Mucinous appendiceal neoplasms and pseudomyxoma peritonei: Morphological and clinical findings, differential diagnosis, and prognosis

Background: Low-grade appendiceal mucinous neoplasms (LAMN) of the appendix have bland cytological features and well-differentiated morphology. Despite this, they may cause a pseudomyxoma peritonei (PMP) disease characterized by mucinous deposits in the peritoneal cavity and may exhibit malignant behavior. Aims and Objectives: In this study, we evaluated the clinical course and histopathological findings of LAMN. The rate of progression to PMP, factors affecting its development, and the clinical course of cases with PMP were investigated. Materials and Methods: Twelve thousand and forty-seven cases who underwent appendectomy were reviewed, and 71 mucinous lesions cases whose slides are in our archive were included in the study. Histopathological findings were re-evaluated. Morphological findings that guide the differential diagnosis, the clinical course of the patients, and factors affecting PMP development were determined. Results: The cases were divided into group 1 non-neoplastic (9.9%), group 2 benign (18.3%), group 3 LAMN (60.6), and group 4 malignant neoplasms (11.3%). Age, gender, appendix diameter, gross appearance, epithelial characteristics, and presence of microcalcification were significantly different between the patient groups. The presence of mucin in the ileocecal region was found to be significant in the development of PMP. It was observed that the additional procedure performed after the appendectomy did not prevent a recurrence. Conclusion: LAMNs are lesions with characteristic findings and different behaviors. The only method to distinguish from the lesions included in the differential diagnosis is to interpret the histopathological findings correctly. Additional operations after appendectomy do not contribute to preventing recurrence.

Abordaje laparoscópico de la neoplasia mucinosa apendicular de bajo grado / Laparoscopic approach of low-grade appendiceal mucinous neoplasm

RESUMEN Las neoplasias mucinosas apendiculares son infrecuentes y se clasifican en neoplasias mucinosas de bajo grado, de alto grado y adenocarcinoma mucinoso. Se consideran precursoras del pseudomixoma peritoneal, caracterizado por la acumulación de mucina dentro de la cavidad abdominal. Informamos tres casos de neoplasia mucinosa apendicular de bajo grado, dos varones y una mujer, que presentaron dolor abdominal agudo, diagnosticados preoperatoriamente mediante tomografía y que fueron manejados con un abordaje laparoscópico (apendicectomía, resección parcial de ciego y colectomía derecha, respectivamente). Los tres casos tuvieron una buena evolución posoperatoria y no hubo complicaciones. Se concluye que las neoplasias mucinosas apendiculares son raras y no tienen una presentación clínica específica. El abordaje laparoscópico es una opción segura y factible. La frecuencia y la duración del seguimiento de estos pacientes es motivo de controversia.

ABSTRACT Appendiceal mucinous neoplasms are rare and are classified in low-grade mucinous neoplasm, high-grade mucinous neoplasm and mucinous adenocarcinoma. They are considered precursors of pseudomyxoma peritonei, characterized by the accumulation of mucin within the abdominal cavity. We report three cases of low-grade appendiceal mucinous neoplasm, two men and one woman, who presented with acute abdominal pain. The diagnoses were made preoperatively by computed tomography scan and the tumors were managed through laparoscopic approach (appendectomy, partial resection of the cecum and right colectomy, respectively). The three patients evolved with favorable postoperative outcome without complications. Appendiceal mucinous neoplasms are rare without specific clinical presentation. The laparoscopic approach is a safe and feasible option. The frequency and duration of surveillance in these patients are still controversial.

Research progress in pathology and imaging of intraductal papillary mucinous neoplasms of the pancreas / 国际生物医学工程杂志

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a cystic tumor of the pancreas with some malignant potential. According to the degree of pancreatic duct expansion and the location of the tumor, it can be divided into main-duct type, branch-duct type, and mixed type. According to the degree of atypia, it can be classified as low-grade dysplasia, high-grade dysplasia, or invasive IPMN. According to histological morphology and immunohistochemistry, there are four subtypes: gastric type, intestinal type, pancreaticobiliary type, and eosinophilic type. Ultrasound is commonly used in screening and follow-up, while CT, MRI/MRCP, and EUS can identify IPMN with high-risk and anxiety characteristics, which are valuable in determining the timing of surgery and patient status. Imaging omics is highly accurate in assessing the degree of dysplasia and can be used to identify people at high risk of malignant transformation. In this article, the clinical manifestations, pathology, and imaging features of IPMN are reviewed in the light of the relevant guidelines and literature in recent years, in order to deepen our understanding of IPMN and thus improve the accuracy of imaging diagnosis.

