Primary peritoneal clear cell carcinoma (PPCCC) is an exceptionally rare malignancy that closely resembles gynecologic epithelial cancers clinically and histologically. Its pathogenesis is poorly understood, with possible origins from Müllerian metaplasia or malignant transformation of endometriosis. A 48-year-old woman with no history of endometriosis or hormone therapy presented with acute lower abdominal pain. Imaging revealed a pelvic mass and a lesion in the pancreatic tail. The patient underwent hysterectomy with bilateral salpingo-oophorectomy and distal pancreatectomy with splenectomy. Intraoperatively, the pelvic mass was located in the peritoneum, while the gynecologic organs appeared grossly normal. Histopathologic examination of the pelvic, pancreatic, and splenic lesions revealed identical features of clear cell carcinoma. Immunohistochemistry demonstrated positivity for PAX8, CK7, and HNF1β, with loss of MSH2 and MSH6. Next-generation sequencing revealed ARID1A loss and somatic PIK3CA mutations. No primary ovarian, endometrial, or renal tumor was detected, supporting a diagnosis of primary peritoneal clear cell carcinoma with metastases to the pancreas and spleen. This case highlights the diagnostic challenges of PPCCC and offers valuable insights into the clinical and pathological spectrum of this underrecognized malignancy.

In adults undergoing distal pancreatectomy, how does stapler resection and closure compare with scalpel resection followed by handsewn closure of the pancreatic remnant?

Intraductal papillary mucinous neoplasm with pseudomyxoma peritonei: a case report

ASO Author Reflections: Arterial Reconstruction in Distal Pancreatectomy with Celiac Axis Resection.

Evidence on pancreatic regeneration and functional recovery after pancreatectomy remains limited. This study investigates the correlation between volumetric changes and endocrine function of the remnant pancreas following pancreaticoduodenectomy (PD) and distal pancreatectomy (DP). We conducted a retrospective cohort study of patients who underwent PD or DP between January 2009 and December 2017 at a single institution. Pancreatic volume was assessed using contrast-enhanced computed tomography preoperatively and at 3 months, 1 year, and 2 years postoperatively. Endocrine function was evaluated using serial C-peptide index (CPI) measurements. A total of 90 PD and 45 DP patients were analyzed. Compared with initial residual volume, remnant pancreas in PD progressively declined (80.79%, 68.67%, and 65.34% at 3 months, 1 year, and 2 years; β = -11.85, p < 0.001), whereas DP patients demonstrated hypertrophic changes (106.25%, 106.62%, and 110.43%; β = 2.97, p = 0.019). New-onset diabetes occurred in 22.7% of PD and 33.3% of DP patients. Postoperative pancreatic duct dilatation was associated with greater atrophy in PD (β = -9.82, p = 0.027). Despite superior volume preservation in DP, PD demonstrated better endocrine functional recovery (CPI/volume ratio: β = 126.9, p < 0.001), corresponding with lower new-onset diabetes incidence. Pancreatic volume and endocrine function recover independently after pancreatectomy. Despite greater volume preservation, DP patients experience more endocrine dysfunction than PD due to higher islet density in the distal pancreas.

A novel partial spleen-preserving distal pancreatectomy procedure: Comparison to the Warshaw technique.

Total pancreatectomy and islet autotransplantation (TPIAT) is a complex surgical procedure performed in transplant centers throughout the United States, with increasing prevalence over the last 2 decades. The goals of TPIAT are to alleviate pain caused by debilitating chronic pancreatitis or acute recurrent pancreatitis by removing the pancreas and to prevent the development of brittle diabetes by infusion of pancreatic islet cells to replace the function of the surgically absent pancreas. Imaging plays a key role in evaluating patients who have undergone TPIAT. Radiologists must be aware of the surgical procedure and its variations, expected postoperative imaging findings, and potential complications for accurate diagnosis. The authors review the background of TPIAT and the indications for and goals of the procedure. The surgical procedure, expected postsurgical anatomy, imaging findings, and spectrum of complications are described. Imaging findings of postoperative complications and sequelae of TPIAT may include bowel or bile leak, anastomotic breakdown, abscess, hematoma, biliary stricture, bowel obstruction due to anastomotic stricture, adhesions, incisional or internal hernia, delayed gastric emptying, bezoar, vascular abnormalities (eg, thrombosis, pseudoaneurysm), omental infarct and/or asymptomatic fat necrosis, and atypical patterns of hepatic steatosis, including nodular hepatic steatosis. ©RSNA, 2026.

