Mucinous Cystic Neoplasm Research Articles

Exploring thenew Kyoto guidelines for managing pancreatic cysts: an overview and comparison with previous guidelines.

The rising use of diagnostic imaging has led to an increase in the incidental detection of pancreatic cysts, with reported incidences of 1.2-2.6% on Computed Tomography and 2.4-49.1% on Magnetic Resonance Imaging. While many of these cysts are asymptomatic and benign, the enhanced imaging techniques have also revealed malignant and premalignant lesions. Mucinous neoplasms, including intraductal papillary mucinous neoplasms (IPMNs) and mucinous cystic neoplasms, are particularly concerning due to their potential for malignant transformation. Recent studies highlight significant variations in dysplasia across IPMN types, with main duct IPMNs showing a higher likelihood of high-grade dysplasia or invasive carcinoma compared to branch duct IPMNs. Management and follow-up of these lesions remain controversial due to inconsistent guidelines. This article reviews and compares six major guidelines: the 2015 American Gastroenterological Association guidelines, the 2017 International Association of Pancreatology (IAP/Fukuoka) guidelines, the 2017 American College of Radiology guidelines, the 2018 American College of Gastroenterology guidelines, the 2018 European Study Group guidelines, and the newly released, 2024 Kyoto guidelines. We summarize key differences in risk factors, surveillance protocols, and surgical referral criteria, with a focus on the updated 2024 Kyoto guidelines and the implications of recent research advancements.

To develop a classification system which helps differentiate cystic intraductal papillary neoplasm of the bile duct from mucinous cystic neoplasm of the liver

ObjectiveTo establish a classification system which differentiates cystic intraductal papillary neoplasm of the bile duct (cystic IPNB) from hepatic mucinous cystic tumors (MCN) based on their radiological difference. MethodsA total of 75 patients pathologically diagnosed as MCN and IPNB in two major hospitals from 2015 to 2024 were enrolled. Radiological features were recorded and compared between these two tumors. Variables with significant differences were included in multivariate logistic regression (LR) analysis. A decision model was built and simplified based on importance ranking of variables. K-nearest-neighbor (KNN) model was introduced to learn distribution of individuals in main dimensions based on multiple correspondence analysis (MCA) and predicted diagnosis. The diagnostic efficacy of the classification system and the KNN model was compared. ResultsSignificant differences existed in Dmax-IVC angle, septation, mural nodule, upstream and downstream biliary dilatation, communication with bile duct between MCN and cystic IPNB. Downstream biliary dilatation and communication with bile duct were highly specific for IPNB (specificity, 97.9 % and 100 %, respectively), which could independently diagnose IPNB. Among four significant indicators in LR analysis, upstream biliary dilatation and Dmax-IVC angle were used for a simplified decision model to attain good applicability. The KNN model based on MCA data achieved highest accuracy (0.910) when K = 11. Overall, the classification system achieved an AUC of 0.882 (0.95CI: 0.797–0.966), compared with 0.911 (0.95CI: 0.818–1.000) in the KNN model, which demonstrated no significant difference (p = 0.655) in differential performance. ConclusionThe classification system combining four important indicators had equivalent performance to KNN model in discrimination, which was simple and applicable for clinical practice, and also accessible on unenhanced examinations.

ASO Visual Abstract: Distinct Indications for Adjuvant Therapy in Resected Invasive Mucinous Cystic Neoplasms of the Pancreas Compared with Pancreatic Ductal Adenocarcinoma.

ASO Visual Abstract: Distinct Indications for Adjuvant Therapy in Resected Invasive Mucinous Cystic Neoplasms of the Pancreas Compared with Pancreatic Ductal Adenocarcinoma.

