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BACKGROUND: Despite growing evidence of the effectiveness of minimally invasive surgery (MIS) for primary gastric cancer, MIS for remnant gastric cancer (RGC) remains controversial due to the rarity of the disease. This study aimed to evaluate the surgical and oncological outcomes of MIS for radical resection of RGC. PATIENTS AND METHODS: Patients with RGC who underwent surgery between 2005 and 2020 at 17 institutions were included, and a propensity score matching analysis was performed to compare the short- and long-term outcomes of MIS with open surgery. RESULTS: A total of 327 patients were included in this study and 186 patients were analyzed after matching. The risk ratios for overall and severe complications were 0.76 [95% confidence interval (CI): 0.45, 1.27] and 0.65 (95% CI: 0.32, 1.29), respectively. The MIS group had significantly less blood loss [mean difference (MD), -409 mL; 95% CI: -538, -281] and a shorter hospital stay (MD, -6.5 days; 95% CI: -13.1, 0.1) than the open surgery group. The median follow-up duration of this cohort was 4.6 years, and the 3-year overall survival were 77.9% and 76.2% in the MIS and open surgery groups, respectively [hazard ratio (HR), 0.78; 95% CI: 0.45, 1.36]. The 3-year relapse-free survival were 71.9% and 62.2% in the MIS and open surgery groups, respectively (HR, 0.71; 95% CI: 0.44, 1.16). CONCLUSIONS: MIS for RGC showed favorable short- and long-term outcomes compared to open surgery. MIS is a promising option for radical surgery for RGC.

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We previously reported the feasibility and efficacy of neoadjuvant chemotherapy without radiotherapy for locally advanced rectal cancer. Here, we report the results of a long-term follow-up study.This was a multi-institutional, prospective phase 2 study of patients with locally advanced rectal cancer. Patients received neoadjuvant chemotherapy with molecularly targeted agents before undergoing total mesorectal excision. Six cycles of modified FOLFOX (mFOLFOX6) with bevacizumab were administered to KRAS-mutant patients, and mFOLFOX6 with cetuximab was administered to KRAS-wild-type patients. Here, we report the secondary end points of overall survival, relapse-free survival, and local recurrence rate.Sixty patients were enrolled in this study. R0 resection was achieved in 98.3% (59/60) patients, and pathological complete response was achieved in 16.7% (10/60) patients. After a median follow-up of 5.4 years, the 5 year overall survival was 81.6%, the 5 year relapse-free survival was 71.7%, and the 5 year local recurrence rate was 12.6%. None of the patients who achieved pathological complete response developed recurrence within 5 years.The use of molecularly targeted agents in the neoadjuvant setting for locally advanced rectal cancer has an acceptable prognosis.

Highlights • Splanchnic venous thrombosis caused by superior mesenteric vein thrombosis is rare. • Delayed diagnosis and/or treatment can result in acute intestinal infarction. • Protein C deficiency is rare. • Insufficient awareness can result in mismanagement of splanchnic venous thrombosis. • Coagulation disorders contribute to acute intestinal infarction of unknown cause., Introduction and importance Splanchnic venous thrombosis (SVT) originating in the superior mesenteric vein (SMV) is rare and may cause acute intestinal infarction (AII). Protein C deficiency (PCD) results in thrombophilia. Presentation of case Acute unexplained SVT originating in the SMV and portal vein was detected in 68-year-old man. Pan-peritonitis and AII were diagnosed and emergency surgery performed. Part of the small intestine was necrotic and partial resection without anastomotic reconstruction was performed. Heparin was administered intravenously continuously from postoperative day (POD) 1. Hereditary, heterozygous, type 1 PCD was diagnosed postoperatively. The anastomosis was reconstructed on POD 16. Warfarin was substituted for heparin on POD 22. No recurrent thrombosis occurred during 2 years of follow-up. Clinical discussion Patients with the rare condition of SVT require prompt diagnosis and treatment and may have underlying disease. PCD can cause SVT even in intact veins and anticoagulation therapy should be administered immediately postoperatively. Misdiagnosis and/or delayed treatment of SVT can result in AII, a life-threatening condition with a high mortality rate. Insufficient clinician awareness can result in serious mismanagement of patients with PCD and SVT; emergency patients with AII caused by unexplained SVT should therefore be further investigated for prothrombotic states and assessment of coagulation–fibrinolysis profiles to clarify the underlying mechanism. Conclusion We here present a thought-provoking emergency case of AII associated with acute SVT caused by underlying PCD that was successfully treated by two-stage surgery and anticoagulation therapy. This case provides a timely reminder for emergency clinicians and gastrointestinal surgeons.