Neoplasia mucinosa apendicular / Appendiceal mucinous neoplasms

RESUMEN La neoplasia mucinosa apendicular es una entidad poco frecuente que representa el 0,2 al 0,7% de todas las apendicectomías. La forma de presentación más frecuente es el hallazgo incidental. También puede diagnosticarse du rante el estudio de un dolor abdominal crónico en el cuadrante inferior derecho o, de manera retros pectiva, por el hallazgo en la anatomía patológica en el marco de una apendicitis aguda. La apendicectomía es el tratamiento indicado; la técnica tiene como objeto impedir la perforación del apéndice para evitar el vuelco a la cavidad peritoneal de moco o células neoplásicas y de esa manera prevenir el desarrollo del pseudomixoma peritoneal.

ABSTRACT Appendiceal mucinous neoplasms are rare and represent 0.2-0.7% of all appendectomies. They usual present as an incidental finding. The diagnosis can be made during the evaluation of chronic abdomi nal pain in the right lower quadrant or may emerge in the pathology report in the setting of an acute appendicitis. Appendectomy is the treatment of choice and care must be taken to avoid perforation of the appendix with dissemination of neoplastic cells or mucus into the peritoneum and thus prevent the develop ment of pseudomyxoma peritonei.

Neoplasia papilomucinosa intraductal de páncreas: experiencia a 10 años, en un único centro / Intraductal papillary mucinous neoplasm of the pancreas: A 10-year experience at a single center

RESUMEN Antecedentes: Debido a la mejoría de los métodos diagnósticos, la incidencia de la neoplasia papilar mucinosa intraductal del páncreas (NPMI) ha aumentado en los últimos años. Objetivo: Presentar la experiencia en el manejo alejado de pacientes con NPMI en un centro de referencia. Material y métodos : Se realizó análisis retrospectivo de pacientes que consultaron al Servicio de Ci rugía General y HPB del Hospital Universitario Fundación Favaloro, por patología pancreática tumo ral entre enero de 2010 y diciembre de 2019. Los pacientes se clasificaron en 2 grupos: A) aquellos con diagnóstico en consultorio de NPMI y B) aquellos en los que la NPMI fue un hallazgo en la pieza quirúrgica (B). Resultados: Ochenta y seis pacientes fueron analizados: 79 (90%) se incluyeron en el grupo A y 7 en el grupo B. De los pacientes del grupo A, en 57 casos (66%) se decidió conducta expectante y seguimiento (AS). De los 22 pacientes restantes, 18 fueron operados (29%) (AC) y 4 tienen la cirugía pendiente. Para el grupo A, la supervivencia a los 5 años fue de 89% para el grupo AS, de 86% en grupo AC, y del 43% para el grupo B (Breslow 0,001, Log-Rank 0,001 vs. grupo A). Conclusión: El diagnóstico y manejo de la NPMI está hoy estandarizado, en las tipo I y III está indicada cirugía, la tipo II debe seguirse por riesgo de malignización; cuando se le indica cirugía, la supervivencia a largo plazo debe ser similar a la del grupo en seguimiento.

ABSTRACT Background: The incidence of intraductal papillary mucinous neoplasm (IPMN) of the pancreas has increased over the past years along with the development of diagnostic imaging tests. Objective: The aim of this study is to describe our experience on long-term management of patients with IPMNs in a reference center. Material and methods: We conducted a retrospective and descriptive analysis of patients with pancreatic neoplasms followed-up at the Department of General Surgery and Hepato-Biliary Surgery, Hospital Universitario Fundación Favaloro, between January 2010 and December 2019. The patients were classified into 2 groups: group A (diagnosis of IPMN made in the outpatient clinic), and group B (diagnosis of IPMN in the pathological examination). Results: Eighty-six patients were analyzed: 79 (90%) in group A and 7 in group B. In group A, a watchful waiting with monitoring (AM) was decided in 57 cases (66%). Of the remaining 22 patients, 18 (29%) patients underwent surgery (AS) and 4 are waiting for surgery. Survival at 5 years was 89% in group AM, 86% in group AS and 43% in group B (Breslow 0.001, log-rank test 0,001 vs. group A). Conclusion : The diagnosis and management of IPMNs is currently standardized. Surgery is indicated in MD-IPMN and mixed type IPMN. Patients with BD-IPMN type should be monitored due to the risk of malignant transformation. When surgery is indicated, long-term survival should be similar to that of the surveillance group.

