Appendiceal Neoplasms Research Articles

Incidental finding of carcinoid tumor postappendectomy and a review from Gulf Cooperation Council countries

Abstract Scanty of research in Gulf Cooperation Council countries address the neuroendocrine tumors (NETs) with a gap in reported cases after appendectomy in Saudi Arabia compared to others. This study aimed to describe the experience of a single tertiary care private hospital in the management of incidentally discovered NETs after appendectomy and to review the series from nearby countries. A retrospective data review was done between January 2016 and January 2018 in the Surgery Department at Dr. Soliman Fakeeh Hospital (Jeddah, Saudi Arabia). Among 458 appendectomies, 3 appendiceal carcinoid tumors were identified (0.67%). All patients present with acute appendicitis. The carcinoid tumor was not diagnosed preoperatively. The mean diameter of the appendix was 10 mm, while the histopathology diameter of the discovered carcinoid was 5 mm. There were no postoperative complications. All patients were disease-free at follow-up. None of them received reoperation or adjuvant therapy.

Challenges and predictive radiological findings in the diagnosis of neuroendocrine tumors in patients with acute appendicitis.

Acute appendicitis is one of the most common surgical emergencies. With antibiotic-first treatment strategies gaining importance, the risk of an appendiceal tumor as an incidental finding or as the cause of appendicitis presents an obstacle to a conservative approach. Neuroendocrine tumors, the most frequent type of appendiceal tumors, are difficult to diagnose preopera-tively due to their small size. This study aims to identify predictive factors for neuroendocrine tumors in patients undergoing surgery for acute appendicitis by analyzing imaging and clinical characteristics, thereby enhancing preoperative diagnostic accuracy and guiding surgical interventions. This retrospective observational study included 1,298 patients who underwent appendectomy from January 2014 to May 2024. After excluding 59 patients with normal pathology results, 40 with variable pathologies, and 49 with inaccessible computed tomography (CT) images, 1,150 patients remained (1,135 with acute appendicitis and 15 with neuroendocrine tumors). Abdominal CT scans were evaluated for appendiceal diameter, wall thickness, cecal wall thickness, periappendiceal fat stranding, fluid collection, lymphadenopathy, intraluminal and free periappendiceal air, mucosal hyperenhancement, the presence of appendicolith, mural calcification, and mural nodules. The presence of a mural nodule protruding into the lumen was significantly higher in neuroendocrine tumor patients compared to those with acute appendicitis, with a sensitivity of 53.3%, specificity of 95.8%, positive predictive value (PPV) of 31.9%, negative predictive value (NPV) of 99.4%, and accuracy of 97.9%. Intraluminal air was also more frequent in neuroendocrine tumor patients, with a sensitivity of 53.3%, specificity of 76.7%, PPV of 2.9%, NPV of 99.2%, and accuracy of 76.4%. Other imaging parameters did not show significant differences between the two groups. This study identifies mural nodules and intraluminal air as significant predictors of neuroendocrine tumors in patients with acute appendicitis, emphasizing the importance of meticulous preoperative imaging evaluations. Incorporating these predictors into diagnostic protocols could improve the preoperative identification of neuroendocrine tumors, enabling more appropriate surgical interventions. Future research should validate these findings through prospective studies and explore advanced imaging techniques to further enhance the detection of appendiceal neoplasms, ultimately improving patient outcomes and reducing overlooked malignancies.

An unusual cecal submucosal mass in an elderly gentleman.

A 60-years-old man with previous hypertension was hospitalized because of colonoscopy revealed a cecal submucosal mass for community health examination 1 month ago. The patient has no symptoms such as poor appetite, fatigue, abdominal pain, diarrhea, nausea and vomiting. Laboratory tests and physical examination was unremarkable. A protrusion with multiple superficial small ulcers on the smooth surface measuring 1.5 × 2.0-cm was found beside the appendiceal orifice by colonoscopy. Abdominal computed tomography demonstrated a high-density mass without enhancement protruding towards the ileocecal cavity at the appendiceal orifice measured 1.5 × 1.8 cm. Laparoscopic ileocecal resection was performed because of appendiceal tumor couldn't be excluded and the patient's strong request. Pathology examination of the postoperative specimen revealed dilated appendix cavity with fecalith inside in the submucosal layer of the ileocecal region. The patient was diagnosed as appendiceal orifice submucosal fecalith. He was discharged home uneventfully and no symptoms was observed in 3 months follow-up.

