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Background Non-operative management is often the treatment of choice in cases of complicated appendicitis and routine interval appendectomy is not usually recommended. Actually, recent studies show an alarming number of appendiceal neoplasms following interval appendectomy. The aim of this study is to evaluate the prevalence of appendiceal neoplasms and their histological types after interval appendectomy for complicated appendicitis in adults. Methods A comprehensive literature search of the PubMed, Scopus and Web of Science databases was conducted according to the PRISMA statement. Studies reporting appendiceal neoplasm rates after interval appendectomy and histopathological characteristics were included. The most recent World Health Organization (WHO) classification of malignant tumours was considered. A pooled prevalence analysis for both prevalence and pathology was performed. Results A total of eight studies was included: seven retrospective series and one randomized controlled trial. The pooled prevalence of neoplasms after interval appendectomy was 11% (95% CI 7-15; I2 = 37.5%, p = 0.13). Appendiceal mucinous neoplasms occurred in 43% (95% CI 19-68), adenocarcinoma in 29% (95% CI 6-51), appendiceal neuroendocrine neoplasm in 21% (95% CI 6-36), globet cell carcinoma in 13% (95% CI -2-28), adenoma or serrated lesions in 20% (95% CI -0-41) of cases. Conclusion The risk of appendiceal neoplasm in patients treated with interval appendectomy for complicated appendicitis is 11%; mucinous neoplasm is the most common histopathological type. Further studies should investigate this association in order to clarify the biological pathway and clinical implications.

Purpose: This study aimed to evaluate the safety and feasibility of single-port laparoscopic surgery (SLS) for appendiceal mucinous neoplasm (AMN) when compared with conventional laparoscopic surgery (CLS).Methods: This retrospective study enrolled patients who underwent surgery for AMN between July 2014 and June 2020 at Seoul National University Bundang Hospital. Patient demographics, surgical data, pathology, hospital stay, postoperative morbidity, and follow-up data were extracted from electronic records for analysis.Results: We enrolled 18 patients who underwent SLS and 22 who underwent CLS. The SLS group included patients who underwent partial cecectomy (14 patients), ileocecectomy (3 patients), and right hemicolectomy (1 patient). The CLS group included patients who underwent appendectomy (4 patients), partial cecectomy (11 patients), ileocecectomy (5 patients), and right hemicolectomy (2 patients). Operation type was not significantly different between groups (P = 0.213). No patient required open surgery in the SLS group in contrast to the CLS group (13.6%; P = 0.238). The operative time tended to be shorter in the SLS group than the CLS group (median [interquartile range]: 52.5 minutes [40–65.2 minutes] and 60 minutes [40–120 minutes], respectively; P = 0.251). Morbidity was 5.5% in the SLS group and 9.0% in the CLS group (P = 0.692). Surgical margins were clear in all cases. The median duration of postoperative hospital stay was 2.0 and 4.0 days in the SLS and CLS groups, respectively (P = 0.013). No recurrence occurred in either group during follow-up.Conclusion: This study indicates that SLS is a safe and feasible surgical approach for AMN.

Background Appendectomy for acute appendicitis is the most common procedure performed emergently by general surgeons in the United States. The current management of acute appendicitis is increasingly controversial as non-operative management gains favor. Although rare, appendiceal neoplasms are often found as an incidental finding in the setting of appendectomy. Criteria and screening for appendiceal neoplasms are not standardized among surgical societies. Methods The National Surgical Quality Improvement Program (NSQIP) database was queried for all patients who underwent appendectomy over a 9-year period (2010–2018). Over the same time period, patients who underwent appendectomy in two municipal hospitals in The Bronx, New York City, USA were reviewed. Results We found a 1.7% incidence of appendiceal neoplasms locally and a 0.53% incidence of appendiceal tumors in a national population sample. Both groups demonstrated an increased incidence of appendiceal carcinoma by age. This finding was most pronounced after the age of 40 in both local and national populations. In our study, the incidence of appendiceal tumors increased with each decade interval up to the age of 80 and peaked at 2.1% in patients between 70 and 79 years. Conclusions Appendiceal adenocarcinomas were identified in patients with acute appendicitis that seem to be associated with increasing age. The presence of an appendiceal malignancy should be considered in the management of older patients with acute appendicitis before a decision to embark on non-operative therapy.

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

Background At present, the term mucocele is outdated, and mucinous appendiceal neoplasm is preferred. Mucinous appendiceal neoplasm is an uncommon pathology that occurs predominantly in middle-aged women. Its classification and management have been the subject of debate in recent decades. The aim of this study was to analyse the incidence, clinical management and survival of these tumours diagnosed in our centre in the last 10 years. Methods This was a retrospective observational study of patients with a diagnosis of appendiceal neoplasms between 2009 and 2018 in our centre. Variables such as sex, age, tumour type, clinical status, diagnosis, treatment and survival were collected. All data were analysed using the statistical program IBM SPSS Statistic® version 25. Results Twenty-nine patients with a diagnosis of appendiceal neoplasm were identified, and 24 corresponded to neoplastic appendiceal mucinous lesions (85.7%). The average age was 59.7 ± 17.6 years. Most patients were women (15 cases; 62.5%). Most of them presented with chronic abdominal pain (37.5%), and the diagnosis was performed by computed tomography (CT) (50%). The treatment was surgical in all cases. The surgical technique depended on the findings and histology of the tumour. Conclusion Mucinous appendiceal neoplasms are an uncommon entity, and their pathological classification and management have recently changed.