9334 Pancreatic Adenocarcinoma Presenting As Diabetic Ketoacidosis

Abstract Disclosure: J. Alvarez: None. M.H. Horani: None. Background: Pancreatic adenocarcinoma is often diagnosed at an advanced stage due to late onset of clinical features including poor appetite, weight loss, weakness, epigastric pain, and rarely impaired glucose tolerance. In patients who present with impaired glucose tolerance or diabetic ketoacidosis without prior history of diabetes, pancreatic adenocarcinoma should be considered. Clinical Case: Patient is a 45-year woman with depression, HTN, irritable bowel syndrome who was found unresponsive by family and presented to the ED for acute encephalopathy. On exam, she was obtunded, tachypneic, with fruity-smelling breath and dry mucous membranes. Initial labs showed glucose of 950 mg/dL (70-99 mg/dL), lipase 539 U/L (8-78 U/L), lactic acid 4 mmol/L 0.5-2 mmol/L), ketone-BHB 170.75 mg/dL (0-2.81 mg/dL), A1c 16.5%. She had no prior history or family history of diabetes. She was admitted to hospital for DKA management.CXR to evaluate dyspnea showed bilateral opacities so CT chest/abdomen/pelvis without contrast was ordered. This showed bilateral multilobar pneumonia, trace pleural effusions, 6.7 x 3.8 cm lobulated fluid collection along pancreatic tail, 12.1 x 6.6 x 4.4 cm RLQ cystic lesion and 4.4 cm lesion vs loculation along the right pelvic sidewall.Initially, GI recommended repeat imaging of the pancreas in 4-6 weeks. OBGYN was consulted for pelvic masses and recommended IR-guided biopsy of right pelvic mass which showed rare mildly atypical mucinous epithelium present, cystic mucinous neoplasm cannot be excluded.CEA and CA125 tumor markers returned elevated. These biopsies showed adenocarcinoma (solid component) and rare atypical mucinous epithelium consistent with cystic mucinous neoplasm (cystic fluid). Patient was deemed medically stable for discharge with recommended follow-up to appropriate specialists. Following discharge, she was officially diagnosed with Stage III (cT4, cN0, cM0) pancreatic adenocarcinoma and started on appropriate chemotherapeutic regimen. Discussion: The current understanding is long-standing type 2 diabetes mellitus as a risk factor for pancreatic cancer based on previous studies.The inverse relationship of pancreatic cancer causing new onset diabetes is more rare, but if cancer is suspected in these patients it could potentially help in the diagnosis of early stage adenocarcinoma. Other case reports have suggested implementation of the enriching new-onset diabetes for pancreatic cancer (ENDPAC) score in addition to CT and EUS as screening for pancreatic cancer may help with earlier diagnosis.

Imaging of pancreatic serous cystadenoma and common imitators.

Pancreatic cystic neoplasms are lesions comprised of cystic components that show different biological behaviors, epidemiology, clinical manifestations, imaging features, and malignant potential and management. Benign cystic neoplasms include serous cystic neoplasms (SCAs). Other pancreatic cystic lesions have malignant potential, such as intraductal papillary mucinous neoplasms and mucinous cystic neoplasms. SCAs can be divided into microcystic (classic appearance), honeycomb, oligocystic/macrocystic, and solid patterns based on imaging appearance. They are usually solitary but may be multiple in von Hippel-Lindau disease, which may depict disseminated involvement. The variable appearances of SCAs can mimic other types of pancreatic cystic lesions, and cross-sectional imaging plays an important role in their differential diagnosis. Endoscopic ultrasonography has helped in improving diagnostic accuracy of pancreatic cystic lesions by guiding tissue sampling (biopsy) or cyst fluid analysis. Immunohistochemistry and newer techniques such as radiomics have shown improved performance for preoperatively discriminating SCAs and their mimickers.

