Total pancreatectomy (TP) has attracted increasing attention because short-term outcomes have improved. However, long-term data remain limited. This study evaluated long-term outcomes after TP, focusing on actual 10-year survivors and causes of death. This study included patients who underwent elective TP for pancreatic disease and were followed for at least 10 years or until death. Patients were classified as 10-year survivors or non-survivors. Clinicopathological characteristics, long-term survival, and causes of death were analyzed. Forty-nine patients were included. Single-stage TP was performed in 30 patients (61.2

Pancreatic head tumors are usually regarded as primary pancreatic neoplasms; however, metastatic lymph nodes from gastrointestinal malignancies may occasionally mimic pancreatic tumors and lead to diagnostic and therapeutic challenges, particularly when the primary lesion cannot be identified preoperatively. In the present case report, a 76-year-old man was referred for evaluation of a pancreatic head mass with elevated serum carbohydrate antigen 19-9 levels. Contrast-enhanced computed tomography and positron emission tomography-computed tomography demonstrated a protruding lesion in the pancreatic head and an enlarged infrapyloric lymph node, without evidence of a primary gastrointestinal tumor. Esophagogastroduodenoscopy was unremarkable and primary pancreatic cancer was suspected. Endoscopic ultrasound-guided biopsy of the infrapyloric lymph node revealed adenocarcinoma; however, the primary site could not be determined. Pancreaticoduodenectomy was planned. Intraoperatively, the lesion appeared to represent a bulky metastatic lymph node rather than a primary pancreatic tumor, and distal gastrectomy was additionally performed. Histopathological examination confirmed metastatic lymph node involvement from occult gastric cancer. The patient remains alive without evidence of recurrence 2 years after surgery. In conclusion, occult gastric cancer metastasis can masquerade as a pancreatic head tumor. Awareness of this diagnostic and surgical challenge may facilitate appropriate intraoperative reassessment when pancreatic head lesions are accompanied by suspicious perigastric lymphadenopathy.

Dedifferentiated liposarcoma (DDLPS), a common subtype of retroperitoneal soft tissue sarcoma, generally shows a limited response to conventional chemotherapy. Recently, immune checkpoint inhibitors (ICIs) have demonstrated promising activity against DDLPS. However, predictive biomarkers for response remain unclear. We present a case of recurrent retroperitoneal DDLPS with a high tumor mutational burden (TMB), demonstrating a pathological complete response (pCR) to pembrolizumab. We also explore the association between the response to ICIs and the tumor microenvironment, including programmed death-ligand 1 (PD-L1), tumor-infiltrating lymphocytes (TILs), and tumor-associated macrophages (TAMs). A 73-year-old man undergoing hormone therapy for metastatic prostate cancer was incidentally found to have a multilobulated retroperitoneal tumor in the right iliac fossa during routine follow-up imaging. He underwent macroscopically complete removal of the tumor, which was histologically diagnosed as DDLPS. Eight months later, recurrence with peritoneal dissemination was detected. He received doxorubicin (6 cycles) followed by pazopanib (13 weeks) and eribulin (3 cycles). The response to all these treatments was progressive disease. Comprehensive genomic profiling of the primary tumor revealed high TMB (13 mutations/megabase). Pembrolizumab 400 mg was administered every 6 weeks. After 11 cycles, imaging revealed a partial response with a 56.7

Background: We investigated the rational extent of regional lymphadenectomy and evaluated the prognostic impact of number-based regional nodal classification in patients with distal cholangiocarcinoma. Methods: This study included 191 patients with distal cholangiocarcinoma who underwent pancreaticoduodenectomy. The nos. 8, 12a-b-c-p, 13, 14, and 17 nodes were dissected routinely. The impact of the extent of lymphadenectomy on prognostic stratification performed using number-based nodal classification was evaluated. Results: The incidence of metastasis in the routinely dissected nodes was 1.0-25.7%, with 5-year overall survival of 0-36.4% in patients with metastasis. The incidence of metastasis in the no. 12p nodes, which were not included in regional nodes in the American Joint Committee on Cancer or International Union Against Cancer staging systems, was 5.8% with a 5-year overall survival of 36.4% in patients with metastasis. When our dissected nodes were adopted (P < .001), number-based nodal classification predicted overall survival better than when regional nodes defined by the International Union Against Cancer or American Joint Committee on Cancer staging systems were used (nos. 8, 12a-b, 13, 14, and 17 nodes with or without no. 9 nodes; P = .004 each). The 5-year overall survival in patients with pN0, pN1 (1-3 positive nodes), and pN2 (>= 4 positive nodes) disease was 57.4%, 37.3%, and 13.6%, respectively (P < .001). The pN classification was an independent prognostic factor (pN1, P = .009; pN2, P < .001). Conclusion: The nos. 8, 12a-b-c-p, 13, 14, and 17 nodes should be prioritized as the rational extent of regional lymphadenectomy for distal cholangiocarcinoma for accurate staging. Number-based regional nodal classification is suitable for prognostic stratification. (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