Adenocarcinoma mucinoso de vesícula biliar originado en neoplasia papilar intracolecística / Mucinous adenocarcinoma of the gallbladder originated from intracholecystic papillary neoplasm

RESUMEN La neoplasia papilar intracolecística con carcinoma mucinoso invasor y células en anillo de sello es una variedad de cáncer de vesícula, una patología agresiva y con mal pronóstico, la cual puede presentarse en forma similar a una colecistitis y, a pesar del tratamiento operatorio y quimioterapia posterior, la supervivencia y pronóstico son peores en relación con los otros cánceres de vesícula. Este artículo tie ne por objetivo describir un tipo histológico muy específico, de baja frecuencia, de cáncer de vesícula y el tratamiento realizado.

ABSTRACT Intracholecystic papillary neoplasm with invasive mucinous adenocarcinoma and signet ring cells is a rare, aggressive variety of gallbladder cancer, with symptoms mimicking cholecystitis. Survival and prognosis are worse that other types of gallbladder cancer despite surgery and chemotherapy. The aim of this article is to describe a case of a rare gallbladder cancer with specific histology and the treatment performed

Endoscopic retrograde cholangiopancreatography combined with SpyGlass in the diagnosis of intraductal papillary mucinous neoplasms of the bile duct / 中华消化内镜杂志

To evaluate the value of endoscopic retrograde cholangiopancreatography(ERCP)and SpyGlass in the diagnosis of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B). Data of patients who underwent ERCP and SpyGlass in Hangzhou First People′s Hospital from January 2016 to December 2019 were analyzed. ERCP and SpyGlass features, complications, clinicopathologic characteristics and prognosis were retrospectively analyzed.A total of 9 patients (5 benign lesions and 4 malignant lesions) were included.ERCP was successfully performed in 9 cases, while SpyGlass was technically successful in 8 cases. Endoscopy showed mucus outflow from the papilla in 5 cases, and the mucus was removed by the balloon of ERCP in 8 cases.ERCP showed bile duct diffuse dilatation and filling defects in all patients. SpyGlass found the mucus in the bile duct in all patients. SpyGlass showed lesion mucosa were fish-egg like without vascular images (Ⅱtype, 3 cases), fish-egg like with vascular images (Ⅲ type, 1 case), villous (Ⅳtype, 4 cases). SpyGlass defined extent of the lesion in 8 cases. SpyGlass found that the lesion involved the intra and extrahepatic bile ducts in one case. Therefore, liver transplantation was recommended to avoid surgical exploration. One type Ⅲ lesion underwent a direct biopsy. The pathology showed moderate dysplasia, which was consistent with the postoperative pathology. No complication occurred. ERCP combined with SpyGlass could clarify the scope of IPMN-B and provide basis for surgical options, which is safe and effective in IPMN-B diagnosis.

Neoplasia Mucinosa de apéndice. Abordaje laparoscópico. Reporte de caso / Neoplastic appendiceal mucinous lesion. Laparoscopic approach. Case report

El mucocele apendicular es la dilatación quística del apéndice con acumulación de material mucinoso. Su forma de presentación más frecuente es dolor en fosa ilíaca derecha, sin embargo, la mitad de los casos son asintomáticos. En el presente trabajo se presenta caso de un paciente con diagnóstico incidental de mucocele apendicular, al cual se le realizó apendicectomía laparoscópica como tratamiento definitivo. Caso clínico: Paciente masculino de 44 años sin antecedentes patológicos conocidos, quien se realiza ultrasonido abdominal que informa tumor quístico en fosa ilíaca derecha. Se decide resolución quirúrgica laparoscópica. Informe histológico: mucocele apendicular sin evidencia de malignidad y recuperación satisfactoria del paciente. Conclusión: La neoplasia mucinosa del apéndice es una patología poco frecuente, puede presentarse con una variedad de manifestaciones clínicas hasta un hallazgo incidental. El tratamiento es fundamentalmente quirúrgico y el abordaje laparoscópico es de elección en pacientes sin evidencia de ruptura y diseminación de la enfermedad(AU)