Ductal hamartoma of the pancreas: A clinicopathologic study

BackgroundBenign ductular proliferative lesions that resemble hepatic von-Meyenburg Complexes(VMC)/bile duct hamartomas have been noted to occur in the pancreas, but their incidence, clinicopathologic features and pathogenesis remains unknown. We present herein 3 patients that presented as cysts and call them pancreatic ductal hamartomas (PDH). MethodsThree cases of PDH were identified form a multi-institutional collaborative group, and their clinicopathological were reviewed. In addition, we also examined 115 consecutive pancreatic resections at our institutions for the presence of incidental PDHs. ResultsThe lesions were detected in each case during imaging for abdominal symptoms or grossing. The clinical suspicion was intra-ductal pancreatic mucinous cystic neoplasm (IPMN) in each case that led to pancreatectomy. The cyst fluid CEA was elevated in 2 of the patients tested. The patient age and gender were 73/M (case1), 68/F (case2) and 73/M (case3). In case1 besides the larger cystic lesion, numerous tiny lesions (0.1–0.3 cm) were seen throughout the pancreas. In case2 this was the only lesion, while in case3 there was another gastric-type IPMN with high-grade dysplasia. PDH were identified in 5(4.3%) of 115 consecutive pancreatectomy specimens. The PDHs measured 0.1–2.3 cm, and the histology is characterized by proliferation of irregular ductal structures lined by bland flattened to low columnar epithelium, variable cystic change and inspissated luminal secretions. The lining epithelium varied from non-mucinous pancreatico-biliary type to mucinous gastric foveolar-type, with occasional squamous metaplasia. SummaryPDH are seen in 4.5% of all pancreatectomy specimens and detected incidentally, but occasionally may become large and/or cystic enough leading to pancreatectomy. Their relationship to pancreatic carcinoma or IPMN remains currently unknown.

Misdiagnosis of pancreatic intraductal papillary mucinous neoplasms and the challenge of mimicking lesions: imaging diagnosis and differentiation strategies.

The rising prevalence of pancreatic cystic lesions (PCLs), particularly intraductal papillary neoplasms (IPMNs), has been attributed to increased utilization of advanced imaging techniques. Incidental detection of PCLs is frequent in abdominal CT and MRI scans, with IPMNs representing a significant portion of these lesions. Surveillance of IPMNs is recommended due to their malignant potential; however, their overlapping imaging features with benign entities can lead to misdiagnosis, overtreatment, and overutilization of healthcare resources. This paper aims to highlight and differentiate lesions often mistaken for IPMNs, providing insight into their imaging characteristics, diagnostic challenges, and distinctive features while highlighting the incidence of wrong diagnosis for these lesions. These lesions include serous cystadenomas, cystic pancreatic neuroendocrine tumors, mucinous cystic neoplasms, lymphoepithelial cysts, duodenal diverticula, pancreatic schwannomas, chronic pancreatitis, retention cysts, intrapancreatic accessory spleens, pancreatic lipomas, choledochal cysts, and others. Utilizing various imaging modalities, including contrast-enhanced CT, MRI, and EUS, alongside histological and molecular analyses, can aid in accurate diagnosis and appropriate management. Understanding these mimicry scenarios is crucial to avoid unnecessary surveillance, interventions, and the burden they place on both patients and healthcare systems. Improved recognition of these lesions can lead to better patient outcomes and resource allocation.

Cost-effectiveness of operative intervention for mucinous cystic neoplasm: the role of post-operative diabetes.

Small mucinous cystic neoplasms (MCN) of the pancreas are managed with operative resection in otherwise healthy patients; however, postoperative diabetes development is not considered in recommendations for resection. Via probabilistic microsimulation Markov modeling, we assessed clinical and economic implications of laparoscopic distal pancreatectomy (LDP) versus surveillance for non-DM patients with suspected MCN of 2 cm without high-risk or worrisome features. Primary outcomes included quality-adjusted life years (QALYs), medical costs (2021 USD), and incremental cost-effectiveness ratios (ICERs). We conducted sensitivity analyses to evaluate the robustness of our model to changes in input parameters. All analyses were repeated for a population with pre-DM. Surveillance resulted in 6.52 QALYs and $61,200, while LDP accumulated 6.12 QALYs and $63,700. Almost 20% of the LDP cohort developed DM over the first 10 years, compared to 11% of the surveillance cohort. In a pre-DM cohort, LDP remained Dominated in the base case, with over 40% developing DM postoperatively. In sensitivity analyses, surveillance remained the preferred strategy in most iterations for both cohorts. Surveillance for small suspected MCNs without high-risk features is the preferred strategy from a clinical and economic standpoint. Consensus guidelines should consider the long-term implications of postoperative diabetes development following LDP.