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Mucinous appendiceal tumors include low-grade appendiceal mucinous neoplasm, high-grade appendiceal mucinous neoplasm, and mucinous adenocarcinoma. Nonmucinous adenocarcinomas are less frequent. Recent consensus guidelines and the latest edition of the World Health Organization classification will allow consistent use of agreed nomenclature. Accurate diagnosis is important not only for patient management but also to allow comparison of results between centers and tumor registries. Serrated polyps are the most common benign polyp in the appendix. They need to be distinguished from low-grade appendiceal mucinous neoplasm, which can also mimic other benign conditions. Goblet cell adenocarcinomas are a distinctive type of appendiceal neoplasm.

Two and more types of tumors rarely occur in the appendix, a constrained space of the human body. We experienced a rare co-occurrence of schwannoma and low-grade mucinous neoplasm (LAMN) in the appendix and obtained a lesson to scrutinize the radiological images in the follow-up period. A computer tomography (CT) performed for surveillance of bile duct stone of a 68-year-old man incidentally detected the appendiceal tumor, which characteristics were not clear at that time, 1-year before admission because of epigastric pain due to acute cholecystitis. CT showed enlargement of tumor in the appendix, and laparoscopic ileocecal resection and cholecystectomy were performed under the diagnosis of acute cholecystectomy and appendiceal tumor. Histopathological examination revealed two types of appendiceal tumors, schwannoma and low-grade appendiceal neoplasm. To the best of our knowledge, co-occurrence of schwannoma and LAMN has never been reported and this complicated our interpretation of clinical images during the course. We report a clinical lesson in handling this rare combination of tumors in the appendix with the relevant literature.

Mucocele of the appendix is a rare entity in pediatrics, and few case reports have been reported in the literature. We present the case of a 5-month-old male with abdominal distension, constipation, and vomiting who was found to have an appendiceal mucocele while being evaluated for total colonic aganglionosis. Laparoscopic-assisted resection was performed, with pathology confirming a low-grade mucinous neoplasm of the appendix. He underwent complete resection, rendering him cured without the need for any further resection or treatment.

Objective The aim of this study was to investigate the clinicopathological characteristics, management, and survival of patients with appendiceal mucinous neoplasm misdiagnosed as ovarian tumor and initially treated by a gynecological oncologists. Methods We retrospectively reviewed data for 71 patients identified from January 1, 2006 to September 31, 2018 with a final pathological diagnosis of appendiceal mucinous neoplasm. All patients were diagnosed as having ovarian tumor preoperatively and initially treated at Department of Gynecological Oncology. Results The median patients' age was 61 years, and the median peritoneal cancer index (PCI) was 16, with a complete cytoreduction score (CCR0/1) achieved in 46.47% of patients. Two patients received hyperthermic intraperitoneal chemotherapy (HIPEC). Estimated 5-year and 10-year survival was 76.7% and 53.4%, respectively. PCI and lactate dehydrogenase (LDH) levels were identified as independent predictors of poor overall survival. Conclusions Because of the high rate of ovarian metastases and the difficulty diagnosing appendiceal mucinous neoplasm preoperatively, this condition should be highlighted in gynecological oncology. Because initial surgical intervention is often performed by gynecological oncologists, updating the management guidelines for appendiceal mucinous tumor is needed because the rates of CCR0/1 resection and HIPEC are much lower in patients who are misdiagnosed.

Introduction Tumors of the ovary and appendix have been well documented in the setting of pseudomyxoma peritonei (PMP) with constant debate over tumor origin. Generally, these tumors are found to have a single primary origin, most commonly the appendix, with metastatic spread to the ovaries. Care presentation Here we present a 61-year-old female who underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH-BSO) for a primary mucinous ovarian carcinoma. She presented to our institution one year later with abdominal pain and a palpable right lower quadrant mass, which on histopathologic exam was found to be a primary low grade mucinous appendiceal neoplasm (LAMN), alluding to the potential of two separate primary disease processes. Discussion/conclusion With two primary, non-synchronous lesions, a thorough literature review suggests that during the patient's initial TAH-BSO, she could have additionally undergone an appendectomy. In doing so, this would provide accurate, complete staging and determine if the two neoplasms were truly primary in origin or metastatic. In addition, new genetic markers are being discovered, such as the Special AT-rich sequence-binding protein 2 (SATB2) marker, which has been found to be positive in those with a LAMN and negative in those with a primary mucinous ovarian carcinoma. By acquiring appropriate and complete staging we can better diagnose and treat these neoplasms.

Pseudomyxoma peritonei (PMP) is a rare condition usually associated with ruptured low-grade mucinous neoplasm of the appendix. Rarely, PMP can originate from mucinous adenocarcinoma of the ovary. However, the coexistence of adenocarcinoma of the endometrium and appendiceal mucinous neoplasm has not been reported. We present the case of a post-menopausal woman with endometrioid endometrial adenocarcinoma with unexpected low-grade appendiceal mucinous neoplasm and PMP.

Ovarian mucinous tumors are one of the common epithelial tumors in the ovary, but their recurrence is extremely rare. In a 37-year-old female patient who had had five operations in 7 years due to recurrent mucinous neoplasms, a mass extending to the umbilicus was recently detected during a routine examination. With this finding the patient underwent cystectomy, total abdominal hysterectomy, and left salpingo-oophorectomy. Two of the five operations were performed during cesarean delivery. In the recent surgical procedure, two cysts were removed. A 20 cm cyst in the pelvic region was diagnosed as a mucinous cystadenoma. The other cyst located in the myometrium was a mucinous cystadenoma with focal borderline change and arose in the previous cesarean scar. The authors present what is believed to be the first case of recurrent mucinous neoplasm arising within a cesarean scar. A review of the literature concerning the topic is also presented.