BACKGROUND Advanced gastric cancer (GC) with liver metastasis is often characterized by multiple and bilobular metastases and may also be associated with extrahepatic metastatic lesions. Hence, many physicians consider that radical surgeries are contraindicated for liver metastases from GC (LMGC). According to the 2017 Japanese treatment guideline for GC, a smaller number of liver metastases without unresectable factors may be an indication for liver resection (LR) with curability. The actual 5-year overall survival (OS) rate ranges from 0 to 0.37. AIM To present the institutional indications for LR for LMGC and identify important factors for prognostic outcomes. METHODS In total, 30 patients underwent LR for LMGC during a 14-year period, and we evaluated the clinical, surgical, and oncological findings. In all patients, radical surgery with intentional lymphadenectomy was performed for the primary GC. The median follow-up duration after the initial LR was 33.7 mo, and three patients with no recurrence died of causes unrelated to the LMGC. The OS and recurrence-free survival rates after the initial LR were assessed. RESULTS Seventeen patients had metachronous LMGC. The initial LR achieved curability in 29 patients. Perioperative chemotherapy was introduced in 23 patients. The median greatest LMGC dimension was 30 mm, and the median number of LMGC was two. Twenty-two patients had unilobular LMGC. The 5-year OS and recurrence-free survival rates were 0.48 and 0.28, respectively. The median survival duration and recurrence-free duration after the initial LR were 16.8 and 8.6 mo, respectively. Twenty-one patients developed recurrence after the initial LR. Additional surgeries for recurrence were performed in nine patients, and these surgeries clearly prolonged the patients’ survival. Pathological serosal invasion was an independent predictor of a poor prognostic outcome after the initial LR. Aggressive LR may be indicated for carefully selected patients with LMGC. CONCLUSION Our results of LR for LMGC seem acceptable. Additional surgeries for recurrence after the initial LR might prolong OS. Pathological serosal invasion is important for poor prognostic outcomes.

Background Gallbladder cancer (GBC) is the most common biliary malignancy and has the worst prognosis, but aggressive surgeries [e.g., resection of the extrahepatic bile duct (EHBD), major hepatectomy and lymph node (LN) dissection] may improve long-term survival. GBC may be suspected preoperatively, identified intraoperatively, or discovered incidentally on histopathology. Aim To present our data together with a discussion of the therapeutic strategies for GBC. Methods We retrospectively investigated nineteen GBC patients who underwent surgical treatment. Results Nearly all symptomatic patients had poor outcomes, while suspicious or incidental GBCs at early stages showed excellent outcomes without the need for two-stage surgery. Lymph nodes around the cystic duct were reliable sentinel nodes in suspicious/incidental GBCs. Intentional LN dissection and EHBD resection prevented metastases or recurrence in early-stage GBCs but not in advanced GBCs with metastatic LNs or invasion of the nerve plexus. All patients with positive surgical margins (e.g., the biliary cut surface) showed poor outcomes. Hepatectomies were performed in sixteen patients, nearly all of which were minor hepatectomies. Metastases were observed in the left-sided liver but not in the caudate lobe. We may need to reconsider the indications for major hepatectomy, minimizing its use except when it is required to accomplish negative bile duct margins. Only a few patients received neoadjuvant or adjuvant chemoradiation. There were significant differences in overall and disease-free survival between patients with stages ≤ IIB and ≥ IIIA disease. The median overall survival and disease-free survival were 1.66 and 0.79 years, respectively. Conclusion Outcomes for GBC patients remain unacceptable, and improved therapeutic strategies, including neoadjuvant chemotherapy, optimal surgery and adjuvant chemotherapy, should be considered for patients with advanced GBCs.