Jehlová konfokální laserová endomikroskopie v diferenciální diagnostice cystických lézí pankreatu

Summary: Background: Pancreatic cystic lesions are relatively common findings, and their prevalence continues to rise, which is also related to the expansion of imaging methods. Up to 15% of pancreatic cancers arise from mucinous cystic tumours of the pancreas, primarily from intraductal papillary mucinous neoplasia (IPMN). Needle-based confocal laser endomicroscopy (nCLE) represents an advanced diagnostic method with the potential to improve the accuracy of diagnosing focal pancreatic lesions. Aim: The aim of the work is to provide a recent overview of the position of nCLE in the diagnosis of pancreatic cystic lesions and some of our own experiences. The nCLE method is based on so-called real-time optical biopsy, which brings better diagnostic results compared to traditional methods such as CT, MRI/MRCP, and EUS, as confirmed by the authors‘ own data. Although nCLE offers certain advantages, its wider use in clinical practice is limited by the high costs of equipment, technical difficulties during examination, and the need for thorough training of endoscopists. The integration of all diagnostic procedures (EUS, cytology, nCLE, and next-generation sequencing) into artificial intelligence algorithms could minimize the disadvantages of individual methods and increase the overall efficiency of diagnosing pancreatic cystic lesions. Conclusion: nCLE represents an innovative method for diagnosing pancreatic cystic lesions, which, in combination with other procedures (microforceps biopsy, next-generation sequencing, assessment of nCLE findings, and overall malignancy risk using artificial intelligence), can further enhance its diagnostic accuracy in this context. Key words: needle-based confocal laser endomicroscopy – pancreatic cystic lesion – optical biopsy – endoscopic ultrasound – artificial intelligence

Pancreatic cystic neoplasms

Pancreatic cystic lesions represent achallenging heterogeneous entity with a potential risk of malignant transformation. The diagnostics include in particular medical history taking with collection of relevant clinical information and high-resolution imaging, preferably using magnetic resonance imaging (MRI) with MR cholangiopancreatography (MRCP) and/or endoscopic ultrasonography. A differentiation between different cystic entities and identification of risk factors are crucial for making appropriate treatment decisions. Only a small proportion of pancreatic cystic neoplasms require surgery. Pancreatic cystic lesions with a relevant risk of malignancy, such as main duct intraductal papillary mucinous neoplasms (IPMN), mucinous cystic neoplasms (MCN), solid pseudopapillary neoplasms (SPN) and general cystic pancreatic lesions with risk factors regardless of the entity, should be resected, whereas an individualized approach is required for branch duct IPMN and serous cystic neoplasms (SCN) and dysontogenetic cysts require no treatment. Parenchyma-sparing and minimally invasive resection techniques should be preferred whenever possible for resecting pancreatic cystic tumors. Approximately 10% of patients develop recurrences over time.

Gradual telomere shortening in the tumorigenesis of pancreatic and hepatic mucinous cystic neoplasms

Mucinous cystic neoplasm (MCN) is one of the precursor lesions of pancreatic ductal adenocarcinoma and intrahepatic cholangiocarcinoma. The aim of this study is to examine the presence of short telomeres in promoting the tumorigenesis of MCN by measuring telomere lengths in distinct components of MCN, including the mucinous lining epithelium, non-mucinous lining epithelium, and ovarian-type stroma. A total of 45 patients with MCN (30 pancreatic and 15 hepatic cases) were obtained. Quantitative telomere-specific fluorescent in situ hybridization was performed to measure the telomere length of specific cell types within MCNs, including mucinous lining epithelium, non-mucinous lining epithelium, and ovarian-type stroma, as well as normal ductal epithelium and adenocarcinoma. Relative telomere lengths tended to decrease between normal ductal epithelium, ovarian-type stroma, non-mucinous lining epithelium, mucinous lining epithelium, and adenocarcinoma regardless of the involved organs. Among the analyzed cell types, relative telomere lengths were significantly different between normal ductal epithelium (3.31 ± 0.78), ovarian-type stroma (2.90 ± 0.93), non-mucinous lining epithelium (2.84 ± 0.79), mucinous lining epithelium (2.49 ± 0.93), and adenocarcinoma (1.19 ± 0.59), respectively (P < 0.001, mixed-effects model). As expected, no difference in relative telomere lengths was observed between normal ductal epithelium and ovarian-type stroma; however, significant differences were observed in pair-wise comparisons between ovarian-type stroma vs. non-mucinous lining epithelium (P = 0.001), non-mucinous lining epithelium vs. mucinous lining epithelium (P = 0.005), and mucinous lining epithelium vs. adenocarcinoma (P < 0.001). These findings suggest gradual telomere shortening occurs in the tumorigenesis of MCN, which may have important implications for the progression of this disease.

