The 60-year-old male patient had suffered from recurrent lower abdominal pain associated with abdominal distention for 6 months; then, following an acute attack, he was subjected to laparoscopic appendectomy. The histopathological examination revealed moderately differentiated appendiceal adenocarcinoma with mucinous areas, without lymphovascular or perineural invasion. The IHC examination was positive for keratin-20 and caudal type homeobox 2, and negative for MutL Homolog 1, MutS Homolog (MSH) 2 and MSH-6. A postoperative colonoscopy revealed diverticulosis, without the presence of polyps or tumors. However, an abdominal axial computerized tomography scan revealed thickening of the distal portion of the appendix, increased density of the greater omentum, and metastases to the liver capsule, spleen and peritoneum. The treatment of choice was right hemicolectomy with peritoneal debulking, followed by 10 cycles of chemotherapy with 5-fluorouracil, leucovorin and oxaliplatin (FOLFOX regimen). After 5 years of follow-up, the patient remains in good condition, without clinical or radiological signs of recurrence. The good response to chemotherapy corresponds with the observations made in other colon cancers with MSI. Therefore, testing for MSI in appendiceal carcinomas may provide useful information on prognosis and predict response to chemotherapy.

Pattern of Venous Collateral Development after Splenic Vein Occlusion in an Extended Whipple Procedure (Whipple at the Splenic Artery) and Long-Term Results.

BACKGROUND: Extended Whipple procedures may require division of the splenic vein (SV). Controversy exists regarding the risk of sequelae of sinistral portal hypertension when the SV is ligated without reimplantation. The aim of this study was to identify postoperative venous collateral patterns and sequelae of SV ligation, as well as long-term results in an extended Whipple procedure. STUDY DESIGN: Patients who had an extended Whipple procedure (Whipple at the Splenic Artery or WATSA) were entered in an institutional database. Evaluation of the venous collaterals was performed at least 5 months postoperatively by imaging. Spleen size and platelet counts were measured before and after operation. RESULTS: Fifteen patients were entered from 2009 to 2014. SV was not reconstructed and the IMV-SV junction was always resected. Two collateral routes developed. An inferior route was present 14/15 patients. It connected the residual SV to the SMV via intermediate collateral veins in the omentum and along the colon. A superior route, present in 10/15 patients connected the residual SV to the portal vein via gastric, perigastric, and coronary veins. Gastrointestinal bleeding did not occur. Mean platelet count and spleen size were not affected significantly. Procedures were long, but few severe complications developed. In 12 patients with adenocarcinoma, the median survival has not been reached. CONCLUSIONS: Patients who have SV ligation in an extended Whipple are protected against sequelae of sinestral portal hypertension by inferior collateral routes. The omentum and marginal veins of the colon are key links in this pathway.