Background This study aimed to clarify surgical outcomes for patients with pT3 gallbladder cancer according to the mode of tumor spread. Methods A total of 85 patients with pT3 gallbladder cancer who underwent curative-intent surgery were analyzed. Each tumor was classified according to the mode of spread. Results Five-year overall survival (OS) in patients with involvement of the liver alone (n = 25), involvement of the extrahepatic bile duct alone (n = 29), involvement of one other organ/structure alone or perforation of the peritoneal side of the serosa alone (n = 9), and involvement of both the liver and one other organ/structure (n = 22) was 36.0%, 29.0%, 22.2%, and 9.1%, respectively. For patients with involvement of the liver alone, 5-year OS was > 30% regardless of type of hepatectomy. Among 29 patients with involvement of the extrahepatic bile duct alone, 8 patients survived ≥ 5 years; 3 underwent extended cholecystectomy and 5 underwent more extensive resection. Despite performing extensive resection, 5-year OS was 12.5% for patients with involvement of both the liver and one other organ/structure. The mode of spread other than involvement of the liver alone or extrahepatic bile duct alone was an independent predictor of worse OS (hazard ratio 1.675; p = 0.046). Conclusions Surgery is an acceptable option for pT3 gallbladder cancer with involvement of the liver alone or extrahepatic bile duct alone regardless of the type of resection procedure. However, the survival benefit of surgery is limited for this tumor with other modes of spread.

The patient was a 61-year-old man with a diagnosis of carcinoma of the pancreatic head. Abdominal computed tomography( CT)showed no distant metastasis, and he underwent subtotal stomach-preserving pancreatoduodenectomy. Immediately after surgery, he received liver perfusion chemotherapy with 5-fluorouracil followed by systemic gemcitabine. Eighteen months after surgery, CT revealed liver metastasis in the S6 segment, and partial hepatectomy was performed. The pathological diagnosis was liver metastasis of pancreatic cancer. Postoperatively, the patient was treated with gemcitabine and S-1 therapy for 1 year and then switched to S-1 monotherapy for about 6 months. Four years after the initial surgery, CT showed 2 metastases in the right lung. After 2 months of S-1 monotherapy, wedge resection of the upper and lower lobes of the right lung was performed. Gemcitabine and nab-paclitaxel therapy were administered, after the metastasectomy, but pleural dissemination appeared on CT 5 years after the initial surgery. Modified FOLFIRINOX therapy was started and continued for 8 months, but CT revealed further disseminated lesions in the diaphragm. Palliative irradiation was provided, but the disease gradually progressed. After multidisciplinary treatment, the patient survived for 6 years and 3 months after the initial surgery.

We report a case of biliary cystadenocarcinoma in which long-term survival was achieved after 2 operations for intrahepatic recurrence. A 72-year-old man with biliary cystadenocarcinoma located mainly in segment 3 of the liver underwent left hepatectomy, extrahepatic bile duct resection, and lymph node dissection. Seven years and 9 months after the initial resection, he underwent partial liver resection(segment 5)for intrahepatic recurrence detected by computed tomography. Fifteen years and 7 months after the initial resection, he underwent repeat partial resection of the liver(segment 5)for intrahepatic recurrence. Histologically, these tumors were confirmed to be recurrence of biliary cystadenocarcinoma. He remains alive and well with no further recurrence 21 years and 6 months after the initial resection. This case and a literature review suggest that hepatic resection is a useful treatment option for intrahepatic recurrence of biliary cystadenocarcinoma.