Prospective multicenter surveillance study of branch-duct intraductal papillary mucinous neoplasm of the pancreas; risk of dual carcinogenesis

BackgroundThe natural history of branch-duct intraductal papillary mucinous cystic neoplasms (BD-IPMNs) in the pancreas remains unclear. This study aimed to answer this clinical question by focusing on the development of concomitant pancreatic ductal adenocarcinomas (cPDAC). MethodsThe Japan Pancreas Society conducted a prospective multicenter surveillance study of BD-IPMN every six months for five years. The primary endpoints were progression of BD-IPMN, progression to high-grade dysplasia/invasive carcinoma (HGD/IC), and cPDAC. Factors predicting the progression of BD-IPMN to HGD/IC and development of cPDAC were also assessed as secondary endpoints. ResultsAmong the 2104 non-operated patients, 348 (16.5 %) showed progression of primary BD-IPMN. Cumulative incidences of BD-IPMN with HGD/IC and cPDAC during the 5.17-year surveillance period were 1.90 % and 2.11 %, respectively, and standard incidence ratios of BD-IPMN with HGD/IC and cPDAC were 5.28 and 5.73, respectively. Of 38 cPDACs diagnosed during surveillance, 25 (65.8 %) were resectable. The significant predictive characteristics of BD-IPMN for progression to HGD/IC were larger cyst size (p = 0.03), larger main pancreatic duct size (p < 0.01), and mural nodules (p = 0.02). Significant predictive characteristics for the development of cPDAC were male sex (p = 0.03) and older age (p = 0.02), while the size of IPMN was not significant. ConclusionCareful attention should be given to “dual carcinogenesis” during BD-IPMN surveillance, indicating the progression of BD-IPMN to HGD/IC and development of cPDAC distinct from BD-IPMN, although the establishment of risk factors that predict cPDAC development remains a challenge (UMIN000007349).

Epigenetic silencing of BEND4, a novel DNA damage repair gene, is a synthetic lethal marker for ATM inhibitor in pancreatic cancer.

Synthetic lethality is a novel model for cancer therapy. To understand the function and mechanism of BEN domain-containing protein 4 (BEND4) in pancreatic cancer, eight cell lines and a total of 492 cases of pancreatic neoplasia samples were included in this study. Methylation-specific polymerase chain reaction, CRISPR/Cas9, immunoprecipitation assay, comet assay, and xenograft mouse model were used. BEND4 is a new member of the BEN domain family. The expression of BEND4 is regulated by promoter region methylation. It is methylated in 58.1% (176/303) of pancreatic ductal adenocarcinoma (PDAC), 33.3% (14/42) of intraductal papillary mucinous neoplasm, 31.0% (13/42) of pancreatic neuroendocrine tumor, 14.3% (3/21) of mucinous cystic neoplasm, 4.3% (2/47) of solid pseudopapillary neoplasm, and 2.7% (1/37) of serous cystic neoplasm. BEND4 methylation is significantly associated with late-onset PDAC (> 50 years, P < 0.01) and tumor differentiation (P < 0.0001), and methylation of BEND4 is an independent poor prognostic marker (P < 0.01) in PDAC. Furthermore, BEND4 plays tumor-suppressive roles in vitro and in vivo. Mechanistically, BEND4 involves non-homologous end joining signaling by interacting with Ku80 and promotes DNA damage repair. Loss of BEND4 increased the sensitivity of PDAC cells to ATM inhibitor. Collectively, the present study revealed an uncharacterized tumor suppressor BEND4 and indicated that methylation of BEND4 may serve as a potential synthetic lethal marker for ATM inhibitor in PDAC treatment.

ASO Author Reflections: Is Adjuvant Therapy Necessary for All Patients with Invasive Mucinous Cystic Neoplasms of the Pancreas? Let's De-escalate.

ASO Author Reflections: Is Adjuvant Therapy Necessary for All Patients with Invasive Mucinous Cystic Neoplasms of the Pancreas? Let's De-escalate.