Clinicopathologic features and diagnostic challenges of small cluster pattern appendiceal neuroendocrine tumors

Appendiceal neuroendocrine neoplasms (NENs) can present with various growth patterns including the traditional triad of histologic patterns-insular, trabecular and tubular. A small cluster pattern was also found in this study and the literature on this specific morphology is limited. In this study, we conducted a comprehensive review of appendiceal NENs from our institution over a ten-year period. Clinical and demographic data were obtained from medical records. Immunohistochemical stains were performed with antibodies specific for synaptophysin, chromogranin, INSM1, CD56, serotonin and peptide YY. The small cluster pattern was found in 29.4 % of all cases evaluated. The tumor cells in these cases were predominantly located at the distal tip of the appendix, associated with fibrous obliteration. These tumors were smaller in size and tended towards less advanced tumor stage, with reduced incidence of lymphovascular and/or perineural invasion. Chromogranin expression was identified in 76 % of these cases. There is a heterogeneous hormone profile with 46.7 % serotonin and 33.3 % peptide YY. In conclusion, the small cluster pattern NENs present with unique histological features and hormone expression profile. Among the various neuroendocrine markers, INSM1 showed superior diagnostic performance, with high sensitivity and minimal non-specific staining.

Prevalence of Neuroendocrine Tumours (NET) in Patients Undergoing Appendicectomy for Acute Appendicitis: A Tertiary Care Study.

Introduction Acute appendicitis is one of the common causes of acute abdomen in adults, which is managed widely with appendicectomy. Neuroendocrine tumours are the most common appendiceal tumours diagnosed incidentally on appendicectomy specimens. Methods Demographic data, presenting complaints, indications for appendicectomy, and the histology findings based on histopathological reports of the patients who have undergone appendicectomy for appendicitis at surgical units of Teaching Hospital, Jaffna, from 1st of January 2019 to 31st of December 2022 were retrospectively analyzed. Results Of the 1341 histopathology reports, 0.2% (n=3) were neuroendocrine tumours (NET). The mean age of the patients with NET was 48.6, and 66.6% of them were females. All three NETs identified in appendicectomy specimens were well differentiated and smaller than 2 cm. All three had negative resection margins and were managed only with appendicectomy. Conclusion NETs of the appendix are the commonest appendiceal neoplasms. The majority of them are diagnosed incidentally in appendicectomy specimens. Surgical management of the tumours is either by appendicectomy or hemicolectomy, which depends mainly on tumor size. Surgical decisions should be tailor-made to the patients based on multi-disciplinary team decisions.

Significance of Histopathology of Appendectomy Specimens: Analysis From a Teaching Hospital of Pakistan.

Background Histopathology of a tissue specimen plays a crucial role in formulating the final diagnosis of any disease. It confirms whether the histopathological findings are in correspondence with the clinical diagnosis and thus suggests an optimal management plan. Standard surgical practices guide that every human tissue specimen must undergo postoperative tissue analysis unless indicated otherwise. Objective To determine the significance of histopathology in determining the final diagnosis of appendectomy specimens. Materials and methods This retrospectiveclinical studyconducted in May 2022 included 100 patients operated for appendectomy from January 1, 2021, to December 31, 2021, in the emergency room of the Department of General Surgery, Unit-III, Lahore General Hospital, Lahore. Data were retrieved from patients' records and the picture archiving and communication system (PACS). A Google Forms-based pro forma(Google, Mountain View, CA) was generated to include the demographic details, clinical manifestations, and histopathology reports of the patients. Descriptive analysis was completed using a Microsoft Excel spreadsheet (Microsoft Corporation, Redmond, WA). Results Fifty-two patients were females out of the total 100. The mean age at presentation was 23.02 ± 12.02 years. Of the samples, 54% were not sent for histopathology. Among the remaining ones, 27% of cases were proven to be acute appendicitis. Alvarado score was 7-10 in 50% of patients. Other lesions proven by histopathology were appendiceal phlegmon (4%), perforated appendix (4%), mucocele (1%), carcinoid tumor (1%), tuberculosis (1%), and adenocarcinoma (1%). Conclusions Histopathological analysis is the gold standard for the tissue diagnosis of a disease. The high percentage of the samples not sent for histopathology is alarmingsince the appendix is not only a site for inflammatory pathologies but for neoplastic lesions as well. This practice depicts that the incidence of non-inflammatory pathologies is being ignored by healthcare professionals and there is a dire need to emphasize the significance of acquiring histopathology reports for the specimens of appendectomy in all circumstances.