357 Background: Remnant gastric cancer (RGC) is defined as a cancer arising in the remnant stomach after initial gastrectomy, regardless of the previous disease or surgical procedure. Despite growing evidence of the effectiveness of minimally invasive surgery (MIS) for primary gastric cancer, MIS for RGC remains controversial due to the rarity of the disease. This study aimed to evaluate the surgical and oncological outcomes of MIS for radical resection of RGC. Methods: We collected the data of patients with RGC who underwent surgery with curative intent between 2005 and 2020 from 17 institutions in Japan, and performed a propensity score (PS) matching analysis to compare the short- and long-term outcomes of MIS with open surgery. Results: A total of 327 patients were included in this study and 186 patients were analyzed after matching. The risk ratios for overall and severe complications were 0.76 (95% confidence interval [CI]: 0.45–1.27) and 0.65 (95% CI: 0.32–1.29), respectively. The MIS group had significantly less blood loss (50 ml vs. 361 ml, P

Patient: Male, 70 Final Diagnosis: Metachronous pancreatic metastasis Symptoms: None Medication: — Clinical Procedure: Surgery Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Pancreatic metastasis from colorectal cancer is rare and can masquerade as primary pancreatic cancer. Case Report: A 70-year-old male was diagnosed with advanced rectal cancer with multiple liver metastases. After neoadjuvant chemotherapy, he underwent radical surgery for the primary tumor and hepatectomy for multiple liver metastases. Adjuvant chemotherapies and additional surgeries were subsequently required for recurrences in the liver, lung, and lymph nodes. A diffuse hypovascular nodule in the pancreatic head and a solitary liver metastasis were detected 2.5 years after the initial surgery and he accordingly underwent further chemotherapy. However, the pancreatic tumor progressed, invading the pancreatic duct and biliary tract. Obstructive jaundice finally prompted discontinuation of chemotherapy and he underwent biliary drainage. His diffuse and hypovascular tumor was clinically and radiographically diagnosed as a primary pancreatic cancer. Pancreatic resection for the pancreatic tumor and hepatectomy for the liver metastasis were performed 4.2 years after the initial surgery, achieving radiographic and surgical curative resection. Pathological examination of the surgical specimen resulted in a definitive diagnosis of metachronous pancreatic metastasis from his primary rectal cancer. Despite further chemotherapy, his general condition worsened; however, he remains alive 5.4 years after the initial surgery, with best supportive care. Conclusions: Pancreatic metastasis originating from rectal cancer can masquerade as primary pancreatic cancer clinically and radiologically. Multimodality treatment is mandatory for metastatic colorectal cancer. Aggressive surgeries for pancreatic metastasis should be considered if curative resection appears possible radiographically and/or intraoperatively.

BACKGROUND Postoperative pancreatic leakage readily results in intractable pancreatic fistula and subsequent intraperitoneal abscess. This refractory complication can be fatal; therefore, intensive treatment is important. Continuous local lavage (CLL) has recently been reevaluated as effective treatment for severe infected pancreatitis, and we report three patients with postoperative intractable pancreatic fistula successfully treated by CLL. We also discuss our institutional protocol for CLL for postoperative pancreatic fistula. CASE SUMMARY The first patient underwent subtotal stomach-preserving pancreaticoduodenectomy, and pancreatic leakage was observed postoperatively. Intractable pancreatic fistula led to intraperitoneal abscess, and CLL near the pancreaticojejunostomy site was instituted from postoperative day (POD) 8. The abscess resolved after 7 d of CLL. The second patient underwent distal pancreatectomy. Pancreatic leakage was observed, and intractable pancreatic fistula led to intraperitoneal abscess near the pancreatic stump. CLL was instituted from POD 9, and the abscess resolved after 4 d of CLL. The third patient underwent aneurysmectomy and splenectomy with wide exposure of the pancreatic parenchyma. Endoscopic retrograde pancreatic drainage was performed on POD 15 to treat pancreatic fistula; however, intraperitoneal abscess was detected on POD 59. We performed CLL endoscopically via the transgastric route because the percutaneous approach was difficult. CLL was instituted from POD 63, and the abscess resolved after 1 wk of CLL. CONCLUSION CLL has therapeutic potential for postoperative pancreatic fistula.