Distinct Indications for Adjuvant Therapy in Resected Invasive Mucinous Cystic Neoplasms of the Pancreas Compared with Pancreatic Ductal Adenocarcinoma

BackgroundSurgical and adjuvant management of mucinous cystic neoplasms (MCNs) lacks formal guidelines and data is limited to institutional studies. Factors associated with receipt of adjuvant therapy and any associated impact on survival remain to be clarified. In the absence of other data, guidelines that recommend adjuvant chemotherapy for invasive pancreatic adenocarcinoma have been extrapolated to MCN.Patients and MethodsThe National Cancer Database (2004–2019) was utilized to identify all patients that underwent pancreatic resection for invasive MCNs. Patients that received neoadjuvant therapy or did not undergo lymphadenectomy were excluded. Patient, tumor, and treatment factors associated with survival were assessed.ResultsFor 161 patients with invasive MCN, median overall survival (OS) was 133 months and 45% of patients received adjuvant therapy. Multivariable analysis demonstrated that poorly differentiated tumors [odds ratio (OR) 4.19, 95% confidence interval (CI) 1.47–11.98; p = 0.008] and positive lymph node status (OR 2.67, 95% CI 1.02–6.98; p = 0.042) were independent predictors of receiving adjuvant therapy. Lymph node positivity [hazard ratio (HR) 2.90, 95% CI 1.47–5.73; p = 0.002], positive margins (HR 5.28, 95% CI 2.28–12.27; p < 0.001), and stage III disease (HR 12.46, 95% CI 1.40–111.05; p = 0.024) were associated with worse OS. Receipt of adjuvant systemic therapy was independently associated with decreased risk of mortality in node positive patients (HR 0.23, 95% CI 0.10–0.69; p = 0.002). Survival was not associated with adjuvant therapy in patients with negative lymph nodes or margin negative status.ConclusionIn contrast to pancreatic ductal adenocarcinoma (PDAC), where adjuvant therapy improves OS for every tumor stage, surgery alone for invasive MCN is not associated with improved OS compared with surgery plus adjuvant therapy in node-negative patients. Surgery alone is likely sufficient for a subset of invasive MCN.

A paradigm shift in diagnosis and treatment innovation for mucinous cystic neoplasms of the liver

This study comprehensively explores the clinical characteristics, diagnostic approaches, and treatment methods for liver mucinous cystic neoplasms (MCN). A retrospective analysis was conducted on seven individuals diagnosed with MCN, admitted to the Fifth Medical Center of the PLA General Hospital between October 2016 and May 2023. Preoperative AFP was negative, while CA19-9 was elevated in two cases. Surgical resection was performed for all patients. The patients showed favorable postoperative recovery. Follow-up revealed an excellent overall survival rate, except for one case of invasive carcinoma resulting in tumor recurrence and metastasis 6 months after surgery. MCN poses a diagnostic challenge due to the absence of distinct clinical and radiological features, leading to potential misdiagnosis and inappropriate treatment. Patients with suspected liver cystic diseases should consider the possibility of MCN. Surgical resection has proven to be a practical approach with satisfactory therapeutic outcomes.

Mucinous cystic neoplasm of the liver: A rare case report

Mucinous cystic neoplasm of the liver is a rare cystic neoplasm accounting for approx. 5% of cystic liver in the liver parenchyma. We describe the histomorphology and immunophenotyping features of MCN-L in a 52-year-old woman who came to the surgical department with complaints of pain abdomen for 1.5 months. The ultrasonography showed a septated cyst located centrally in the liver. The CT abdomen showed a large hypodense multiloculated cystic lesion in the liver with thin enhancing septae and multiple small cysts involving the entire left lobe with extension into the right lobe and having a large infra-hepatic exophytic component. A provisional diagnosis of a Hydatid cyst was made. Drainage of the cyst with laparoscopic deroofing was done. Histopathology examination showed a cyst lined by cuboidal to columnar epithelium with mucinous cytoplasm and basally oriented nuclei. Subepithelium shows ovarian-like stroma. MCN-L has a lower frequency as compared to the counterpart of MCN in the pancreas, and further molecular studies are necessary to clarify the biology of MCN-L. The relevance of this rare entity's clinical and radiological suspicion, histological recognition and immunohistochemistry is emphasised.