Low‐grade appendiceal mucinous neoplasms are rare and difficult to diagnose preoperatively because of a lack of characteristic symptoms. A 24‐year‐old female with no symptoms before pregnancy underwent an elective cesarean section at 38 weeks of gestation because of cephalo‐pelvic disproportion. Although no abnormalities were detected in the newborn, uterus, or uterine adnexa, a sausage‐like, swollen appendix was noted. The patient underwent appendectomy. Pathology showed the tumor was a low‐grade appendiceal mucinous neoplasm., Here, we report the case of a 24‐year‐old pregnant women with incidentally discovered low‐grade appendiceal mucinous neoplasm (AMN) during cesarean section. To the best of our knowledge, there is a preoperatively diagnosed AMN complicating pregnancy. Previous reports suggested that AMN is detected during an operation performed for incidental reasons, such as acute abdomen, in all cases.

Mucinous adenocarcinoma of the urachal remnant is a nonurothelial malignancy that may be asymptomatic until locally advanced or metastatic. We describe a 37-year-old woman with invasive ductal breast carcinoma who underwent 18Fluorine-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) computed tomography (CT) demonstrating a non-FDG avid pelvic mass, initially suspected to represent a pedunculated uterine fibroid. Magnetic resonance imaging revealed a mixed solid-cystic mass separate from the uterus, suspicious for urachal neoplasm, confirmed as mucinous adenocarcinoma on histopathology. Urachal tumors may not be FDG-avid and represent a potential pitfall on FDG PET/CT.

Aims High-grade appendiceal mucinous neoplasm (HAMN) was recently proposed as a disease entity histologically analogous to low-grade appendiceal mucinous neoplasm (LAMN), but characterised by high-grade cytological atypia. The pathogenesis and clinical features of HAMN have not been fully elucidated. Methods and results Nine cases of HAMN, eight LAMN, 10 appendiceal mucinous adenocarcinomas (MACA) and five appendiceal serrated polyps resected between 2008 and 2017 contributed by three medical centres underwent targeted next-generation sequencing of 50 cancer-related genes. The patients in each category had similar profiles with respect to gender, age, tumour stage and follow-up intervals. Both LAMN and HAMN harboured mutations of KRAS [nine of nine and eight of eight (100%), respectively] and GNAS [five of eight (63%) and five of nine (56%), respectively] in significantly higher proportions than MACA [KRAS, seven of 10 (70%, P = 0.04); GNAS: one of 10 (10%, P = 0.02)] and serrated polyps [KRAS, one of five (20%, P = 0.0007); GNAS: none of five (0%, P = 0.04)]. Four cases of HAMN, but none of LAMN, harboured mutations of TP53 [four of nine (44%)] and/or ATM [two of nine (22%)]. Three cases of HAMN (33%) showed extra-appendiceal spread with retention of the same mutational profiles in the intra- and extra-appendiceal components. The 10 cases of MACA harboured a similar prevalence of TP53 mutations (n = 5, 50%) as HAMN but, unlike LAMN and HAMN, some harboured mutations in PIK3CA, APC, FBXW7, PTEN and SMAD4. Conclusions HAMN and LAMN share high rates of KRAS and GNAS co-mutations supporting a common histogenesis and distinguishing them from MACA. Acquisition of TP53 or ATM mutations by HAMN may drive its progression to a more advanced phenotype.

Purpose Cystic lymphangioma of the pancreas was first recognized in 1913 as a form of benign cyst. It is extremely rare, accounting for less than 1% of the lymphangiomas. Case report Herein, we report a case of cystic pancreatic lymphangioma diagnosed in a 51-year-old female patient who was hospitalized for a colicky upper abdominal pain for a month. Radiological imaging revealed a large multiloculated cystic pancreatic mass with enhancing septations. The patient underwent distal pancreatectomy, and the histological examination showed cystic lymphangioma. Conclusion Although very uncommon, cystic lymphangioma should be placed in the differential diagnosis list of pancreatic cystic lesions, at least about the multi-septated ones in asymptomatic female patients which are discovered incidentally.

Endometriosis is a relatively common condition among women, and pancreatic endometriosis has been reported on rare occasions. Such pancreatic lesions are difficult to diagnose and distinguish from other cystic lesions of the pancreas preoperatively. This report describes a case of pancreatic endometriosis in a 51-year-old female patient. Imaging demonstrated an enlarging cyst with findings concerning for a mucinous neoplasm. The patient underwent robotic distal pancreatectomy and splenectomy. Histopathology revealed an endometriotic cyst. Pancreatic endometriosis can be difficult to distinguish from other lesions of the pancreas. Surgical resection should be undertaken in cases where malignancy is suspected.

Prostate cancer is the most common visceral malignancy diagnosed in males. Surveillance for post-treatment neoplasms is very crucial. Here we report the first case of recurrent metastatic prostate cancer presenting as acute appendicitis in a background of a high-grade appendiceal mucinous neoplasm. In addition, this case also includes an unusually early presentation of a secondary primary malignancy after radiation therapy. A 70-year-old male with a history of prostate adenocarcinoma status post-proton radiation therapy presented with recurrent poorly differentiated prostate adenocarcinoma with disease progression and extra-prostatic extension. He underwent salvage proton therapy and testosterone replacement therapy. Two years later, the patient presented with right lower quadrant pain. A computed tomography scan showed perforated acute appendicitis with intra-abdominal abscess, which was treated with interval appendectomy. Upon histologic analysis, metastatic prostatic adenocarcinoma was noted in the appendiceal wall and mesoappendix. In addition, an incidental background of high-grade appendiceal mucinous neoplasm was found. Four months later, he presented with persistent abdominal pain, rapid weight loss, fatigue, and fever for 3 months. An abdominal CT scan revealed a 6.1 cm rectal mass. Pathologic analysis diagnosed an aggressive post-radiation spindle cell sarcoma, intermediate to high grade. The patient opted for palliative care. This case shows that a clinical presentation of acute appendicitis in an older patient may sometimes portend a neoplastic rather than infectious etiology. Clinical history and patient epidemiology should always be considered when evaluating an older patient with clinical signs and symptoms of acute appendicitis.