BACKGROUND Aneurysm of the inferior pancreaticoduodenal artery (IPDA) is rare among visceral artery aneurysms. Aneurysm and/or pancreatitis may have a causal relation with hemosuccus pancreaticus (HP). HP causes an obscure bleeding in the digestive tract, and this rare disease may lead to life-threatening condition. Although interventional radiology is generally employed as the initial treatment for visceral aneurysms, aneurysmic recanalization is a critical problem. CASE REPORT A 58-year-old male was incidentally diagnosed as groove pancreatitis, and his pancreatitis was successfully treated by conservative management. One year later, an IPDA aneurysm was detected in image studies. Gastrointestinal bleeding was objectively observed, and a diagnosis of asymptomatic HP was made. Arterio-pancreatic duct fistula was suspected, but was not identified. Coil embolization was successfully completed. Six months later, he suffered a relapse of HP, and visited our emergency unit. Pseudocystic lesion around metallic coils were confirmed. Subtotal stomach-preserving pancreaticoduodenectomy without any extended resections was performed. Intentional dissections of nerve plexuses and lymph nodes were all waived. Even a pancreatography of the resected specimen did not clarify his arterio-pancreatic duct fistula. He was discharged at postoperative day 10, and smoothly returned to his work. CONCLUSIONS Pancreatic juice-related complications after advanced pancreaticoduodenectomy for malignancies are often intractable. However, simple pancreaticoduodenectomy which omits extended resections and intentional dissections is safe and feasible for benign diseases. After the initial interventional radiology for pancreatic aneurysms, an elective pancreatic surgery should be considered to avoid unwanted recanalization and refractory HP.

Case series Patients: Female, 53-year-old • Female, 70-year-old • Female, 53-year-old • Female, 48-year-old • Male, 66-year-old • Female, 43-year-old • Male, 76-year-old Final Diagnosis: Local recurrence Symptoms: Asymptomatic Medication: — Clinical Procedure: Surgical resection Specialty: Surgery Objective: Unusual setting of medical care Background: Advanced malignancies in the lower abdomen easily invade the retroperitoneal and pelvic space and often metastasize to the paraaortic and pelvic lymph nodes (LNs), resulting in paraaortic and/or pelvic tumor (PPT). Case Reports: A total of 7 cases of aggressive malignant PPT resection and orthotopic replacement of the abdominal aorta and/or iliac arteries with synthetic arterial graft (SAG) were experienced during 16 years. We present our experience with aggressive resection of malignant PPTs accompanied by arterial reconstruction with SAG in detail. The primary diseases included 2 cases endometrial cancer and 2 cases of rectal cancer, and 1 case each of ovarian carcinosarcoma, vaginal malignant melanoma, and sigmoid cancer. Surgical procedures are described in detail. Briefly, the abdominal aorta and iliac arteries were anastomosed to the SAG by continuous running suture using unabsorbent polypropylene. Five Y-shaped and 2 I-shaped SAGs were used. This en bloc resection actually provided safe surgical margins, and tumor exposures were not pathologically observed in the cut surfaces. Graphical and surgical curability were obtained in all cases in which aggressive malignant PPT resections were performed. The short-term postoperative course of our patients was uneventful. From a vascular perspective, the SAGs remained patent over the long term after surgery, and long-term oncologic outcomes were satisfactory. Conclusions: To our knowledge, this case series is the first report of aggressive malignant PPT resection accompanied by arterial reconstruction with SAG. This procedure is safe and feasible, shows curative potential, and may play a role in multidisciplinary management of malignant PPTs.

Introduction and importance The most common liver malignancies are hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic tumors. Hepatocellular carcinoma and intrahepatic cholangiocarcinoma may invade the portal vein (PV). An association between diffuse large B-cell lymphoma (DLBCL) and primary biliary cholangitis (PBC) remains unclear. We herein report a thought-provoking case of a difficult-to-diagnose liver tumor with PV thrombosis in a PBC patient. Presentation of case A 66-year-old woman had PBC, systemic sclerosis, diabetes, and osteoporosis. A solitary liver tumor accompanied by macrovascular thrombosis in the PV was detected incidentally. Based on dynamic imaging findings, we considered the tumor to be intrahepatic cholangiocarcinoma, and right lobectomy with lymphadenectomy was performed. Unexpectedly, pathological assessment made a definitive diagnosis of DLBCL that did not invade the vessels and bile duct. In fluorine-18-fluorodeoxyglucose positron emission tomography, abnormal accumulations were clearly observed in the breast tissue and peritracheal, parasternal, mediastinal, and pericardial lymph nodes. The patient achieved complete remission after systemic chemotherapy, and there has been no recurrence 3 years after surgery. Clinical discussion Primary lymphoma in the liver is rare, and we did not consider our patient's tumor as primary liver lymphoma. Our case actually showed no tumor thrombosis in the PV. Although autoimmune disorders may increase the risk of non-Hodgkin's lymphoma, an association between DLBCL and PBC is still unclear, and we must remember that DLBCL may develop rarely in a PBC patient. Conclusion Our case report provides a timely reminder for clinicians and surgeons in the fields of hepatology and hematology., Highlights • The most frequent liver neoplasms are HCC, ICC, and metastatic tumors. • HCCs often cause portal thrombosis. • Primary liver lymphoma is rare, and generally does not invade the vessels. • An association between diffuse large B-cell lymphoma and PBC remains unclear. • We describe a thought-provoking case of a difficult-to-diagnose liver tumor in PBC.