Clinico-Pathological Characteristics and Long-Term Outcomes of Incidental Appendiceal Carcinoid Tumors in Children

Background: Appendiceal carcinoid tumors are uncommon in children and are usually found incidentally during histopathological examination after appendectomy. Objectives: The aim of this study was to investigate the clinicopathologic characteristics and long-term outcomes of appendiceal carcinoid tumors in children. Methods: Patients under 18 years old with a diagnosis of appendiceal carcinoid tumor were analyzed. Demographical, clinical, radiological, surgical, and pathological data and long-term outcomes were evaluated. Results: A total of 10 (0.64%) appendiceal carcinoid tumors were found in 1562 appendectomy specimens. The mean age of children with appendiceal carcinoid tumors was 12.6 years, and the male-to-female ratio was 2:3. Tumor localization was at the tip of the appendix in 9 (90%) patients. Seven (70%) tumors were smaller than 1 cm, while 3 (30%) tumors were between 1 and 2 cm. The depth of tumor penetration reached the subserosa in 8 (80%) patients and the mesoappendix in the remaining 2 (20%) cases. Surgical margins were intact in all patients, and no additional surgery was performed. The patients were followed up for an average of 42.4 months, and no recurrence was detected. Conclusions: Appendiceal carcinoid tumors usually have nonspecific clinical-radiological findings and are almost always detected incidentally during the histopathological analysis of appendectomy specimens resected due to acute appendicitis. According to histopathology results, close follow-up and advanced surgical and medical treatments should be considered when necessary. Although the survival of patients is good, the possibility of colorectal malignancies should not be overlooked during follow-up.

Appendiceal Mucinous Incidental Neoplasm a Rare Case Report and Literature Review

Mucinous appendiceal tumors consist of mucinous adenocarcinoma, low-grade appendiceal mucinous neoplasm, and high-grade appendiceal mucinous neoplasm. The incidence of non-mucinous adenocarcinomas is reduced. The most recent edition of the World Health Organization classification and recent consensus guidelines will enable the consistent application of agreed nomenclature. Not only is precise diagnosis essential for effective patient management, but it also facilitates the comparison of results across centers and tumor registries. The most prevalent benign adenoma in the appendix is serrated. It is imperative to differentiate these conditions from low-grade appendiceal mucinous neoplasms, as the latter can also resemble harmless ailments. Adenocarcinomas of the goblet cells are a rare subtype of appendiceal neoplasm. While appendiceal neoplasms are uncommon, they are not entirely so, and even the most seasoned pathologists may find them difficult to diagnose. In addition, appendiceal neoplasia classification and terminology have been subjects of contention for decades. Nonmucinous appendiceal neoplasms are less prevalent than mucinous tumors, and their association with other appendiceal neoplasm subtypes remains uncertain. A literature review of appendiceal mucinous neoplasms identified during laparoscopic appendicectomies is presented here.

Role of colectomy in the management of appendiceal tumors: a retrospective cohort study

BackgroundAppendiceal tumors represent a range of histologies that vary in behavior. Recommendations for treatment with appendectomy versus right hemicolectomy (RHC) for different tumor types are evolving and sometimes conflicting. This study sought to characterize variation in the United States around surgical treatment of major appendiceal tumor types over time and describe differences in outcomes based on procedure.MethodsPatients diagnosed with appendiceal goblet cell adenocarcinoma (GCA), mucinous adenocarcinoma, neuroendocrine neoplasm (NEN), or non-mucinous adenocarcinoma from 2004–2017 were identified in the National Cancer Database. Trends in RHC over time and predictors of RHC were identified. Surgical outcomes for each histologic type and stage were compared.ResultsOf 18,216 patients, 11% had GCAs, 34% mucinous adenocarcinoma, 31% NENs, and 24% non-mucinous adenocarcinoma. Rate of RHC for NEN decreased from 68% in 2004 to 40% in 2017 (p = 0.008) but remained constant around 60–75% for other tumor types. Higher stage was associated with increased odds of RHC for all tumor types. RHC was associated with higher rate of unplanned readmission (5% vs. 3%, p < 0.001) and longer postoperative hospital stay (median 5 days vs. 3 days, p < 0.001). On risk-adjusted analysis, RHC was significantly associated with increased survival versus appendectomy for stage 2 disease of all tumor types (HRs 0.43 to 0.63) and for stage 1 non-mucinous adenocarcinoma (HR = 0.56).ConclusionsMost patients with appendiceal tumors undergo RHC, which is associated with increased readmission, longer length of stay, and improved survival for stage 2 disease of all types. RHC should be offered selectively for appendiceal tumors.

Incidence trends and survival analysis of appendiceal tumors in the United States: Primarily changes in appendiceal neuroendocrine tumors.