A 58-year-old woman presented with a complaint of weight loss. Abdominal computed tomography showed dilatation of the biliary and pancreatic ducts and a mural nodule in the pancreatic duct. The diagnosis was intraductal papillary mucinous neoplasm(IPMN). Endoscopic retrograde cholangiopancreatography(ERCP)and cholangioscopy revealed a fistula between the common bile duct and the IPMN. A sudden increase in hepatobiliary enzymes was noted preoperatively. ERCP showed that the common bile duct was obstructed by mucus. A nasobiliary drainage tube was inserted into the bile duct endoscopically and kept open by daily tube washing, and the liver dysfunction improved. Total pancreatectomy, splenectomy, and regional lymph node dissection were performed. Histological examination confirmed that the primary tumor was mixed invasive intraductal papillary mucinous adenocarcinoma. The patient remains alive and well with no evidence of recurrence 18 months after resection.

A 66-year-old man was referred to our hospital with fever and abdominal pain. CT showed a mass in the intrapancreatic bile duct but no wall thickness in the perihilar bile ducts. Neither regional lymphadenopathy nor distant metastasis was observed. Biliary cytology showed adenocarcinoma. The diagnosis was distal cholangiocarcinoma, and pancreatoduodenectomy was performed. Intraoperative frozen section examination of the ductal resection margins at the right and left hepatic ducts was positive for carcinoma in situ, and the operation ultimately completed with R1 resection. Histological examination confirmed a diagnosis of cholangiocarcinoma with superficial spread and a single positive lymph node. Adjuvant chemotherapy with S-1 was administered for 1 year. Anastomotic recurrence at the hepaticojejunostomy was found 5 years after resection; biopsy specimens revealed adenocarcinoma. Thereafter, S-1 chemotherapy was resumed, and the patient remains alive and well 9 years and 1 month after resection.

We report a case of intrahepatic cholangiocarcinoma(ICC)with lymph node metastases in which long-term survival was achieved after surgery followed by chemotherapy. A 69-year-old man underwent left hepatectomy, extrahepatic bile duct resection, and lymph node dissection for ICC located mainly in segment 4 of the liver with enlarged lymph nodes in the hepatoduodenal ligament. The histopathologically confirmed diagnosis was ICC(T2N1M0, Stage ⅣA)with 3 positive lymph nodes(No. 12a1, No. 12p1, and No. 12p2). He received chemotherapy with gemcitabine(GEM)plus cisplatin(CDDP)for 9 months, followed by GEM monotherapy for 4 months, and then S-1 monotherapy was started. A right lung nodule was detected 12 months after the initiation of S-1 monotherapy. He received GEM plus S-1 therapy for 28 months, followed by S-1 monotherapy, leading to disappearance of the lung nodule. He remains alive and well without disease 78 months after surgery. Our experience in this case suggests that radical resection followed by chemotherapy may provide a survival benefit in selected patients who have ICC with nodal disease.

Purpose This study aimed to elucidate the impact of anatomic location of residual disease (RD) after initial cholecystectomy on survival following re-resection of incidental gallbladder cancer (IGBC). Methods Patients with pT2 or pT3 gallbladder cancer (36 with IGBC and 171 with non-IGBC) who underwent resection were analyzed. Patients with IGBC were classified as follows according to the anatomic location of RD after initial cholecystectomy: no RD (group 1); RD in the gallbladder bed, stump of the cystic duct, and/or regional lymph nodes (group 2); and RD in the extrahepatic bile duct and/or distant sites (group 3). Results Timing of resection (IGBC vs. non-IGBC) did not affect survival in either multivariate or propensity score matching analysis. RD was found in 16 (44.4%) of the 36 patients with IGBC; R0 resection following re-resection was achieved in 32 patients (88.9%). Overall survival (OS) following re-resection was worse in group 3 (n = 7; 5-year OS, 14.3%) than in group 2 (n = 9; 5-year OS, 55.6%) (p = 0.035) or in group 1 (n = 20; 5-year OS, 88.7%) (p < 0.001). There was no survival difference between groups 1 and 2 (p = 0.256). Anatomic location of RD was independently associated with OS (group 2, HR 2.425, p = 0.223; group 3, HR 9.627, p = 0.024). Conclusion The anatomic location of RD independently predicts survival following re-resection, which is effective for locoregional disease control in IGBC, similar to resection for non-IGBC. Not all patients with RD have poor survival following re-resection for IGBC.