BACKGROUND Although arterial hemorrhage after pancreaticoduodenectomy (PD) is not frequent, it is fatal. Arterial hemorrhage is caused by pseudoaneurysm rupture, and the gastroduodenal artery stump and hepatic artery (HA) are frequent culprit vessels. Diagnostic procedures and imaging modalities are associated with certain difficulties. Simultaneous accomplishment of complete hemostasis and HA flow preservation is difficult after PD. Although complete hemostasis may be obtained by endovascular treatment (EVT) or surgery, liver infarction caused by hepatic ischemia and/or liver abscesses caused by biliary ischemia may occur. We herein discuss therapeutic options for fatal arterial hemorrhage after PD. AIM To present our data here along with a discussion of therapeutic strategies for fatal arterial hemorrhage after PD. METHODS We retrospectively investigated 16 patients who developed arterial hemorrhage after PD. The patients’ clinical characteristics, diagnostic procedures, actual treatments [transcatheter arterial embolization (TAE), stent-graft placement, or surgery], clinical courses, and outcomes were evaluated. RESULTS The frequency of arterial hemorrhage after PD was 5.5%. Pancreatic leakage was observed in 12 patients. The onset of hemorrhage occurred at a median of 18 d after PD. Sentinel bleeding was observed in five patients. The initial EVT procedures were stent-graft placement in seven patients, TAE in six patients, and combined therapy in two patients. The rate of technical success of the initial EVT was 75.0%, and additional EVTs were performed in four patients. Surgical approaches including arterioportal shunting were performed in eight patients. Liver infarction was observed in two patients after TAE. Two patients showed a poor outcome even after successful EVT. These four patients with poor clinical courses and outcomes had a poor clinical condition before EVT. Fourteen patients were successfully treated. CONCLUSION Transcatheter placement of a covered stent may be useful for simultaneous accomplishment of complete hemostasis and HA flow preservation.

When performing pancreaticoduodenectomy with resection of the confluence of the superior mesenteric vein and portal vein, division of the splenic vein may cause sinistral portal hypertension resulting in gastrointestinal bleeding, splenic congestion, and hypersplenism. To prevent these adverse events, it is important to intentionally decompress the splenic vein. This report is of a 68-year-old woman with stage IA carcinoma of the head of the pancreas who survived for more than six years following tumor resection and pancreaticoduodenectomy and distal splenorenal shunt. A 68-year-old woman was diagnosed with carcinoma of the head of the pancreas that involved the confluence of the superior mesenteric vein, portal vein, and splenic vein. No unresectable cancer sites or distant metastases were detected. Pancreaticoduodenectomy with resection of the confluence of the superior mesenteric vein and portal vein was performed. The superior mesenteric vein and portal vein were anastomosed in the end-to-end fashion, and the remnant splenic vein was anastomosed to the superior aspect of the left renal vein in the end-to-side fashion. At 22 months after the initial surgery, the patient underwent partial lung resection for a metachronous lung metastasis. For 6 years after the initial surgery, the venous reconstructions have maintained their patency without any obstruction of splenic venous flow, and the patient has remained in good health without further metastases or recurrences. This case has shown the importance of early diagnosis of carcinoma of the head of the pancreas, as appropriate and timely surgical management can result in good outcome. This patient responded well and remains alive six years following pancreaticoduodenectomy and preservation of the spleen with the use of a distal splenorenal shunt.