The appendicular mucocele is a cystic dilation of the appendix with accumulation of mucinous material. The most frequent form of presentation is pain in the right iliac fossa; however, half of the cases are asymptomatic patients. In the present work we present a case of a patient with an incidentally diagnosis of appendicular mucocele who underwent laparoscopic appendectomy as definitive treatment. Clinical case: A 44-year-old male patient with no known pathological history, who underwent an abdominal ultrasound that reported a cystic tumor in the right iliac fossa. Laparoscopic surgical resolution was decided, the histological examination revealed appendicular mucocele without evidence of malignancy and satisfactory recovery of the patient. Conclusion: Mucinous neoplasia of the appendix is an uncommon pathology, patients are often asymptomatic or have nonspecific symptoms, and the diagnosis is made incidentally in the course of other examination. Treatment is primarily surgical and the laparoscopic approach is the choice in patients without evidence of rupture and dissemination of the disease(AU)

Clinicopathological analysis and surgical strategy of primary appendiceal neoplasms / 中华胃肠外科杂志

Objective: To investigate the clinicopathological features, surgical methods and prognosis of primary appendiceal neoplasms. Methods: A descriptive case series study was performed. Clinical data of patients diagnosed with primary appendiceal neoplasms in Peking University First Hospital from 2006 to 2017 were retrospectively analyzed. Patients who underwent surgery and were confirmed as appendiceal neoplasms by postoperative pathology were included. Cases of cecal tumor invading the appendix and other organ tumors implanting in the appendix and cases of recurrent appendix tumors were excluded Pathological classification was based on the 4th edition of the WHO classification of digestive tract tumors (2010 edition), and the efficacy of operation methods of low grade appendiceal mucinous neoplasm (LAMN) were analyzed. Results: A total of 115 patients were enrolled, including 52 males and 63 females with a median age of 59 (51, 71) years. Clinical symptoms usually manifested as dormant pain in the right lower quadrant, migrating right lower abdominal pain, fever and bloating. Twenty-four cases were accidentally discovered during surgery, and 21 cases were found by physical examination. The preoperative diagnosis rate of CT and ultrasound was 40.2% (43/107) and 25.5% (24/94) respectively. The postoperative pathological types contained 83 cases of LAMN, 12 cases of mucinous adenocarcinoma, 9 cases of appendiceal neuroendocrine neoplasms (aNEN), 2 cases of mucinous adenocarcinoma with signet ring cells, 3 cases of serrated adenoma, 2 cases of goblet cell carcinoid, 2 cases of lymphoma, 1 case of leiomyoma and 1 case of schwannomas. All the patients underwent surgical resection, including 41 cases of appendectomy, 21 cases of partial cecectomy, 48 cases of right hemicolectomy, and 5 cases of combined organ resection due to appendiceal tumor infiltration or dissemination. Eighteen cases were diagnosed with pseudomyxoma peritonei (PMP) during operation. A total of 98 patients were enrolled for follow-up. The median follow-up time was 58 (5-172) months. The 5-year disease-free survival (DFS) rate was 84.5% and 5-year overall survival (OS) was 88.2%. Multivariate analysis revealed that high-grade malignancy tumors (HR=25.881, 95% CI: 2.827-236.935, P=0.004) and PMP formation (HR=42.166, 95% CI: 3.470-512.439,P=0.003) were independent risk factors for prognosis. Patients undergoing right hemicolectomy presented longer operation time, more blood loss and higher morbidity of complication as compared to those undergoing appendectomy and partial cecectomy (all P<0.05), while no significant differences in 5-year DFS (P=0.627) and 5-year OS (P=0.718) were found. Conclusions: Primary appendiceal neoplasms usually have no typical features, accompanied with low preoperative diagnosis rate. The common pathological types are LAMN, mucinous adenocarcinoma and aNEN. Appendectomy or partial cecectomy for LAMN may achieve satisfactory prognosis. High-grade malignancy tumors and PMP formation are independent risk factors for prognosis.