Background Appendicular neoplasms are rare, most commonly as carcinoids followed by appendicular mucinous neoplasms (AMN). To date, there remains controversy regarding the best treatment of AMN and factors affecting its prognosis. Method Retrospective chart review of patients operated for appendicular pathology (January 2011–December 2018, follow up to December 2020) at our institution. For all AMN patients, data included pre-operative clinical presentation, and operative/post-operative findings. Results 12454 patients underwent appendectomy, of whom 50 (0.4%) had AMN histopathologically (mean age = 47.2). Most patients had laparoscopic appendectomy as primary surgery. Low grade AMN was the most common subtype (n = 41, 82%), and pseudomyxoma peritonei (PMP) was found in 8 (16%) patients. Based on histopathology and margin involvement, the 50 patients were categorized into 3 prognostic categories of recurrence risk (no risk, 24 patients; low risk, 8; high recurrence risk, 18 patients). Disease-free survival (DFS) was lowest for high recurrence risk group (P, Highlights • Laparoscopic approach may be feasible for appendiceal mucinous neoplasms (AMN). • AMN can be categorized into 3 risk recurrence groups (No risk, low, and high). • AMN with acellular mucin at the margin may not require secondary surgery. • Higher tumor markers may indicate high risk of AMN recurrence.

Appendiceal mucinous neoplasm (AMN) can present with a spectrum of disease. Predicting factors in development of pseudomyxoma peritonei (PMP) from AMN could aid in management and treatment. The aim of this study was to determine factors predictive of PMP from AMN. This was a retrospective multicenter study of all patients diagnosed with AMN from 2006-2017. Diagnosis of PMP was compared by (1) patient demographics, (2) tumor characteristics, and (3) surgery. Secondary end points were disease-specific survival (DSS) and overall survival (OS).One-hundred thirty-eight patients with AMN were identified. Thirty-six patients (26.1%) had a ruptured appendix at presentation, and 12 patients (8.7%) were diagnosed with PMP during the study period. Eight patients presented with PMP at the time of surgery. No demographic factors were predictive of PMP. Operative approach and extent of initial resection did not correlate with PMP. Tumor rupture at presentation was the only factor associated with PMP, though only 14% of patients who presented with simple rupture eventually progressed to PMP.OS was not different between those who were diagnosed with PMP and those who were not. DSS was significantly lower for the group diagnosed with PMP ( P = .007). Tumor rupture at presentation did not influence OS or DSS. The only factor found to be significantly associated with PMP was tumor rupture at presentation. Diagnosis of PMP did not affect OS but did lead to decreased DSS.In conclusion, though a majority of patients who presented with rupture did not go on to develop PMP, tumor rupture at presentation was the only factor significantly associated with PMP. Diagnosis of PMP did not affect OS at 5 years. In patients with AMN who present with a ruptured appendix on final pathology, we recommended continued surveillance, though overall risk of PMP is relatively low.

Occasionally, radiologically diagnosed acute appendicitis is found to harbour underlying appendiceal neoplasm on post-surgical histopathology. Conversely, a situation in which radiologically, the appendix demonstrates features consistent with an underlying tumour but post-operative pathology finds no evidence of neoplastic change is rare. We describe a case of a 50-year-old man who presented with a markedly dilated "mass-like" appendix with minimal inflammatory changes on a computed tomography scan. Radiological findings were suspicious for an appendiceal neoplasm/mucocele (i.e. low-grade mucinous neoplasm). However, the post-surgical histopathological diagnosis did not concur with the radiological diagnosis and instead demonstrated findings compatible with acute appendicitis without neoplastic change. In this case report we provide a histopathological correlation and an explanation as to how this may have happened with the hope of helping radiologists avoid this pitfall in the future.

Appendiceal mucinous neoplasm (AMN) is defined as a distended, mucous-filled appendix. It is a rare condition seen in only 8% of cases of appendiceal tumors. Appendiceal mucocele associated with endometriosis is extremely rare with only 16 cases described in the literature to date. In view of the scarcity of the finding, few data are available on preoperative assessment of AMN in women with endometriosis. The goal of the present study was to describe the imaging features that could be suggestive of AMN in the context of endometriosis, as well as the specific management and outcomes.