Giant pancreatic cyst with extension to spleen managed by laparoscopy

To review the etiology, clinical presentation, diagnostic evaluation, management, and prognosis of giant pancreatic cysts. Giant pancreatic cysts, defined as cysts exceeding 5 cm in diameter, include a diverse group of lesions such as pseudocysts, serous cystadenomas, mucinous cystic neoplasms (MCNs), and intraductal papillary mucinous neoplasms (IPMNs). Their clinical significance stems from potential complications and the necessity to differentiate benign from malignant cysts. A comprehensive review of the literature was conducted, focusing on the pathophysiology, symptomatology, diagnostic modalities, treatment options, and outcomes associated with giant pancreatic cysts. The etiology of giant pancreatic cysts varies from benign conditions like pseudocysts and serous cystadenomas to potentially malignant or malignant neoplasms such as MCNs and IPMNs. Clinical presentation ranges from asymptomatic cases to severe abdominal symptoms and complications. Diagnostic evaluation includes imaging modalities like ultrasound, CT, MRI/MRCP, and endoscopic ultrasound (EUS) with fine-needle aspiration (FNA) for cytology and biochemical analysis. Management strategies depend on the type and characteristics of the cyst, with options including observation, endoscopic drainage, surgical resection, and percutaneous drainage. The prognosis varies, with benign cysts generally having a favorable outcome, while cysts with malignant potential require timely surgical intervention to improve survival rates. Giant pancreatic cysts pose a diagnostic and therapeutic challenge due to their varied etiologies and potential complications. Accurate diagnosis through advanced imaging and fluid analysis is crucial. Management should be tailored based on the cyst type, symptoms, and malignancy risk, involving a multidisciplinary approach to optimize patient outcomes. Further advancements in diagnostic and therapeutic techniques are anticipated to enhance the management of these complex lesions.

Epidermoid Cyst arising within an Intrapancreatic Accessory Spleen [ECIPAS] mimicking a pancreatic mucinous cystic neoplasm-a case report with literature review

Background: Accessory spleens are congenital embryological aberrations usually found within the splenic hilum with no clinical significance. An Intra Pancreatic Accessory Spleen (IPAS) is an uncommon benign pancreatic lesion encountered clinically. The occurrence of an Epidermoid Cyst arising within an IPAS (ECIPAS) is exceedingly rare and is often misdiagnosed as a pancreatic pathology such as mucinous cystic neoplasm, cystic degeneration within a solid tumor such as a neuroendocrine tumor, or a lymph node. Case report: A 68-year-old male presented with intermittent post-prandial abdominal pain for over 2 years. Abdominal computer tomography identified a 5.2 cm calcified cyst within the pancreatic tail and a mucinous pancreatic neoplasm/pancreatic pseudocyst was considered in the differential diagnosis. The results of endoscopic ultrasound and fine needle aspiration were indeterminate. Due to persistent abdominal pain, the patient underwent a laparoscopic distal pancreatectomy with splenectomy. A gross examination of the distal pancreatic/splenectomy specimen confirmed a well-demarcated cystic lesion with brownish fluid within the pancreatic tail. Microscopic examination revealed a nonpathological pancreas separated by a fibrous capsule with a large cyst arising within an intrapancreatic accessory spleen. The cyst was lined with multilayered non-keratinized stratified squamous epithelium positive for pancytokeratin, CA 19-9, CK5/6, and p63 with no lymphocytic infiltrates and absent hair/ dermal appendages confirming an epidermoid cyst. CD8 outlined the dendritic network of the littoral cells of the splenic tissue in the cyst wall. Post-operative follow-up at 6 weeks was uneventful. Conclusion: The preoperative diagnosis of ECIPAS is extremely difficult as this entity shares overlapping radiological features with other cystic lesions such as mucinous pancreatic cysts. It is important for anatomic pathologists to recognize and consider the intrapancreatic compartment as a possible site for accessory spleen. As histopathology is the final determinant of this diagnosis, increased clinical awareness with an accurate diagnosis of this entity may prevent patients from unnecessary surveillance and/or extensive oncological-based surgical resection.

Atypical Image Change of Hepatic Mucinous Cystic Neoplasm.

Atypical Image Change of Hepatic Mucinous Cystic Neoplasm.