MRI characteristics and malignancy risk prediction model for intraductal papillary mucinous neoplasm of the pancreas / 中华胰腺病杂志

Objective:To investigate the MRI features of intraductal papillary mucinous tumor (IPMN) of the pancreas and establish a prediction model for predicting the malignancy risk.Methods:The clinical data of 260 IPMN patients who underwent MRI and pathological confirmed in the First Affiliated Hospital of Naval Medical University from October 2012 to April 2020 were retrospectively analyzed. According to the pathological results, all patients were divided into benign group (including IPMN with low-grade dysplasia) and malignant group (including IPMN with high grade dysplasia and invasive carcinoma). According to international consensus of prediction model modeling, patients were divided into training set and validation set in chronological order. A prediction model was developed based on a training set consisting of 193 patients (including 117 patients with benign IPMN and 76 patients with malignant IPMN) between October 2012 and April 2019, and the model was validated in 67 patients (including 40 patients with benign IPMN and 27 patients with malignant IPMN) between May 2019 and April 2020. The multivariable logistic regression model was adopted to identify the independent predictive factors for IPMN malignancy and establish and visualized a nomogram. The ROC was drawn and AUC was calculated. The decision curve analysis was used to evaluate its clinical usefulness.Results:The IPMN type, cyst size, thickened cyst wall, mural nodule size, diameter of main pancreatic duct (MPD) and the abrupt change in the caliber of the MPD with distal pancreatic atrophy in the training set and validation set, and jaundice and lymphadenopathy in the training set were significantly different between benign group and malignant group ( P<0.05). The multivariable logistic regression model of characteristics included the jaundice, cyst size, mural nodule size ≥5 mm, the abrupt change in caliber of the MPD with distal pancreatic atrophy were independent risk factors for IPMN maligancy. The model for predicting IPMN malignancy was -0.35+ 2.28×(jaundice)+ 1.57×(mural nodule size ≥5 mm)+ 2.92×(the abrupt change in caliber of the MPD with distal pancreatic atrophy)-1.95×(cyst <3 cm)-1.05×(cyst≥3 cm). The individualized prediction nomogram using these predictors of the malignant IPMN achieved an AUC of 0.85 (95% CI 0.79-0.91) in the training set and 0.84 (95% CI 0.74-0.94) in the validation set. The sensitivity, specificity and accuracy of the training set were 72.37%, 85.47% and 80.31%, respectively. The sensitivity, specificity and accuracy of the validation set were 81.48%, 75.00% and 77.61%, respectively. The decision curve analysis demonstrated that when the IPMN malignancy rate was >0.16, the nomogram diagnosing IPMN could benefit patients more than the strategy of considering all the patients as malignancy or non-malignancy. Conclusions:The nomogram based on MRI features can accurately predict the risk of malignant IPMN, and can be used as an effective predictive tool to provide more accurate information for personalized diagnosis and treatment of patients.

Progress in diagnosis and treatment for intraductal papillary mucinous neoplasm of pancreas / 中华肝胆外科杂志

The intraductal papillary mucinous neoplasm (IPMN) become the focus of clinical study because of the potential malignancy. Based on the latest clinical guidelines and relevant literatures, this review summarized the epidemiologic features, pathological and histological features, pathogenesis, clinical manifestations, laboratory and radiology examination, therapy and follow-up to better understand the diagnosis and treatment for IPMN.

Colloid carcinoma arising from intraductal papillary mucinous neoplasm of pancreas: imaging features and differentiation from ductal adenocarcinoma / 中华放射学杂志