BACKGROUND Appendiceal tumor has recently been treated differently from colorectal cancer. However, obtaining knowledge of this disease is difficult because of its rareness. OBJECTIVE This study aimed to investigate the clinicopathological characteristics of appendiceal tumors in a Japanese cohort. DESIGN This was a nationwide multi-institution retrospective observational study. SETTING This study was conducted at the participating tertiary referral hospitals. PATIENTS Patients with appendiceal tumor who were diagnosed between 2000 and 2017 were included. MAIN OUTCOME MEASURES The primary outcome measured was the total survivability. RESULTS A total of 922 patients from the 43 facilities that participated were identified. Of these, 114 patients were diagnosed with cancer other than adenocarcinomas. The remaining 760 patients, with the exception of 48 patients with unknown survival data, were eligible for the final cohort analysis. Of these 760 patients, 515 (67.8%) had mucinous adenocarcinomas and 245 (32.2%) had nonmucinous adenocarcinomas. Within the mucinous adenocarcinoma group, 267 patients (35.1%) were diagnosed as having low-grade appendiceal mucinous neoplasm. The 5-year survival rate of mucinous adenocarcinoma (83.1%) was superior to that of nonmucinous adenocarcinoma (62.0%; p < 0.01). Regarding mucinous adenocarcinoma, multivariate analysis revealed that high differentiation grade (American Joint Committee on Cancer grade 2/3), distant metastases, and R2 operation were significantly associated with a higher 5-year mortality rate. The 5-year survival rate was significantly better for low-grade appendiceal mucinous neoplasms (93.3%) than for other mucinous adenocarcinomas (72.1%; p < 0.01). LIMITATIONS This study was limited by its retrospective study design. CONCLUSIONS We cumulatively investigated appendiceal tumors in a multicenter retrospective study; this is the first such report from Asia. Grouping the grades as per the American Joint Committee on Cancer was useful as a prognostic indicator of appendiceal mucinous adenocarcinomas, including low-grade appendiceal mucinous neoplasm. See Video Abstract at http://links.lww.com/DCR/B282. ANALISIS DE LAS CARACTERISTICAS CLINICOPATOLOGICAS DE LOS TUMORES APENDICULARES EN JAPON: UN ESTUDIO CLINICO RETROSPECTIVO COLABORATIVO MULTICENTRICO: UNA ENCUESTA NACIONAL JAPONESA: El tumor apendicular recientemente se ha tratado de manera diferente al cancer colorrectal. Sin embargo, obtener conocimiento de esta enfermedad es dificil debido a su rareza.Este estudio tuvo como objetivo investigar las caracteristicas clinicopatologicas de los tumores apendiculares en una cohorte Japonesa.Este fue un estudio observacional retrospectivo de multiples instituciones a nivel nacional.Este estudio se realizo en los hospitales de referencia terciarios participantes.se incluyeron pacientes con tumor apendicular que fueron diagnosticados entre el 2000 y 2017.El resultado primario medido fue la supervivencia total.Se identificaron un total de 922 pacientes de las 43 instalaciones que participaron. De estos, 114 pacientes fueron diagnosticados con cancer distinto de adenocarcinomas. Los 760 pacientes restantes, excepto 48 pacientes con datos de supervivencia desconocidos, fueron elegibles para el analisis de cohorte final. De estos 760 pacientes, 515 (67,8%) tenian adenocarcinomas mucinosos y 245 (32,2%) tenian adenocarcinomas no mucinosos. Dentro del grupo de adenocarcinoma mucinoso, 267 pacientes (35,1%) fueron diagnosticados con neoplasia mucinica apendicular de bajo grado (LAMN). La tasa de supervivencia a 5 anos del adenocarcinoma mucinoso (83.1%) fue superior a la del adenocarcinoma no mucinoso (62,0%) (p

Background Appendiceal mucinous neoplasms (AMNs) are a rare and complex spectrum of disease involving a mucinous tumor within the appendix, which can range from benign to malignant. If not limited to the appendix, the mucinous tumor can spread diffusely throughout the peritoneum. Objectives Because of the low incidence of AMN, this study describes the diagnosis and treatment process for this disease, which is not well known to most care providers. Methods An extensive analysis of the current literature, including incidence, diagnosis, and surgical treatment, was performed. A review of the pre- and postoperative needs for patients undergoing surgery was also conducted. Findings There is minimal information in the nursing literature about AMN and the complexity of surgical management. Nurses play an important role in caring for these patients and their unique needs both before and after surgery.

The management and surveillance of low-grade appendiceal mucinous neoplasm (LAMN) is a confusing topic in the colorectal MDM. This study was aiming to provide a local protocol for our trust for such cases. From prospectively maintained database, patients who underwent appendicectomy between 2011 and 2017 were identified. Those with histological confirmation of LAMN were included. Retrospective analysis of operative notes, investigations and follow-up, return to theatres or development of pseudomyxoma peritonei (PMP) was performed. Four thousand six hundred twenty-eight patients had appendicectomy; 36 were diagnosed with LAMN. Age range was 30-88 (20 females). Fifteen had their operation as emergency and 13 elective, either for abnormal-looking appendix on CT scan or as part of elective right hemicolectomy. In 8 patients, LAMNs were diagnosed incidentally during other surgery either intraoperatively or on histology. Two patients had mucus in the peritoneal cavity (not sampled). The remaining 34 had either contained mucocele or inflamed appendix. Regarding follow-up, 28 patients had CT scans only, and 2 had ultrasound scan (USS). Two had both CT and USS. Three had CT and MRI scans. One patient did not have radiological investigations. Eighteen patients had colonoscopies (50%). Three patients had no follow-up, while 33 had 5-year follow-up. Ten patients are still currently under follow-up. None of the 36 patients required further surgeries related to LAMN, and none has developed PMP to date of the study. We have developed a local protocol based on our findings and literature review for management and surveillance of LAMN in line with national centres.

Intraductal tubulopapillary neoplasm (ITPN) is a distinctive type of pancreatic tumor first discovered more than three decades ago. ITPNs currently account for less than 1% of all pancreatic exocrine tumor cases recognized, and less than 5% of pancreatic intraductal tumors. A patient’s presentation is often nonspecific in comparison to other intraductal pancreatic neoplasms. We discuss a 52-year-old female presenting with abdominal pain and weight loss with ITPN, and go on to define the typical presentation, clinical features, and pathology behind the tumor.

Background and objectives Due to the rarity of appendiceal mucinous neoplasms (AMNs), there are few established treatment guidelines. The clinical course varies from incidental detection to progressive spread with pseudomyxoma peritonei (PMP). This study investigated the extent of resection on the prognosis and outcomes of AMNs. Methods This multicenter retrospective study evaluated patients with AMN who underwent surgery between 4/2006 to 9/2017. Primary endpoints included overall survival (OS) and disease-specific survival (DSS). Secondary endpoints included PMP incidence and treatment with cytoreductive surgery (CRS). Results Of the 138 patients with AMN, 70 patients (54%) underwent appendectomy, 26 (19%) cecectomy, and 37 (27%) right hemicolectomy. The median age was 59.7 years and 57 patients (41%) were male. Males were less likely to undergo cecectomy (P = .03). Rupture rates, tumor characteristics, and incidence of PMP were similar across surgery groups. Median follow-up was 61.3 months. Five-year OS and DSS for the total cohort were 94.9% and 98.6%, respectively, and remained similar across all surgery groups. CRS patients were more likely to undergo right hemicolectomy with no difference in survival by surgery type (P = .03). Conclusions Patients with AMN have a good overall prognosis and there may be minimal benefit to performing extended surgical resection in these patients.