Patient: Male, 70 Final Diagnosis: Labial fistula Symptoms: Intractable discharge Medication: — Clinical Procedure: Intraluminal drainage via a rectus abdominis musculocutaneous flap Specialty: Surgery Objective: Unusual setting of medical care Background: Anastomotic failure after gastroenterological surgery is usually treated by intraperitoneal drainage and a mature ductal fistula. A ductal fistula may develop into a labial fistula. Although a ductal fistula is controllable, a labial fistula is intractable. We report a case of a labial fistula that communicated with the duodenal stump after gastrectomy. This condition was successfully treated by intraluminal drainage with continuous suction (IDCS) via a rectus abdominis musculocutaneous flap (RAMF). Case Report: A 70-year-old male underwent distal gastrectomy with intentional lymphadenectomy because of advanced gastric cancer. Digestive reconstruction was completed by the Billroth II method. Pancreatic leakage, intraperitoneal abscess, and anastomotic failure of gastrojejunostomy occurred after surgery. The duodenal stump was ruptured at postoperative day (POD) 26, and ductal fistula associated with the duodenum was observed. Unfortunately, this ductal fistula developed into a labial fistula at POD 90, and a high output of duodenal juice was observed. Additional surgery was proposed at POD 161. The broken stump and labial fistula were covered by a pedunculated RAMF, and a dual drainage system (a combination of a Penrose drain and a 2-way tube) travelled through the RAMF. The tip position of the drainage system was located in the duodenum, and the IDCS was effectively introduced. The secondary ductal fistula finally matured through the RAMF, and was subsequently closed at POD 231. The intractable labial fistula was successfully treated, and the patient was discharged at POD 235. Conclusions: A high-output labial fistula, which communicated with the duodenal stump after gastrectomy, was refractory in our patient. Effective IDCS through an RAMF was useful for replacement of the labial fistula with a secondary ductal fistula.

Patient: Male, 70 Final Diagnosis: Splenic artery aneurysm Symptoms: Asymptomatic Medication: — Clinical Procedure: Surgery and Endoscopy Specialty: Surgery Objective: Unknown ethiology Background: Visceral arterial aneurysms are rare. Most splenic arterial aneurysms (SAAs) are saccular and are in the distal third of the splenic artery. Suggested major causes of SAAs are atherosclerosis, pregnancy, and inflammation. We report the case of a patient who with a SAA extending almost the full length of his splenic artery. Case Report: A solitary true aneurysm that extended almost the entire length of the splenic artery was incidentally detected in an asymptomatic 70-year-old male patient with a history of myasthenia gravis and diabetes mellitus. His SAA was severely calcified, but other arteries showed no calcification. The aneurysm had been slightly enlarged toward the celiac artery for 2 years, and aneurysmectomy and splenectomy were performed. Vascular clips were carefully placed at the intact splenic artery without disturbing arterial flows from the celiac artery. Arterial branch from the SAA was ligated at an intact area, and the pancreatic capsule was densely adherent with the calcified aneurysm wall. The pancreas was preserved, although the pancreatic parenchyma was widely exposed during aneurysmectomy. Pathological examination revealed no atherosclerotic changes. Postoperatively, a pancreatic fistula developed, which was treated by placing an intraperitoneal drain and retrograde pancreatic drainage tube. Nevertheless, the intractable pancreatic fistula triggered a bacteriogenic infection, resulting in intraperitoneal abscess. Continuous local lavage via transnasal continuous infusion and endoscopic transgastric drainage was performed, until the fistula closed. He was healthy at 9 months after surgery. Conclusions: A SAA that had the rare form and solitary origin was treated. Continuous local lavage has a therapeutic potential for a pancreatic juice-related bacteriogenic complication.

Highlights • Diagnosis of functioning neuroendocrine neoplasms (NENs) in the pancreas is challenging. • Adrenocorticotropic hormone (ACTH) regulates adrenal cortisol production. • Functioning NENs may cause hypercortisolism as a result of ectopic ACTH secretion. • Systematic endocrine examination and functional imaging studies are vital. • Making a precise diagnosis enables appropriate treatment of NENs., Introduction and importance Definitive diagnosis of functioning neuroendocrine neoplasms (NENs) in the pancreas is challenging. Adrenocorticotropic hormone (ACTH) regulates adrenal cortisol production. Ectopic ACTH secretion by functioning NENs may cause hypercortisolism. Presentation of case A 62-year-old woman who was receiving medications for hypertension and hyperlipidemia was referred to our hospital because of abnormal blood tests. Diabetes mellitus was initially diagnosed. Dynamic computed tomography and endoscopic ultrasound revealed a 35-mm diameter hypovascular tumor in the distal pancreas and multiple liver metastases. Endoscopic ultrasound-guided fine-needle aspiration resulted in a diagnosis of neuroendocrine carcinoma. The patient developed pancreatic leakage progressing to peritonitis, abscess formation, pleural effusion, and ascites after the fine-needle aspiration biopsy. Her clinical condition deteriorated to a septic state, necessitating emergency surgery comprising distal pancreatectomy, intraperitoneal lavage, and drainage. Wound healing was protracted and accompanied by ongoing high white blood cell counts and neutrophilia. She also developed a gastric ulcer postoperatively. Systematic endocrine investigations were performed because hypercortisolism caused by a functioning NEN was suspected. Eventually, a definitive diagnosis of an ACTH-producing NEN in the pancreas was made. Systemic chemotherapy was proposed; however, the patient and her family opted for palliative treatment only. She died 42 days after the initial diagnosis. Clinical discussion We here present a patient with ACTH-dependent hypercortisolism attributable to a pancreatic NEN who died of progressive cancer after a delay in definitive diagnosis. Conclusion Detailed investigation, including systematic endocrine examination and functional imaging studies, are important for precise diagnosis of, and appropriate treatment for, NENs.