Objective:To investigate the imaging features of colloid carcinoma arising from intraductal papillary mucinous neoplasm (IPMN) of pancreas and the differentiation features from ductal adenocarcinoma arising from IPMN, using the pathological findings as the reference.Methods:Twenty-four patients with pathologically confirmed colloid carcinoma from November 2013 to January 2020 in Changhai Hospital, Navy Medical University were included in this study. The clinical manifestations, imaging features and pathological data were retrospective reviewed. Thirty patients of ductal adenocarcinoma arising from IPMN confirmed by pathology were selected as the control group. CT and MRI features of two groups were blindly analyzed by two radiologists, including the lesions location, type of IPMN, size, components, density or signal, calcification, dilation and size of the main pancreatic duct (MPD), pancreatic parenchymal atrophy, fistula formation. The χ 2 test or Fisher exact probability was used to compare the imaging features between the two groups. Results:As for IPMN with colloid carcinoma, 16 cases were located in the head of the pancreas, 7 cases in the body and tail of the pancreas, and 1 case showed diffused changes of the pancreas. Mass was found in twenty-two cases, with the size of 54.5 (29) mm. Nineteen cases were solid-cystic, 4 were cystic and 1 was solid. Thick wall and internal separation with mild enhancement were displayed. Five cases were found with high signal on T 1WI. Thirteen cases had calcification and 2 cases had gas in the tumor. The size of MPD was (13±5) mm. Pancreatic parenchymal atrophy was found in 21 cases and fistula formation was found in 8 cases. The mass size of IPMN with colloid carcinoma was significantly greater than that of IPMN with ductal adenocarcinoma [31 (16) mm, Z=-3.758, P<0.001]. Solid-cystic mass was more found in IPMN with colloid carcinoma and solid mass was more found in IPMN with ductal adenocarcinoma ( P<0.001). Calcification ( P=0.001), fistula formation ( P=0.031), and high signal on T 1WI ( P=0.034) were more found in IPMN with colloid carcinoma than IPMN with ductal adenocarcinoma. Conclusion:Compared with IPMN with ductal adenocarcinoma, the solid-cystic mass, calcification, fistula formation and high signal on T 1WI were more commonly found in IPMN with colloid carcinoma.

Atypical imaging findings of common pancreatic tumors / 中华消化外科杂志

The particularity of pancreatic anatomical location, the complexity of secretory function, and the diversity of pathology lead to complex imaging findings of pancreatic tumors. The common pancreatic tumors include pancreatic ductal adenocarcinoma, solid pseudopaillary neo-plasm, neuroendocrine neoplasm, intraductal papillary mucinous neoplasm, serous cystadenoma and mucinous cystic neoplasm. Atypical imaging findings are important reasons for misdiagnosis. Based on relevant clinical experiences, the authors analyze and summarize the atypical imaging findings of six kinds of common pancreatic tumors, aiming to improve radiologists and clinicians comprehensive understanding of pancreatic tumors.

Perfil clínico y análisis de sobrevida en pacientes con neoplasia mucinosa papilar intraductal diagnosticados por ecoendoscopía / Clinical profile and survival analysis in patients with intraductal papilar mucinous neoplasm by endoscopic ultrasound

RESUMEN Introducción. La neoplasia mucinosa papilar intraductal (IPMN) se diagnostica ahora con mayor frecuencia debido al mayor uso de los métodos de imágenes, y conlleva un desafío clínico su manejo y seguimiento por su probable transformación maligna. Objetivos. Conocer las características por ecoendoscopía (USE), evolución clínica y sobrevida de los pacientes diagnosticados de IPMN por USE. Materiales y métodos. Estudio de cohorte retrospectivo con análisis de sobrevida en pacientes diagnosticados de IPMN por USE entre 2013 y 2018 en el Hospital Nacional Edgardo Rebagliati Martins (HNERM). Se analizaron variables demográficas y ecoendoscópicas, además de seguimiento hasta el fallecimiento o 6 meses después del término del estudio. Se realizó el análisis de sobrevida con los métodos de Kaplan-Meyer y de regresión de Cox. Resultados. Se incluyeron 133 pacientes con IPMN. Edad media fue 68,6 años, 80 (60,2%) fueron mujeres. Según subtipos, 89 (66,9%) fueron de rama secundaria, 23 (17,3%) de ducto principal y 21 (15,8%) de tipo mixto. La principal localización fue cabeza de páncreas (41,4%). En el seguimiento, 22 (16,5%) fueron a cirugía, 22 (16,5%) fallecieron después de una mediana de seguimiento de 522 días. En 8 pacientes (6%) se detectó neoplasia maligna. La tasa de sobrevida global fue 86.8% (IC 95%, 79,6-91,6) al año y de 81.9% (IC95%, 73,3-88,0) a los 3 años. En análisis univariado los factores asociados a la sobrevida fueron los subtipos de IPMN-DP (p=0,02) y mixto (p=0,005), sexo masculino (p=0,004), tamaño de lesión ≥30 mm (p=0,000), nódulos (p=0,014) y Wirsung ≥10 mm (p=0,01). En el análisis multivariado, los factores predictores asociados con la sobrevida fueron: IPMN-DP (HR=6,3, p=0,005), IPMN mixto (HR=4,9, p=0,008) y tamaño de lesión ≥30 mm (HR=7,1, p=0,000). Conclusiones. El diagnostico de IPMN de ducto principal y mixto se asocian como factores predictores de sobrevida, al igual que el tamaño de la lesión ≥30 mm.