Incidentally discovered pancreatic cysts have become more common with increasing use of abdominal cross-sectional imaging. Tools that help us to better risk stratify a pancreatic cyst include advanced imaging techniques, such as pancreatic protocol computed tomography (CT) scan or magnetic resonance imaging (MRI) with cholangiopancreatography. Endoscopic ultrasound (EUS) and fine-needle aspiration (FNA) are invasive measures to better define and sample cysts especially if high-risk features are present. EUS may also yield pancreatic cyst fluid for analysis of carcinoembryonic antigen (CEA) which is elevated in mucinous cysts. This case highlights a rare finding of a mucinous, epidermoid cyst in an intrapancreatic accessory spleen (IPAS) with high-risk features on EUS.

ObjectivePancreatic cancer can arise from precursor lesions called intraductal papillary mucinous neoplasms (IPMN), which are characterised by cysts containing papillae and mucus-producing cells. The high frequency of KRAS mutations in IPMN and histological analyses suggest that oncogenic KRAS drives IPMN development from pancreatic duct cells. However, induction of Kras mutation in ductal cells is not sufficient to generate IPMN, and formal proof of a ductal origin of IPMN is still missing. Here we explore whether combining oncogenic KrasG12D mutation with an additional gene mutation known to occur in human IPMN can induce IPMN from pancreatic duct cells.DesignWe created and phenotyped mouse models in which mutations in Kras and in the tumour suppressor gene liver kinase B1 (Lkb1/Stk11) are conditionally induced in pancreatic ducts using Cre-mediated gene recombination. We also tested the effect of β-catenin inhibition during formation of the lesions.ResultsActivating KrasG12D mutation and Lkb1 inactivation synergised to induce IPMN, mainly of gastric type and with malignant potential. The mouse lesions shared several features with human IPMN. Time course analysis suggested that IPMN developed from intraductal papillae and glandular neoplasms, which both derived from the epithelium lining large pancreatic ducts. β-catenin was required for the development of glandular neoplasms and subsequent development of the mucinous cells in IPMN. Instead, the lack of β-catenin did not impede formation of intraductal papillae and their progression to papillary lesions in IPMN.ConclusionOur work demonstrates that IPMN can result from synergy between KrasG12D mutation and inactivation of a tumour suppressor gene. The ductal epithelium can give rise to glandular neoplasms and papillary lesions, which probably both contribute to IPMN formation.

Background. We describe a case of a young male with a history of appendectomy one year ago, who developed symptoms of stump appendicitis, and after removing this stump, histopathology showed low grade neoplasm.Summary. Stump appendicitis is an uncommon complication after appendectomy and may lead to serious complications. Management of low-grade appendiceal mucinous neoplasm (LAMN) is controversial, and we discuss the importance of the case.Conclusion. The case of young male post stump appendectomy with histopathology showing LAMN in the stump of the appendix, which to our knowledge, is the first in the medical literature and, discuss the stump appendicitis and incomplete appendectomy concerning malignancy, mucinous neoplasm, and adenocarcinoma.

Cyst fluid biomarkers may be used to identify pancreatic cyst subtypes. Biomarkers are selected based on their ability to accurately distinguish mucinous from nonmucinous cysts and to risk stratify cysts based on malignant potential. Biomarkers of interest include but are not limited to amylase, oncogenes, DNA analysis, and epigenetic markers. The introduction of next-generation sequencing and molecular panels has aided in improved diagnostic accuracy and risk stratification. This review presents the diagnostic performance of currently available biomarkers and proposes an algorithm to incorporate their use in the diagnosis of pancreatic cysts.

Highlights • Morbidly obese patients do get cancer and require laparotomy for resection of the malignancy. • Sleeve gastrectomy can follow a successful oncologic intervention with minimal increase in morbidity and mortality. • Doing the oncologic procedure, HIPEC, and then sleeve gastrectomy helps prevent tumor seeding at the gastric staple line. • Patient satisfaction is high with a favorable prognosis expected from a simultaneous oncologic and bariatric intervention., Introduction Morbidly obese patients may require a laparotomy to resect a malignancy. In some patients the cancer resection can be combined with the bariatric procedure to concomitantly treat both diseases. Presentation of case A morbidly obese patient with peritoneal metastases from an appendiceal mucinous neoplasm was evaluated and definitively treated with Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC), at the same time the patient was treated for morbid obesity with sleeve gastrectomy and removal of a previous laparoscopic adjustable gastric banding (LAGB). Discussion The clinical features and treatments of a cancer patient who underwent a combined surgical oncologic and bariatric procedure is presented. A second-look cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) preceded a sleeve gastrectomy. At the time of surgical exploration the prognosis from an oncologic perspective was acceptable. The near total gastric resection was performed without complications. Conclusions With short term follow-up, this patient’s outcome was favorable suggesting that surgical oncologic and bariatric procedures can be combined. Further, clinical investigations are indicated in this common clinical setting.

