Juvenile polyposis syndrome with gastric and duodenal polyposis presenting with refractory anemia and protein-leakage gastroenteropathy in a patient with SMAD4 mutation: a case report.

BACKGROUND: Juvenile polyposis syndrome (JPS) is an autosomal dominant, inherited disorder characterized by multiple hyperproliferative polyps of the gastrointestinal tract, particularly of the colon, rectum, and stomach. SMAD4 mutations are frequently associated with multiple polyposis of the stomach; the condition causes severe bleeding and hypoproteinemia, which may progress to severe dysplasia and adenocarcinoma formation. We report our experience with the first case of total gastrectomy with pancreaticoduodenectomy following two partial jejunectomies for JPS, who presented with refractory anemia and protein-losing gastroenteropathy due to polyposis of the stomach and duodenum. CASE PRESENTATION: A 33-year-old Japanese man presented with the chief complaint of shortness of breath on exertion. His family history included gastric polyposis (mother, aunt, and cousin) and cerebral infarction (grandmother). Blood testing at the initial visit indicated iron-deficiency anemia, whereas endoscopy revealed multiple polyps in the duodenum and jejunum. Genetic testing revealed a 4 bp deletion (TGAA) in exon 5 of the SMAD4 gene; two partial small bowel resections were performed, but polyps grew in the remaining stomach, duodenum, and small intestine. The patient developed hypoalbuminemia and anemia, and required central venous nutrition and blood transfusion. However, because the hyponutrition and anemia remained poorly controlled, a total gastrectomy with concomitant pancreaticoduodenectomy was performed. Malnutrition and anemia improved, and there was no polyp recurrence in the remaining intestinal tract at 18 months after the surgery. CONCLUSIONS: We report a case of JPS with refractory anemia and protein-losing gastroenteropathy that was treated with total gastrectomy with concomitant pancreaticoduodenectomy. Although the surgery was highly invasive, the patient's nutritional status and anemia improved postoperatively, and the treatment was successful. However, to determine the appropriate surgical procedure, a detailed examination of the gastrointestinal lesions and the effects of the surgical invasion on nutritional status must be undertaken.

A new fistula risk score using sarcopenic obesity and subcutaneous fat area for predicting postoperative pancreatic fistula after pancreaticoduodenectomy.

BACKGROUND/PURPOSE: Postoperative pancreatic fistula (POPF) is a serious complication of pancreaticoduodenectomy and current predictors of POPF are inadequate. We developed a new fistula score to more accurately predict POPF. METHODS: We retrospectively reviewed 169 patients who underwent pancreaticoduodenectomy between January 2010 and August 2021 at our institution and examined patients' risk factors according to the occurrence of grade B/C POPF. Muscle and fat were assessed on preoperative computed tomography images and cutoff values were determined by receiver operating characteristic curve analysis. RESULTS: Grade B/C POPF occurred in 38 (22.5%) patients. Multivariate analysis of patients' risk factors revealed that sarcopenic obesity (odds ratio [OR] 2.94; p = .033), L3 subcutaneous fat area (SFA) ≥ 98.0 cm2 (OR 2.69; p = .049), and soft pancreatic texture (OR 27.5; p = .002) were independent risk factors of grade B/C POPF occurrence. In addition, a new fistula risk score based on these factors revealed that 63.6% of patients with high scores developed grade B/C POPF, while those with negligible or low scores did not. CONCLUSIONS: A new fistula risk scoring system based on sarcopenic obesity, SFA, and pancreatic texture may accurately predict POFP.

Impact of Early Enteral Nutrition on Delayed Gastric Emptying and Nutritional Status After Pancreaticoduodenectomy.

BACKGROUND: Although early enteral nutrition (EEN) is an accepted practice after pancreaticoduodenectomy (PD), the impact of EEN on postoperative complications or nutritional status remains unclear. We aimed to investigate the impact of EEN on delayed gastric emptying (DGE) and nutritional status after PD. METHODS: A total of 143 patients underwent PD between January 2012 and September 2020. We excluded patients who underwent a two-stage pancreatojejunostomy, in whom the enteral tube was accidentally pulled out, or with insufficient information in their medical records. The incidence of postoperative complications was compared between patients who received EEN (EEN group, n = 21) and those who did not (control group, n = 21) after propensity score matching. Univariate and multivariate analyses were performed to identify the risk factors affecting the incidence of these complications. Nutritional status was assessed at postoperative months 1, 3, and 6. RESULTS: The incidence of grade B/C DGE in the EEN group was significantly lower than that in the control group (4.8% vs. 28.6%, p = 0.03). There was no significant difference in overall morbidity, incidence of any other postoperative complications, or all-grade DGE. In multivariate analysis, EEN was associated with a reduction in the incidence of grade B/C DGE (p < 0.01). In the analysis of nutritional status, EEN was significantly associated with better nutritional status at postoperative month 1. CONCLUSION: EEN can lead to a lower clinically relevant DGE rate and better nutritional status in the early postoperative period in patients undergoing PD.

Gastrointestinal stromal tumor of the small bowel complicated by torsion: A case report.