ABSTRACT Introduction: Intraductal papillary mucinous neoplasms (IPMN) are diagnosed more frequently because the higher use of radiologic exams, in that sense they are a great challenge to define its management and treatment in relation to its potential malignant transformation. Objective: To describe IPMN clinical profile, endoscopic ultrasound (EUS) characteristics and survival in all patients diagnosed with IPMN by EUS at HNERM. Materials and methods: Retrospective cohort of patients with IPMN diagnosed at HNERM by EUS from 2013 to 2018. Descriptive statistics was used for clinical profile and EUS characteristics. Kaplan Meir Method and Cox regression analysis was applied for survival analysis. Results: 133 patients with IPMN were included. Medium age was 68.6 years, 80 (60.2%) were female. According to IPMN subtypes, 89 (66.9%) originated from secondary branch, 23 (17.3%) from main duct (MD) and 21 (15.8%) were mixed type (MT). Head of pancreas was the main localization (41.4%). In follow-up, 22 (16.5%) were derived to surgery. Mortality occurred in 16.5% (22 cases) after a median follow-up of 522 days. Malignant transformation was diagnosed in 6% (8 cases). Survival was 86.8% (IC 95%, 79.6-91.6) at 1 year and 81.9% (IC95%, 73.3-88.0) at 3 years. Univariate analysis demonstrated that factors associated to survival were MD-IPMN (p=0.02) y MT-IPMN (p=0.005), male gender (p=004), nodule size ≥30 mm (p=0.000), presence of nodules (p=0.014) and Wirsung ≥10 mm (p=0.01). Multivariate analysis showed that predictive factors for survival were MD-IPMN (HR=6.3, p=0.005), MT-IPMN (HR=4.9, p=0.008) and nodule size ≥30 mm (HR=7.1, p=0.000). Conclusions: Diagnosis of MD-IPMN and MT-IPMN are predictive factors for survival as well as nodule size ≥ 30mm.

A prospective study of discrepancy between clinical and pathological diagnosis of appendiceal mucinous neoplasm

PURPOSE: Appendiceal tumoral lesions can occur as benign, malignant, or borderline disease. Determination of the extent of surgery through accurate diagnosis is important in these tumoral lesions. In this study, we assessed the accuracy of preoperative CT and identified the factors affecting diagnosis.METHODS: Patients diagnosed or strongly suspected from July 2016 to June 2019 with appendiceal mucocele or mucinous neoplasm using abdominal CT were included in the study. All the patients underwent single-incision laparoscopic cecectomy with the margin of cecum secured at least 2 cm from the appendiceal base. To compare blood test results and CT findings, the patients were divided into a mucinous and a nonmucinous group according to pathology.RESULTS: The total number of patients included in this study was 54 and biopsy confirmed appendiceal mucinous neoplasms in 39 of them. With CT, the accuracy of diagnosis was 89.7%. The mean age of the mucinous group was greater than that of the nonmucinous group (P = 0.035). CT showed that the maximum diameter of appendiceal tumor in the mucinous group was greater than that in the nonmucinous group (P < 0.001). Calcification was found only in the appendix of patients in the mucinous group (P = 0.012). Multivariate analysis revealed that lager tumor diameter was a factor of diagnosis for appendiceal mucinous neoplasm.CONCLUSION: The accuracy of preoperative diagnosis of appendiceal mucinous neoplasms in this study was 89.7%. Blood test results did not provide differential diagnosis, and the larger the diameter of appendiceal tumor on CT, the more accurate the diagnosis.

Appendiceal schistosomiasis complicated by low - grade appendiceal mucinous neoplasm: a report of two cases / 中国血吸虫病防治杂志

This report presents two cases with appendiceal schistosomiasis complicated by low-grade appendiceal mucinous neoplasm.

Intraductal Papillary Mucinous Neoplasm Presenting as Bleeding Duodenal Mass: A Surgical Rarity

@#Intraductal papillary mucinous neoplasm (IPMN) is a rare pancreatic neoplasm. The presentation varies from recurrent pancreatitis, steatorrhea and weight loss to incidental findings during imaging studies. The recognition of IPMN is crucial in deciding for prompt surgical intervention, which is the best treatment modality for this precancerous condition. Here, we report a case of 55-year-old man with massive upper gastro intestinal bleeding arising from a huge fungating duodenal mass. In view of massive bleeding, a decision for emergency Whipple's pancreaticoduodenectomy was made. Final histological diagnosis confirmed as IPMN. To the best of our knowledge, this is the first case of IPMN presented with a huge fungating duodenal mass causing massive UGIB requiring surgical intervention.