ASO Visual Abstract: Fluorescence Robot-Assisted Spleen-Preserving Distal Pancreatectomy (Warshaw Technique): A Spleen-Preserving Strategy with Intraoperative Confirmation of Splenic Perfusion (with Video).

ABSTRACT Aim This nationwide observational study investigated recent trends in pancreatic cancer (PC) incidence and surgical management in Japan. Methods Annual data on PC incidence (2016–2021) and surgical procedures (2016–2023) were obtained from the Cancer Information Database and the National Database of Health Insurance Claims and Specific Health Checkups (NDB), respectively. Surgical procedures were categorized by type (distal pancreatectomy (DP, 2016–2023)/pancreatoduodenectomy (PD, 2020–2023)) and approach (open/laparoscopic). Crude and age‐adjusted rates per 100 000 person‐years were calculated. Temporal trends were evaluated using linear and Poisson regression to estimate annual risk ratios (RRs). Results Over the study period, the annual average PC incidence was 43 015, and the average number of PC surgery was 13 899. Age‐adjusted PC incidence rates rose significantly among males, females, and both sexes (RR = 1.007, 1.016 and 1.011, respectively; p &lt; 0.0001). A particularly notable rise was observed among females aged 10–29 years (RR range: 1.347–1.449; all p &lt; 0.0009). DP rates increased significantly among males, females, and both sexes (RR = 1.033, 1.032, and 1.033, respectively; p &lt; 0.0001), with marked increases among individuals aged 65–89 years for both sexes (RR range: 1.018–1.114; all p &lt; 0.0012). PD volumes also rose during 2020–2023, although the limited analytic window precluded formal rate‐based trend evaluation. In 2023, PD comprised 65.6% of PC surgeries (9444/14397), while DP comprised 34.4% (4953/14397). Conclusion These nationwide findings highlight age‐ and sex‐specific signals—notably an apparent rise among young women and increased DP use among older adults—that warrant further validation.

Usually conducted through open access, minimally invasive (MIS) distal pancreatectomy with celiac axis resection (DP-CAR) is uncommon and technically demanding.1,2 Resectability, especially when the superior mesenteric artery (SMA) is potentially involved, is usually assessed only after irreversible steps, such as pancreatic transection, are undertaken.2,3 PATIENT AND METHODS: We describe a laparoscopic DP-CAR with left gastric artery (LGA) preservation in a 68-year-old woman with locally advanced pancreatic ductal adenocarcinoma. After neoadjuvant FOLFIRINOX therapy, surgery was scheduled. Temporary clamping of the common hepatic artery, combined with indocyanine green (ICG) fluorescence and intraoperative ultrasound, verified adequate hepatic perfusion via the gastroduodenal artery. Given the tumor's proximity to the SMA, a ligament of Treitz artery-first approach allowed the periadventitial dissection of the SMA, confirming resectability before any irreversible maneuvers.3,4 This approach also facilitated a posterior plane beneath Gerota's fascia. Gastric viability was also determined using ICG, as the patient refused gastrectomy. Intraoperatively, an independent origin of the LGA without tumor infiltration enabled its preservation. A small arterial injury during celiac dissection was successfully managed laparoscopically without conversion. The surgery was achieved with no requirement for conversion to open surgery. The patient's postoperative course was uneventful, with discharge on day seven and confirmed R0 resection on pathology. At eight months follow-up, she remains recurrence-free and asymptomatic. The ligament of Treitz approach facilitates the assessment of tumor resectability and dissection of the SMA adventitia before undertaking irreversible surgical steps.

Distal pancreatectomy with celiac axis resection (DP-CAR) with reconstruction of the left gastric artery (LGA) is a technically challenging procedure. The middle colic artery is commonly used for LGA reconstruction. This study highlights our novel arterial reconstruction of the LGA using the common hepatic artery (CHA) supplying the replaced left hepatic artery (rLHA) during DP-CAR. A 65-year-old man diagnosed with locally advanced unresectable pancreatic body cancer underwent DP-CAR following systemic chemotherapy. As a rLHA arising from the LGA was present, arterial reconstruction was necessary. After confirming resectability, the CHA and LGA were encircled. Following division of the pancreas and radical lymphadenectomy, the origin of the celiac axis (CA) was divided. Subsequently, the CHA and LGA were transected and anastomosed.An indocyanine green fluorescence system was used to confirm adequate arterial blood supply and satisfactory tissue perfusion. Operative time was 215 min, with an estimated blood loss of 35 mL. This study demonstrated a novel arterial reconstruction of the LGA supplying the rLHA during DP-CAR. The CHA may be a candidate for the reconstruction of the LGA in DP-CAR.