Mucocele of the appendix is a rare entity in pediatrics, with few case reports in the literature. We present a case of a 17-year-old male with right lower quadrant pain who was found to have an appendiceal mucocele while being evaluated for appendicitis. Laparoscopic-assisted resection was performed, with pathology confirming a low-grade mucinous neoplasm of the appendix, a tumor scarcely described in the pediatric population. He underwent complete resection with negative margins, rendering him cured without the need for any further resection or treatment. We review the literature on this topic.

Endometriosis of the appendix can be an incidental finding or a cause of appendicitis, intussusception, perforation or retention mucocele. Intestinal metaplasia of appendiceal endometriosis may occur, which can lead to a misdiagnosis of low-grade appendiceal mucinous neoplasm. On a retrospective search of the pathology database from 2001 to 2015, we identified 78 appendiceal endometriosis cases and intestinal metaplasia was present in 10/78 (13%) cases. In most of the cases (90%), the foci of intestinal metaplasia were mainly localized close to the mucosa. Intestinal and endometrial hybrid glands were present in 9/10 (90%) cases. These cases were often associated with marked appendiceal distortion, luminal obliteration and mass formation, causing concern for a mucinous neoplasm clinically and pathologically. Our findings indicate that intestinal metaplasia in appendiceal endometriosis is not an uncommon phenomenon, which can be mistaken for a mucinous neoplasm. Endometriosis should be kept in mind when a diagnosis of appendiceal mucinous neoplasm is made, especially in a young woman with a clinical history of endometriosis.

Biliary mucinous cystic neoplasms are rare parenchymal neoplasms with a considerable malignant potential. Due to a lack of diagnostic imaging criteria, histopathologic evaluation remains the definitive method of diagnosis. Resection is the treatment of choice. Here, the authors present a case of biliary mucinous neoplasm in a 39-year-old female with the associated radiographic and histopathologic findings.

Objective: To determine the most common imaging features of pseudomyxoma peritonei (PMP), as well as the histologic subtypes of the primary tumors. Materials and Methods: We reviewed 30 cases of women with pathologically confirmed PMP. Only computed tomography scans were available. All cases were retrospectively studied by four radiologists, working independently. We identified the most common imaging findings, the predominant primary site of the disease, and the growth pattern. The most common sites of recurrence were also analyzed. Results: The most common computed tomography finding was peritoneal/omental nodules (including “omental caking”), followed by visceral scalloping and non-mucinous ascites. The most common site of the primary tumor was the appendix (in 63.3%), followed by the ovaries (in 16.6%), and 16.6% of the tumors were of undetermined origin. There was one case of synchronous appendiceal and ovarian tumors. Low-grade mucinous neoplasm was the most common histologic subtype, accounting for 84.2% of the appendiceal tumors and 40% of the ovarian tumors. Conclusion: Although PMP is a relatively rare entity, radiologists must be aware of its possible imaging findings, common locations, and possible patterns of recurrence. The origin of the primary tumor should also be investigated. Future studies are needed in order to determine which preoperative imaging findings predict surgical outcomes and to characterize the main findings of radiological recurrence. Resumo Objetivo: Determinar as características de imagem mais frequentes do pseudomixoma peritonial (PMP), bem como os subtipos histológicos dos tumores primários. Materiais e Métodos: Foram revisados 30 casos confirmados patologicamente de PMP em mulheres. Somente a tomografia computadorizada estava disponível. Todos os casos foram estudados de forma independente e retrospectiva por quatro radiologistas. Os autores relataram os achados de imagem mais frequentes, a localização predominante da doença primária e o padrão de crescimento. Os locais de recorrência mais comuns também foram analisados. Resultados: Os achados tomográficos mais frequentes foram nódulos peritoniais/omentais (incluindo “bolo omental”), seguidos por scalloping visceral e ascite não mucinosa. Os padrões de localização mais comuns do PMP também foram documentados. A grande maioria dos tumores primários foi de origem apendicular (63,3%), seguida de origem ovárica (16,6%) e indeterminada (16,6%). Houve um caso síncrono de tumor apendicular e ovário. A neoplasia mucinosa de baixo grau foi o subtipo histológico mais frequente, representando 84,2% dos tumores do apêndice e 40% dos tumores primários ovarianos. Conclusão: Embora o PMP continue sendo uma entidade relativamente rara, o radiologista deve estar ciente de seus possíveis achados de imagem, locais comuns e possíveis padrões de recorrência. A investigação do tumor primário também deve ser encorajada. Futuros estudos são necessários para prever o resultado cirúrgico da imagem pré-operatória e caracterizar os principais achados de recorrência radiológica.

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.

A 49-year-old woman presented with clinical signs, pre-operative imaging, and intra-operative findings suggestive of acute appendicitis. A laparoscopic appendectomy was performed. Final pathology revealed a low-grade appendiceal neoplasm with serrated architecture, and secondary acute inflammation of the appendix (5 cm in length x 0.7 cm in diameter) with a congested and hemorrhagic serosal surface. The main concern in the management of patients with low-grade appendiceal mucinous neoplasm (LAMN) is that these dysplastic tumors share such similar clinical, imaging, and intraoperative features with simple acute appendicitis, which is deemed definitively cured following surgical removal of the appendix. A definitive diagnosis of LAMN is often delayed until the final pathology report; however, this finding can have implications on the further management of the patient, especially with the risk of recurrence in situations of peritoneal dissemination or positive surgical margins. Reporting LAMN cases will increase awareness of this rare disease and will contribute to improved management in the future.