Characterization of Intraductal Papillary Neoplasm of the Bile Duct with Respect to the Histopathologic Similarities to Pancreatic Intraductal Papillary Mucinous Neoplasm

Intraductal papillary neoplasms of the bile duct (IPNBs) are known to show various pathologic features and biological behaviors. Recently, two categories of IPNBs have been proposed based on their histologic similarities to pancreatic intraductal papillary mucinous neoplasms (IPMNs): type 1 IPNBs, which share many features with IPMNs; and type 2 IPNBs, which are variably different from IPMNs. The four IPNB subtypes were re-evaluated with respect to these two categories. Intestinal IPNBs showing a predominantly villous growth may correspond to type 1, while those showing papillay-tubular or papillay-villous growth correspond to type 2. Regarding gastric IPNB, those with regular foveolar structures with varying numbers of pyloric glands may correspond to type 1, while those with papillary-foveolar structures with gastric immunophenotypes and complicated structures may correspond to type 2. Pancreatobiliary IPNBs that show fine ramifying branching may be categorized as type 1, while others containing many complicated structures may be categorized as type 2. Oncocytic type, which displays solid growth or irregular papillary structures, may correspond to type 2, while papillary configurations with pseudostratified oncocytic lining cells correspond to type 1. Generally, type 1 IPNBs of any subtype develop in the intrahepatic bile ducts, while type 2 IPNBs develop in the extrahepatic bile duct. These findings suggest that IPNBs arising in the intrahepatic ducts are biliary counterparts of IPMNs, while those arising in the extrahepatic ducts display differences from prototypical IPMNs. The recognition of these two categories of IPNBs with reference to IPMNs and their anatomical location along the biliary tree may deepen our understanding of IPNBs.

Surgical Indications and Postsurgical Follow-up Strategy for Pancreatic Cystic Neoplasm / 대한췌담도학회지

The increasing discovery of pancreatic cystic neoplasm is a recent trend because of the widespread use and development of imaging techniques. Physicians have to recognize the different characteristics of the cystic neoplasms so that a determination may be selected regarding the potential for malignancy. Appropriate evaluation of pancreatic cystic lesion includes a multidisciplinary approach involving gastroenterologists with experience in endoscopic ultrasound, radiologist, and pancreatic surgeons. The selective approach is important in management of this neoplasm with minimizing incorrect diagnosis and unnecessary surgery. Considering the characteristic features of pancreatic cystic neoplasm, the clinical decision should be tailored according to needs and conditions of the individual patients.

Predictive Value of Localized Stenosis of the Main Pancreatic Duct for Early Detection of Pancreatic Cancer

BACKGROUND/AIMS: In this study, we aimed to evaluate the predictive value of localized stenosis of the main pancreatic duct (MPD) for early detection of pancreatic cancer.METHODS: Among 689 patients who underwent endoscopic retrograde pancreatography from January 2008 to September 2018, 19 patients with MPD findings were enrolled. These patients showed findings for indicating suspicious pancreatic cancer at an early stage (FiCE); FiCE was defined as a single, localized stenosis in the MPD without a detectable mass (using any other imaging methods) and without other pancreatic diseases, such as definite chronic pancreatitis, intraductal papillary mucinous neoplasm, and autoimmune pancreatitis. Final diagnoses were established by examining resected specimens or through follow-up examinations after an interval of >5 years.RESULTS: Among 19 patients with FiCE, 11 underwent surgical resection and 8 were evaluated after a >5-year observation period. The final diagnosis of the MPD stenosis was judged to be pancreatic cancer in 9 patients (47%), including 3 with intraepithelial cancer, and to be a non-neoplastic change in 10. The sensitivity, specificity, and accuracy of preoperative pancreatic juice cytology were 75%, 100%, and 88%, respectively.CONCLUSIONS: The predictive value of FiCE for pancreatic cancer prevalence was 47%. Histological confirmation with pancreatic juice cytology is necessary before surgical resection.