Objective: To assess the impact of celiac axis stenosis (CAS) on postoperative outcome after pancreatoduodenectomy (PD) and total pancreatectomy (TP), and to describe treatment strategies based on CAS severity and etiology. Summary Background Data: Asymptomatic CAS may compromise hepatic and gastric perfusion after PD and TP, potentially increasing morbidity. The role of preoperative CAS detection and treatment remains unknown. Methods: International retrospective study at four high-volume centers in four countries (2018–2024). All preoperative CT imaging was re-assessed. CAS &gt;50% stenosis was graded as B/C and considered clinically relevant. Etiology was classified as atherosclerotic, median arcuate ligament [MAL]-related, or mixed. Outcome was associated with CAS severity, etiology, and treatment. Standardized management protocols per center were identified. Results: Among 1,698 patients undergoing PD and TP, CAS was identified in 16% (n=279). CAS grade B/C (6.5%, n=111) was independently associated with severe complications (OR 2.20, P &lt;0.001), bile leak (OR 2.67, P =0.007), liver perfusion failure (OR 2.60, P &lt;0.001), and gastric ischemia (OR 11.29, P &lt;0.001). Outcomes differed by etiology: atherosclerotic CAS was associated with higher bile leak rate (22.7% vs. 5.7%; P =0.018) than MAL-related CAS. Centers with standardized protocols identified and treated CAS more frequently. Conclusions: CAS grade B/C is an underrecognized yet potentially modifiable risk factor for severe complications after PD and TP. Preoperative identification of CAS grade B/C, including etiology, may allow targeted intervention but larger studies are required.

Acute pancreatitis is an uncommon but clinically important complication in people living with HIV (PLWH) and has been linked to HIV itself, older nucleoside reverse transcriptase inhibitors, protease inhibitors via hypertriglyceridemia, and multiple opportunistic or metabolic comorbidities. Atraumatic splenic rupture (ASR) is rare and has been described in association with acute or chronic pancreatitis and, more rarely, with HIV infection. However, the coexistence of chronic HIV infection, pancreatitis with pancreatic tail pseudocyst, and ASR has seldom been reported. We present a complex case highlighting the interaction between long-standing HIV infection, chronic pancreatitis, and splenic injury. To our knowledge, no previous report has described chronic HIV infection complicated simultaneously by acute-on-chronic pancreatitis, a pancreatic tail pseudocyst, and atraumatic splenic rupture. A 35-year-old man with a 9-year history of HIV infection on antiretroviral therapy (ART) presented with acute worsening of upper abdominal pain and dizziness on the background of intermittent epigastric pain over one year. He had no history of abdominal trauma, alcohol abuse, gallstones, or hypertriglyceridemia, and had never received didanosine or stavudine. Initial assessment revealed pallor, hypotension, generalized abdominal tenderness with peritoneal signs, severe anemia, leukocytosis, and markedly elevated serum amylase and lipase levels. Contrast-enhanced abdominal CT showed hemoperitoneum, irregular laceration and heterogeneous enhancement of the spleen, chronic pancreatitis with atrophic, calcified pancreas and dilated main pancreatic duct, and a pseudocyst in the pancreatic tail abutting the splenic hilum. Emergency laparotomy revealed approximately 1500 mL of hemoperitoneum, a ruptured upper pole splenic laceration extending towards the hilum, and a pancreatic tail pseudocyst adherent to the splenic hilum. Splenectomy plus distal pancreatectomy with drainage were performed. Pathology confirmed chronic pancreatitis with pseudocyst formation and splenic rupture without malignancy. Postoperative recovery was uneventful apart from reactive thrombocytosis, which was managed with antiplatelet therapy. The patient remained well with no recurrence of pancreatitis or splenic complications at 15-month follow-up. This case illustrates a plausible "pancreas-spleen axis" in which chronic pancreatitis with a pancreatic tail pseudocyst leads to local vascular and parenchymal fragility, predisposing to ASR in a patient with chronic HIV infection and incomplete immune reconstitution. It emphasizes the need to consider ASR in PLWH presenting with acute abdomen, particularly when imaging shows pancreatic tail pathology. Early CT, prompt surgical decision-making, and multidisciplinary management between infectious disease specialists and surgeons are critical for favorable outcomes.