We report an 82-year-old man who developed ventricular tachycardia and Torsades de Pointes (TdP) after oral administration of garenoxacin, a novel quinolone antibiotic agent that differs from the third-generation quinolones, for pneumonia. He had hypokalemia (K 2.3 mmol/L) induced by licorice and also had received disopyramide for arrhythmia, bicalutamide for prostate cancer, and silodosin for prostate hypertrophy. After taking him off all drugs and administering spironolactone supplemented with potassium, his low serum potassium level was ameliorated. Therefore, although garenoxacin reportedly causes fewer adverse reactions for cardiac rhythms than third-generation quinolone antibiotics, one must be cautious of the interference of other drugs during hypokalemia in order to prevent TdP.

A 59-year-old woman with ulcerative colitis developed red eyes, pleural effusion, eosinophilia and urinary abnormalities after restarting of sulphasalazine treatment. Light microscopy of a kidney biopsy revealed segmental necrotizing glomerulonephritis without deposition of immunoglobulin or complement. Proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) titer was elevated at 183 ELISA units (EU) in sera (normal range less than 10 EU), myeloperoxidase-ANCA was negative. PR3-ANCA titer was 250 and 1,070 EU in pleural effusions on right and left side, respectively. Although cessation of sulphasalazine treatment resulted in improvements in fever, red eyes, chest pain, titer of C-reactive protein and volume of the pleural effusions, we initiated steroid therapy, because PR3-ANCA titer rose to 320 EU, eosinophil count increased to 1,100 cells/microl, and the pleural effusion remained. One month after steroid therapy, the pleural effusion disappeared, and PR3-ANCA titer normalized 3 months later. This case suggests that sulphasalazine can induce PR3-ANCA-positive necrotizing glomerulonephritis.

A 61-year-old Japanese woman with rapidly progressive glomerulonephritis exhibited both anti-glomerular basement membrane (GBM) antibodies (920 EU) and myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA; 66 EU). Multiple plasma exchanges with fresh frozen plasma preceded by 500 mg/day intravenous methylprednisolone and 30 mg/day oral prednisolone decreased anti-GBM antibody and MPO-ANCA antibody titers to 106 EU and below 10 EU (normal ranges), respectively. Thrombotic thrombocytopenic purpura (TTP) manifests itself as a moderate decrease in the activity of disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13 (ADAMTS13) protein levels to 35% of normal; ADAMTS13 deficiency is only symptomatic when levels are less than 50%. This patient’s disease was resistant to extensive plasma exchange, leading to her death from respiratory distress and perforation of the alimentary tract secondary to cytomegalovirus infection. Autopsy demonstrated severe crescentic glomerulonephritis associated with linear IgG, IgA, IgM, and C3 deposits along the glomerular capillary walls. This case is an uncommon example of combined double antibody-positive crescentic glomerulonephritis and refractory TTP.

A 61-year-old Japanese man with nephrotic syndrome due to focal segmental glomerulosclerosis was initially responding well to steroid therapy. The amount of daily urinary protein decreased from 15.6 to 2.8 g. Within 14 days of the oral bisphosphonate (alendronate sodium) administration, the amount of daily urinary protein increased rapidly up to 12.8 g with acute renal failure. After discontinuing the oral alendronate, the patient underwent six cycles of hemodialysis and four cycles of LDL apheresis. Urinary volume and serum creatinine levels recovered to the normal range, with urinary protein disappearing completely within 40 days. This report demonstrates that not only intravenous, but also oral bisphosphonates can aggravate proteinuria and acute renal failure.