Appendiceal neoplasms account for 1% of appendectomy specimens. Common subtypes include mucinous cystadenoma, adenocarcinoma, and neuroendocrine tumors (NETs). The simultaneous presence of appendicular mucinous and NETs is a rare event. Depending on the tumors' morphological distribution in the affected organ, they are qualified as either "collision" or "combined" tumours. We herein present the case of a 50-year-old male who presented with acute appendicitis and who was subsequently found to have pseudomyxoma peritonei (PMP) due to a perforated combined mucinous and neuroendocrine tumours. The patient was treated by right hemicolectomy and cytoreductive surgery (CRS) with oxaliplatin-based hyperthermic intraperitoneal chemotherapy (HIPEC). He was cancer free 20 months later. Due to the limited clinical experience with this presentation, no formal recommendations exist as to its management other than those applicable to each cancer alone. The efficacity of treatment on the long-term prognosis on these combined tumors is yet to be elucidated.

Unexpected findings are not infrequently encountered, either intraoperatively or in the postoperative settings. This chapter will discuss when an appendectomy pathology report returns adenocarcinoma, carcinoid, or appendiceal mucinous neoplasm.

Introduction and importance Low-Grade Appendiceal Mucinous Neoplasms (LAMN) are noninvasive epithelial tumors of the appendix. Symptomatology is unspecific and can simulate appendicitis. The multidisciplinary approach for the correct diagnosis and management of this kind of tumor is important, as the prognosis depends on it. Presentation of the case A 70-years-old man with an appendiceal picture is presented. McBurney and Blumberg's signs were found and the Echography has reported an acute appendicitis. He underwent laparoscopic surgery, founding a Tumor in the Cecum of 10 × 5 cm. We decided to perform an appendectomy with a total cecectomy. The pathological diagnosis was a LAMN. Ten months later, a Computed Tomography (CT) Scan control was performed reporting no evidence of Tumoral activity or Distance Metastasis, neither pseudomyxoma peritonei (PMP) evidence. Discussion LAMN has been a very controversial tumor. The definitive management is appendectomy. There are controversies about the laparoscopic and open approach, and when there are positive margins. The stage was a pTis (LAMN) pNx according to the 8th edition of the American Joint Committee on Cancer (AJCC). The prognosis depends on the tumoral stage and the presence of PMP. In our case, we have performed a CT scan follow up and there was no Tumoral activity or Distance Metastasis, neither PMP evidence. Conclusion LAMN is a very interesting and rare tumor. The diagnosis of this case was a real challenge. Our management was simple due to the tumoral stage. Pathology played a very important role in treatment and prognosis., Highlights • The clinical presentation of LAMN is unspecific, it can simulate an appendicitis. • Surgical approach can be by laparoscopy depending on the tumoral staging. • The diagnosis is crucial to determine the management and prognosis. • The approach must be multidisciplinary and cooperative between medical departments.

Intraductal papillary mucinous neoplasms (IPMNs) are mucin-producing papillary neoplasms of the pancreatic or biliary ductal system that exhibit variable cellular atypia and cause ductal dilation. There are few reported cases of IPMN arising from the biliary tree in the literature. It has a higher propensity to undergo malignant transformation compared to IPMN arising from the pancreatic duct. An 80-year-old male underwent cross-sectional tomography (CT) imaging of the abdomen for evaluation of prostate adenocarcinoma, which revealed an incidental 2.3 × 2.7 cm soft tissue mass centered at the porta hepatis with diffuse dilatation of the left intrahepatic biliary ductal system and mild prominence of the right intrahepatic ductal system. Endoscopic ultrasound showed 2 adjacent hilar masses involving the common hepatic duct and the left hepatic duct with protrusion of the tissue into the lumen of the duct and upstream ductal dilatation. Endoscopic retrograde cholangiopancreatography revealed a large filling defect in the common hepatic duct extending into the left hepatic duct. A large amount of clot and soft tissue with a fish-egg appearance was retrieved. The patient underwent left hepatic lobectomy, radical resection of the common hepatic duct with Roux-en-Y hepaticojejunostomy to the right hepatic duct. Histopathological examination of the resected specimen revealed intraductal papillary mucinous neoplasm with diffuse high-grade dysplasia. Follow-up CT scan of the abdomen 2 months after the surgery was negative for any masses.

Multicystic mesotheliomas of peritoneal origin (also termed benign multicystic mesotheliomas) are uncommon and are exceptionally rare in the omentum. Lack of familiarity of this entity could lead to overinterpretation as a malignancy or present a challenge at frozen section. We describe a case of multicystic mesothelioma arising in the omentum found incidentally at laparotomy for endometriosis. Frozen section evaluation presented a challenge. Grossly, the mass in the omentum was multicystic and mucoid, raising the concern for a mucinous neoplasm. The cysts, however, were lined by flattened cells without atypia. At permanent section evaluation, calretinin positivity confirmed the mesothelial origin. Awareness of this entity is important, as it may be interpreted as a malignancy, particularly intraoperatively.

Neoplasms of the appendix are very rare. They usually show glandular or neuroendocrine differentiation, and when they both occur in the same area, it is called a “collision tumor.” Low-grade mucinous neoplasms associated with appendiceal diverticula are also uncommon. The appendectomy specimen of a 60-year-old man contained dense and mucoid luminal content on the distal tip, and similarly a solid, yellow, lumen-obscuring tumor with a diameter of 1.5 cm at the base of the appendix was detected. Microscopically, there were three diverticula that comprised herniation of the mucosal layer through the appendiceal wall. Interestingly, all of the diverticula and the normal-appearing appendiceal wall were lined by adenomatous epithelium. The luminal portion had pools of mucin-containing, rare clusters of low-grade epithelium that gave rise to the diagnosis of a “low-grade mucinous neoplasm.” The solid-appearing tumor was diagnosed as a “neuroendocrine neoplasm,” and there was no transition zone between these two types of tumors. There are some cases that have been reported as low-grade mucinous neoplasms associated with appendicular diverticula and collision tumors consisting both mucinous neoplasms and carcinoid tumors in the literature; our case has a unique appearance with two different types of tumors both in the appendix wall and within multiple diverticula.