This systematic review and meta-analysis assessed long-term outcomes following total pancreatectomy with islet autotransplantation (TPIAT). Seventeen studies including 1332 patients were analyzed. The pooled insulin independence rate was 34%, with higher rates for non-chronic pancreatitis indications (68%) versus chronic pancreatitis (33%). TPIAT is effective in preserving endocrine function. Further studies are needed to validate outcomes across extended indications and to standardize reporting, incorporating metabolic markers and patient-reported quality-of-life endpoints over long-term follow-up.

Recurrence of intraductal papillary mucinous neoplasms (IPMNs) in the remnant pancreas after surgery is a significant clinical challenge. A 68-year-old woman was incidentally found to have a 50-mm mixed-type intraductal papillary mucinous carcinoma (IPMC) in the pancreatic head during a health check. She underwent subtotal stomach-preserving pancreaticoduodenectomy with pancreatogastrostomy. Pathology revealed intestinal-type IPMC (pStage IA) with venous invasion and negative margins. Adjuvant oral S-1 chemotherapy was administered for six months. Eighteen months later, she developed rapid increases in HbA1c and tumor markers. Computed tomography showed 8-mm main pancreatic duct dilation in the remnant pancreas. Upper gastrointestinal endoscopy revealed a papillary tumor extending into the gastric mucosa, and biopsy confirmed adenocarcinoma. She was diagnosed with recurrent IPMC in the remnant pancreas and underwent total pancreatectomy with splenectomy. Intraoperative gastric endoscopy determined the extent of gastric wall resection. Postoperative chylous leakage occurred but resolved, and she was discharged on day 26. Pathology revealed non-invasive gastric-type IPMC, distinct from the intestinal-type lesion in the initial surgery. This case represents a rare metachronous recurrence of gastric-type IPMC following resection of intestinal-type IPMC, emphasizing the need for vigilant long-term surveillance and awareness of potential histologic subtype changes.

A 59-year-old man underwent computed tomography (CT) for evaluation of abdominal pain, which revealed a pancreatic tumor. The patient was subsequently referred to our hospital for further evaluation. Contrast-enhanced CT revealed a 22-mm lesion in the pancreatic tail with a contrast effect extending from the arterial phase. In addition, a 20-mm lesion with slightly less contrast enhancement was observed on the head side of the lesion. Endoscopic ultrasound-guided fine-needle tissue acquisition was performed on the lesion in the pancreatic tail, leading to the diagnosis of pancreatic acinar cell carcinoma, and distal pancreatectomy was performed. Preoperative imaging suggested the presence of two separate lesions; however, the resected specimen showed that the lesions had formed a single mass owing to their extension into the main pancreatic duct. The poorly enhanced region on the head side was primarily composed of edema and inflammatory changes with few tumor cells, whereas the enhanced region on the tail side consisted mostly of tumor cells. This difference in composition was thought to account for the variation in contrast enhancement. Awareness of imaging differences combined with histopathological correlations may aid in biopsy site selection and provide value in routine clinical practice.

Congenital hyperinsulinism (CHI) is a rare but significant cause of persistent hypoglycemia in neonates and infants. Mutations in several genes, including ABCC8 and KCNJ11, are known to cause CHI. However, data on CHI from our region remain limited. To assess the genetic spectrum, clinical characteristics, and outcomes of patients with CHI. This was a single-centre observational study conducted in the Department of Endocrinology and the Multidisciplinary Research Unit at Sher-i-Kashmir Institute of Medical Sciences, Srinagar, involving six unrelated patients with clinically suspected CHI who underwent detailed clinical evaluation and targeted genetic testing using a 19-gene panel associated with CHI, including hyperinsulinism-hyperammonaemia syndrome. Genetic analysis was performed using Sanger sequencing, followed by parental segregation analysis to determine the inheritance patterns. The mean age at presentation was 24.2 days. Parental consanguinity was present in 3/6 cases. Three patients (50%) presented with hypoglycemic seizures, while the remaining presented with feeding refusal and lethargy. Likely pathogenic variants were identified in three patients (50%). The most commonly affected gene was ABCC8 (n = 2), followed by KCNJ11 (n = 1). Detected ABCC8 variants included a likely novel pathogenic splice variant (c.1009_1011 + 11del) and a frameshift variant (c.453del), whereas the KCNJ11 missense variant identified was c.107 T > A. The likely pathogenic homozygous novel variant, c.1009_1011 + 11del, resulted in a 14 base pair deletion in the intron six and exon six junction of the gene. The mutation affected the invariant GT donor splice site downstream of exon 6 (5 splice). Clinically, two patients responded to diazoxide therapy, while four were classified as diazoxide-unresponsive. Partial pancreatectomy was performed in two of the diazoxide-unresponsive cases. The mean duration of follow-up was 22 months (range: 2-42 months). This study documents a likely novel ABCC8 pathogenic variant from a region with limited prior data on CHI, enhancing global understanding of its genetic diversity. Our findings emphasize the importance of integrating genetic testing into standard diagnostic protocols for timely and tailored interventions. The main limitations of this study include the use of Sanger sequencing as the sole genetic testing approach and the absence of functional validation of the identified variants.