INTRODUCTION: Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that originate from the gastrointestinal tract wall. Approximately 20-30 % of GISTs originate from the small intestine. GISTs of the small intestine generally present with a palpable mass, distention, and abdominal pain and may exhibit acute abdomen at the onset. Herein, we describe a rare case of a pedunculated GIST of the small intestine complicated by torsion. PRESENTATION OF CASE: A 69-year-old woman presented with lower abdominal pain. Abdominal contrast-enhanced computed tomography showed a 73 × 62 × 57-mm3 tumor in the pelvic cavity with enhanced margins and reduced contrast. It was presumed that the tumor had caused hemorrhagic infarction. Emergency laparotomy was performed, and the pedunculated tumor was found to be twisted 360° clockwise at the pedicle with hemorrhage and necrosis due to torsion. We performed partial resection of the small intestine including the tumor. Histopathological examination revealed tightly arranged spindle-shaped cells with hemorrhage, congestion, and inflammatory cell infiltration. Immunohistochemical staining showed positivity for CD34, CD117, and DOG1. CONCLUSIONS: Torsion of a pedunculated small intestine GIST, although very rare, requires emergency surgery and should be recognized as a cause of acute abdomen in patients with GIST. Immediate surgery is mandatory if torsion of a small intestinal GIST is suspected because the GIST or intestine may become necrotic owing to hemorrhagic infarction.

Primary spindle cell tumor originating from the liver that was difficult to diagnose.

BACKGROUND: It has been reported that hepatocellular carcinoma (HCC) with spindle cell tumor accounts for 1.8% of all HCCs, but spindle cell tumors that do not show an obvious conventional HCC are extremely rare. In this report, we describe a case of resection of a primary spindle cell tumor of the liver that was difficult to diagnose. CASE PRESENTATION: A 75-year-old man presented with fever and right chest pain. He was suspected of a giant primary diaphragmatic tumor of extrahepatic origin by imaging studies. The preoperative differential diagnosis included benign masses such as myxoid sarcoma and schwannoma, and we planned a diaphragmatic resection. Intraoperatively, however, dissection of the tumor from the liver was not possible, requiring an extended right posterior segmentectomy with combined resection of the diaphragm. The patient had a good postoperative course and 1 year has passed since the surgery with no recurrence. The pathology showed that the mass was located just below the hepatic capsule/parenchymal region and was adherent to the diaphragm, but there was no continuity. The morphology suggested a low-grade mesenchymal tumor such as a solitary fibrous tumor and perivascular epithelioid cell tumor, but immunostaining was negative, making the diagnosis difficult. Although some areas of high proliferative activity were observed, finally, the diagnosis of primary spindle cell tumor of the liver with smooth muscle differentiation was made based on the positive results of muscle markers such as αSMA, desmin, and h-caldesmon. CONCLUSIONS: Spindle cell tumor arising from the liver is so rare that preoperative and pathological diagnosis is often difficult to reach. Although further studies are needed to elucidate and better understand this uncommon clinical entity, we consider that complete resection is necessary for the above case, which may contribute to long-term survival.

The survival benefit of surgery for perihilar cholangiocarcinoma in octogenarians: Shinshu University experience.

BACKGROUND AND OBJECTIVES: In today's aging society, surgery is increasingly performed for older patients with perihilar cholangiocarcinoma (PHCC). This study aimed to examine survival and oncological outcomes in octogenarians with PHCC who underwent surgery. METHODS: We retrospectively examined 480 consecutive PHCC patients treated for PHCC between 1991 and 2020. Two hundred forty-seven underwent resection. Patient and clinical characteristics, including postoperative complications, overall survival (OS), and disease-free survival (DFS) were analyzed. RESULTS: The proportion of octogenarians with PHCC undergoing surgery increased from 5.2% in 1991-2000 to 13.4% in 2016-2020. R0 resection and postoperative complication rates were comparable between octogenarians and younger patients. Five-year OS (30.0% vs. 31.7%) and DFS (20.0% vs. 29.5%) did not significantly differ. One-year OS was significantly better in octogenarians who underwent resection than those who did not (95.0% vs. 17.6%; p < .001). Two octogenarians who developed Clavien-Dindo grade III complications had undergone right hemihepatectomy (RH). Posthepatectomy liver failure and biliary leakage (Grade B) were also observed in octogenarians who underwent RH. CONCLUSION: In well-selected octogenarians with PHCC, surgery offers short- and long-term outcomes comparable with those of their younger counterparts. RH may be considered in octogenarians on an individual basis.

Safety and Oncological Benefit of Hepatopancreatoduodenectomy for Advanced Extrahepatic Cholangiocarcinoma with Horizontal Tumor Spread: Shinshu University Experience.