Primary synovial sarcoma of the spermatic cord is quite rare and has not been reported in Japanese literature. We report a case of primary synovial sarcoma of the spermatic cord and localized dissemination of the tumor in a patient who experienced recurrence of intra-abdominal bleeding 7 years after curative resection of the primary lesion. A 70-year-old man was admitted with disturbance on urination and lower abdominal pain. Computed tomography (CT) of the abdomen revealed two lesions:a 10-cm intrapelvic tumor with hemorrhage and a 4-cm tumor adjacent to the bladder. Curative excision of the tumors was performed. Histological examination revealed that the larger lesion was a primary tumor of the spermatic cord with proliferation of spindle cells in cellular fascicles in a monotonous pattern, which was compatible with histologic findings of monophasic fibrous synovial sarcoma. The smaller lesion was a disseminated tumor. The diagnosis of synovial sarcoma was confirmed by the detection of a SS18 (SYT) -SSX1 fusion gene. After discharge, the patient received adjuvant chemotherapy, including ifosfamide and doxorubicin. No recurrence was evident thereafter. Seven years after the operation, the patient experienced sudden abdominal pain and swelling and was transferred to our hospital. CT showed a 17-cm tumor with massive hemorrhage in the omental bursa. Through catheterization of the superior mesenteric artery, bleeding from a branch of the dorsal pancreatic artery was identified. Because of the difficulty of catheterizing the bleeding branch, he underwent emergency resection of the tumor and partial resection of the colon. Histologic examination and genetic testing revealed that the tumor was a recurrence of the synovial sarcoma. After discharge, the patient received treatment with gemcitabine and docetaxel. However, 7 months after the second surgery, intraperitoneal manifestations recurred. The patient died 14 months after the second resection. This case suggests that curative surgical resection of the primary synovial sarcoma of the spermatic cord contributes to prolonged survival. However, because the recurrence rate of synovial sarcoma is high, multidisciplinary treatment, including chemotherapy and radiotherapy, might be necessary.

Purpose Outcomes following surgery for advanced gallbladder carcinoma remain unsatisfactory. This study aimed to determine the surgical outcome and effectiveness of adjuvant chemotherapy according to TNM stage in patients with gallbladder carcinoma. Methods A total of 200 patients undergoing surgery for gallbladder carcinoma were enrolled. Clinicopathological data were evaluated and surgical outcomes were compared between patients with and without adjuvant chemotherapy according to TNM stage. Results The 5-year overall survival (OS) after resection for patients with stage I (n = 27), IIA (n = 18), IIB (n = 28), IIIA (n = 25), IIIB (n = 43), IVA (n = 7), and IVB (n = 52) disease was 90.8%, 94.4%, 73.6%, 33.7%, 57.7%, 14.3%, and 11.8%, respectively (p < 0.001). R0 resection was performed in all patients with stage I or II disease, in 89.7% of those with stage III disease, and 69.5% of those with stage IV disease. For patients with stage III disease, adjuvant chemotherapy was associated with improved OS (5-year OS, 60.9% vs. 41.1%; p = 0.028) and was an independent prognostic factor (hazard ratio, 2.045; p = 0.039). For patients with stage IV disease, adjuvant chemotherapy appeared to affect OS (5-year OS, 25.1% vs. 5.3%; p = 0.041); R0 resection (hazard ratio, 1.882; p = 0.040) was the only independent prognostic factor. Conclusion TNM stage clearly predicts survival after resection of gallbladder carcinoma. R0 resection with adjuvant chemotherapy is recommended for long-term survival in the multimodal management of patients with stage III or IV gallbladder carcinoma.

•Living donor liver transplantation was an effective treatment for more than 30-year survived patients after Kasai operation.•Repeat cholangitis and delivery were the risk factors for late native liver failure after Kasai operation.•Limited availability of living donor was the problem for adult post-Kasai liver failure.