Pancreatic surgical procedures are technically challenging and associated with a high level of surgical stress and postoperative pain. The most common surgical intervention in the pancreas is a pancreatoduodenectomy seconded by a distal pancreas resection and total pancreatectomy. Optimal pain management is crucial for ensuring early mobilization and has been shown to reduce the length of hospital stay and the incidence of postoperative complications. The optimal strategy, however, remains a matter of controversy, and the advantages and harms related to the use of different analgesic interventions remain unclear. This systematic review aims to investigate the benefits and harms of analgesic interventions in adult patients undergoing total pancreatectomy, pancreatoduodenectomy and distal pancreatectomy. The protocol adheres to The Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols guidelines. A search will be conducted in Medline, EMBASE, and The Cochrane Library's CENTRAL for published and ongoing trials. We will include randomized clinical trials that assess the postoperative effect of an intervention compared to a control. A risk of bias assessment will be performed according to risk of bias volume 2 (ROB2), and trial sequential analysis will be conducted. Furthermore, a meta-analysis will be conducted in accordance with the recommendations outlined in the Cochrane Handbook for Systematic Reviews and Interventions. The Review Manager Software version 5.4 will be used to handle the analyses. The overall quality of evidence for outcomes derived from the meta-analysis, risk of bias, and trial sequential analysis will be evaluated using the GRADE approach.

Intraductal papillary mucinous neoplasms (IPMN) are the most common cystic pancreatic lesions, with increasing incidence due to advances in imaging. Their management is complex due to their malignant potential and association with other neoplasms, weighed against morbidity and mortality of pancreatic resection. This study aimed to evaluate survival and surgical outcomes in patients with IPMN exhibiting high-risk features for malignant transformation. This retrospective study reviewed the medical records of 22 patients with adenocarcinoma arising in degenerated IPMN, diagnosed between 2008 and 2024. Inclusion criteria were clinical and radiological signs of pancreatic neoplasm consistent with degenerated IPMN (2023 Kyoto guidelines) and histopathological confirmation. Endoscopic ultrasound (EUS) was not performed in most cases due to the study's timeframe and strong Magnetic Resonance Imaging (MRI) indication of malignancy. Whipple procedures or total pancreatectomy were performed, excluding patients with advanced arterial infiltration or metastases. Demographic, clinical, surgical, and histopathological data, including tumor markers, tumor size, postoperative complications (pancreatic fistula, hemorrhage, delayed gastric emptying) and 30-day mortality were analyzed. Overall survival (OS) and disease- free survival (DFS) were estimated using the Kaplan-Meier method. The cohort comprised 68.2% men with a median age of 70.5 years. Common comorbidities included hypertension, chronic obstructive pulmonary disease (COPD) and diabetes. Most patients were symptomatic presenting with jaundice, pain, or weight loss. Preoperative findings included elevated bilirubin and CA 19-9 levels. Based on Kyoto guidelines, most patients exhibited high-risk stigmata. Surgical procedures primarily involved Whipple procedures, with a median operative time of 360 minutes. Postoperative complications occurred in 45.5% of patients. Median hospital stay was 19 days. Median follow-up was 23 months. Overall survival was 86.4% at 12 months, 72.7% at 24 months, and 68.2% at 60 months. Disease-free survival was 86.4% at 12 months, 81.8% at 24 months, and 72.7% at 60 months. Recurrence occurred in 45.5% of patients, primarily in the lungs, liver, and residual pancreas. Despite the limitations of the small sample size and retrospective design, this study supports the 2023 Kyoto guidelines, demonstrating that surgical management of invasive IPMN can achieve substantially longer survival similar to classic pancreatic adenocarcinoma. Multidisciplinary evaluation is crucial for identifying signs of invasion and malignant degeneration, guiding surgical intervention.

Techniques for Robotic Pancreatectomy.