BACKGROUND: Although hepatopancreatoduodenectomy (HPD) is the only means of achieving R0 resection of widespread extrahepatic cholangiocarcinoma, its safety and oncological benefit remain controversial because of its inherent high risk of mortality and morbidity. OBJECTIVE: The aim of this study was to retrospectively analyze short- and long-term outcomes and evaluate the safety and oncological benefit of this advanced procedure. METHODS: The study cohort comprised 37 consecutive patients who had undergone major HPD. Portal vein embolization was performed before surgery in 20 (54%) patients with future remnant liver volume < 35%. RESULTS: The median operative time and blood loss were 866 min and 1000 mL, respectively. Concomitant vascular resection was performed in five patients (14%). The overall morbidity and mortality rates were 100% and 5.4% (n = 2), respectively. Nineteen patients (51%) had major (Clavien-Dindo grade III or higher) complications, the most common being intra-abdominal infection (49%) and post-hepatectomy liver failure (46%, grade B/C: 32%/5%), followed by postoperative pancreatic fistula (30%, grade B/C). R0 resection was achieved in 31 patients (84%). The 1-, 3-, and 5-year overall survival (OS) rates were 83%, 48%, and 37%, respectively. In patients with R0 resection, 5-year OS was comparable between patients who had undergone major HPD and major hepatectomy alone (41% vs. 40%, p = non-significant). CONCLUSIONS: HPD is a valid treatment option for extensive cholangiocarcinoma, offering long-term survival benefit at the cost of relatively high but acceptable morbidity and mortality rates. HPD is advocated in selected patients provided that it is considered possible to achieve R0 resection.

Impact of Remnant Carcinoma in Situ at the Ductal Stump on Long-Term Outcomes in Patients with Distal Cholangiocarcinoma.

BACKGROUND: The management of positive ductal margins with carcinoma in situ (R1-CIS) after resection is controversial. The aim of this study was to evaluate the impact of R1-CIS on survival in patients who underwent resection for distal cholangiocarcinoma. METHODS: We enrolled 121 consecutive patients with distal cholangiocarcinoma. Poor prognostic factors were investigated by multivariable analysis, and we performed a stratified analysis to evaluate the impact of R1-CIS on survival in patients with or without prognostic factors. RESULTS: Multivariable analysis identified node-positive status as the prognostic factor (P = 0.003). Stratified by lymph node status, overall survival (OS) in the R0 group was significantly better than that in the R1-CIS group in node-negative patients (57.1% vs 30.0%; P < 0.050). Although OS was comparable between the two groups in node-positive patients (5-year OS: 22.2% vs 20.0%, respectively; P = not significant). Furthermore, OS in patients in whom R0 was achieved by additional resection was significantly better than that in patients with R1-CIS (5-year OS: 66.7% vs 30.0%, respectively; P < 0.050). CONCLUSIONS: Remnant CIS is associated with a poor prognosis in patients with node-negative distal cholangiocarcinoma. Every effort should be made to achieve negative bile duct margins.

Preoperative C-reactive protein-to-albumin ratio predicts long-term outcomes in extrahepatic cholangiocarcinoma patients.

BACKGROUND: Systemic inflammation may have prognostic value in some malignancies and association with lymph node metastasis. This study aimed to evaluate the impact of systemic inflammatory biomarkers on long-term and oncological outcomes as well as to assess the association between biomarkers with lymph node metastasis in extrahepatic cholangiocarcinoma patients. METHODS: We enrolled 271 consecutive patients who underwent surgical resection for extrahepatic cholangiocarcinoma. Poor prognostic factors were compared to identify the biomarkers that were most associated with overall survival (OS) and disease-free survival (DFS) using receiver operating characteristic curves and multivariable analysis. Furthermore, we evaluated the relationship between biomarkers and lymph node metastasis. RESULTS: Four and two biomarkers were predictive for OS and DFS, respectively, among which, the C-reactive protein-to-albumin ratio (CAR) had the highest area under the curve values (OS: 0.631, DFS: 0.624). Multivariable analysis showed that a high CAR was an independent prognostic factor for both OS and DFS (P = .002 and P < .001, respectively). Although a high CAR was not significantly correlated with lymph node metastasis (P = .645), carbohydrate antigen 19-9 showed a significant correlation (P < .001). CONCLUSIONS: Preoperative CAR is the most accurate prognostic factor for OS and DFS in extrahepatic cholangiocarcinoma patients and is independent of lymph node metastasis.

Left-sided portal hypertension caused by idiopathic splenic vein stenosis improved by splenectomy: a case report.

BACKGROUND: Splenic vein stenosis and occlusion, which are known causes of left-sided portal hypertension, often occur secondary to trauma, pancreatitis, or invasion or compression by pancreatic tumors. However, few reports have described idiopathic splenic vein stenosis. CASE PRESENTATION: A 70-year-old man was referred to our hospital for examination of isolated gastric varices. He had no history of liver disease, pancreatitis, or abdominal trauma. Computed tomography revealed stenosis of almost the entire length of the splenic vein, and development of gastric fundal and short gastric varices. No inflammatory changes or neoplastic lesions of the pancreas were observed in any imaging study. The patient was diagnosed with left-sided portal hypertension caused by idiopathic splenic vein stenosis, and splenectomy was performed. The postoperative course was smooth, and improvement of the gastric varices was shown by upper gastrointestinal endoscopy at 3 months after the operation. CONCLUSIONS: Idiopathic splenic vein stenosis is an extremely rare cause of left-sided portal hypertension. Splenectomy is one of the most effective treatments for left-sided portal hypertension caused by idiopathic splenic vein stenosis.