A 68-year-old male patient received a living donor kidney transplantation 8 years earlier for end-stage kidney disease secondary to IgA nephropathy. His post-transplantation follow-up had been routinely performed with laboratory examinations, ultrasound, and computed tomography (CT). His kidney graft function had been excellent and stable, as shown by a baseline serum creatinine level of 1.0 mg/dL. At referral, regular follow-up ultrasound and CT showed allograft hydroureteronephrosis. He did not have any complaints, but his physical examination revealed right inguinal bulging that was 3.5 × 3.5 cm. Abdominal enhanced CT revealed transplant allograft hydroureteronephrosis due to ipsilateral herniation of ureteroneocystostomy into the right inguinal canal. His serum creatinine level was slightly elevated (1.1 mg/dL). Then, he underwent an open right inguinal hernia repair. Paraperitoneal allograft hydroureteronephrosis and bladder herniation was confirmed at surgery, and hernioplasty with polypropylene mesh reinforcement was successfully performed. The postoperative course was uneventful. He was discharged on the seventh day after surgery. Six weeks after surgery, CT revealed disappearance of allograft hydroureteronephrosis and no sign of inguinal hernia recurrence with the serum creatinine stable at 1.0 mg/dL. Transplant ureteral obstruction due to inguinal hernia is a rare complication after kidney transplantation. However, transplant ureter or bladder herniation should be considered in the differential diagnosis of graft hydroureteronephrosis for preventing allograft loss.

Solitary fibrous tumor (SFT), a mesenchymal fibroblastic tumor with a hypervascular nature, rarely develops in the pelvis. Resection of a giant SFT occupying the pelvic cavity poses an increased risk of developing massive hemorrhage during resection, although surgical resection is the most effective treatment method for this tumor to achieve a potential cure. SFT rarely develops with Doege–Potter syndrome, which is known as a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia (NICTH) secondary to SFT that secretes insulin-like growth factor-II (IGF-II). We present a case of a giant pelvic SFT with Doege–Potter syndrome, which was successfully treated with transcatheter arterial embolization (TAE) followed by surgical resection. A 46-year-old woman presented with a disorder of consciousness due to refractory hypoglycemia. Images of the pelvis showed a giant and heterogeneously hypervascular mass displacing and compressing the rectum. Endocrinological evaluation revealed low serum levels of insulin and C-peptide consistent with NICTH. Angiography identified both the inferior mesenteric artery and the bilateral internal iliac artery as the main feeders of the tumor. To avoid intraoperative massive bleeding, super-selective TAE was performed for the tumor 2 days prior to surgery. Hypoglycemia disappeared after TAE. The tumor was resected completely, with no massive hemorrhage during resection. Histologically, it was diagnosed as IGF-II-secreting SFT. Partial necrosis of the rectum in the specimen was observed due to TAE. The patient was followed up for 2 years and no evidence of disease has been reported. Preoperative angiography followed by TAE is an exceedingly helpful method to reduce intraoperative hemorrhage when planning to resect SFT occupying the pelvic cavity. Complications related to ischemia should be kept in mind after TAE, which needs to be planned within 1 or 2 days before surgery. TAE for tumors may be an option in addition to medical and surgical treatment for persistent hypoglycemia in Doege–Potter syndrome.

An 82-year-old man with a diagnosis ofintraductal papillary mucinous carcinoma(IPMC)underwent pancreaticoduodenectomy followed by adjuvant chemotherapy with S-1. Six months after surgery, he had upper abdominal pain, and CT demonstrated a recurrent intraabdominal tumor located at the surgical incision scar. It was diagnosed as a solitary peritoneal recurrence, and palliative radiation therapy at a dose of 30 Gy was performed for the relief of abdominal pain after administration ofoxycodone. He was free ofpain without pharmacological therapy and received subsequent chemotherapy with nabpaclitaxel plus gemcitabine(GnP). He remains free ofpain and alive without progression ofthe disease 24 months after recurrence. Hypofractionated-accelerated radiotherapy is feasible and results in pain relief for local recurrence of IPMC.

A 77-year-old woman presented with peritoneal metastases from a pancreatic neuroendocrine tumor(p-NET). At the age of 56 years, she underwent distal pancreatectomy for p-NET, which was pathologically diagnosed as G2. She underwent right hemihepatectomy for liver metastasis(S6)from the p-NET 10 years post-pancreatectomy. Eight years post-hepatectomy, radiofrequency ablation(RFA)was attempted for liver metastasis(S4)from the p-NET. However, RFA was not completed because of hematoma development along the needle tract of RFA. She underwent partial hepatectomy for this lesion 6 months post-RFA. Two years post-RFA, localized peritoneal metastases on the right diaphragm were detected. She underwent en bloc tumor resection with partial resection of the diaphragm. She remains alive and well with no evidence of disease 2 years post-resection of the peritoneal metastases from the p-NET.