Appendiceal tumors are considered to be a relatively rare tumor of the gastrointestinal tract and the prognosis is unclear. This study comprehensively investigated trends in the epidemiology and survival of appendiceal tumors in the United States over the past approximately 20 years. Patients with pathologically confirmed appendiceal tumors from 2000 to 2017 were selected from the Surveillance, Epidemiology and End Results (SEER) database. Age-adjusted incidence rates were calculated by SEER*Stat 8.4.0. The Kaplan-Meier method was used to analyze survival and prognostic factors were investigated by a multivariate Cox proportional risk model. Ultimately, 13,546 patients with appendiceal tumors between 2000 and 2017 were included. The annual incidence of colonic adenocarcinoma and mucinous adenocarcinoma remained relatively stable. Interestingly, the annual incidence of appendiceal neuroendocrine tumors (aNETs) increased significantly, from 0.03 to 0.90 per 100,000 person-years, with the most dramatic increase in the number of patients with localized disease. Patients with aNETs showed a significant improvement in survival between 2009-2017, compared to the period 2000-2008. Moreover, this improvement in survival over time was seen at all stages (localized, regional, distant) of aNETs. However, this improved survival over time was not seen in colonic and mucinous adenocarcinoma. The incidence of appendiceal neoplasms has increased significantly over the past nearly two decades, which is mainly due to the increased incidence and significant migration to earlier stages in aNETs. We must note that despite the increased incidence of aNETs, survival rates have improved at different disease stages.

Retrospective Clinico-pathological and Morphological Analysis of Appendicular Tumors on Appendectomy Specimens for Acute Appendicitis

Background and Study Aims: Appendiceal Tumors are rare. Difficult to diagnose, these tumors are often accidentally discovered following acute appendicitis. The aim of this study is to describe the clinical, pathological and morphologic characteristics of these tumors in order to define an appropriate management. Patients and Methods: From a pathological database, all appendiceal tumors found on appendectomy specimens performed between January 2017 and July 2019 were retrospectively analysed. Results: Among the 1480 appendectomies performed, 15 cases of appendiceal tumors were collected (1.01%). The mean age was estimated at 44.7 ± 21.5 years (17-80). Pain in the right iliac fossa was present in all patients. Abdominal ultrasonography coupled with computed tomography (CT) scan led to the diagnosis of appendiceal tumors in 40% of cases (6/15). All patients underwent emergency surgery. An appendectomy was performed in 13 patients (86%). A mucinous tumor was found in 9 patients (60%). In 40% of the cases (6/15), it was a neuroendocrine tumor. Conclusion: Appendiceal tumors are often discovered incidentally on appendectomy specimens for acute appendicitis. They can be seen at any age and their diagnosis is not easy, even with the support of imaging. Ideally, the diagnosis of these tumors should be made preoperatively; this can change the surgical approach and avoid the need for additional surgery. All appendectomy specimens should be examined in histopathology.

Incidence rate and histology of appendiceal neoplasms in complicated versus uncomplicated appendicitis: A meta-analysis and systematic review

IntroductionStudies evaluating the rate and histology of appendiceal neoplasms between complicated and uncomplicated appendicitis include a small number of patients. Therefore, we sought a meta-analysis and systematic review comparing the rates and types of appendiceal neoplasm between complicated and uncomplicated appendicitis.MethodsWe included articles published from the time of inception of the datasets to September 30, 2022. The electronic databases included English publications in Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, and Scopus.ResultsA total of 4962 patients with appendicitis enrolled in 4 comparative studies were included. The mean age was 43.55 years (16- 94), and half were male (51%). Based on intra-operative findings, 1394 (38%) had complicated appendicitis, and 3558 (62%) had uncomplicated appendicitis. The overall incidence rate of neoplasm was 1.98%. No significant difference was found in the incidence rate of appendiceal neoplasm between complicated (3.29%) and uncomplicated (1.49%) appendicitis (OR 0.44, 95% CI 0.16- 1.23; p < 0.087; I2 = 54.9%). The most common appendiceal neoplasms were Neuroendocrine Tumors (NET) (49.21%), Nonmucinous Adenocarcinoma (24.24%), Mixed Adeno-Neuroendocrine Tumor (MANEC) (11.40%), Mucinous Adenocarcinoma (4.44%). There was a significant difference between complicated and uncomplicated appendicitis in rates of adenocarcinoma (50% vs. 13%), NET (31% vs. 74%), MANEC (19% vs. 13%) (P < 0.001).ConclusionWhile there was no significant difference in the overall neoplasm rate between complicated and uncomplicated appendicitis, the NET rate was significantly higher in uncomplicated appendicitis. In comparison, the Adenocarcinoma rate was considerably higher in Complicated appendicitis. These findings emphasize the importance of evaluating risk factors for neoplasm when considering appendectomy in patients with appendicitis.

Appendiceal carcinoid tumor presenting as perforated appendicitis in pediatric patients: A case series and review of the literature

Appendiceal carcinoid tumor presenting as perforated appendicitis in pediatric patients: A case series and review of the literature

Duplication of the appendix masquerading as appendiceal tumor: a case report

BackgroundThis case report highlights the exceptional rarity of appendix duplication in adults, a condition that closely mimics appendiceal tumors, posing diagnostic challenges. The novelty of this case lies in its presentation of a Type A duplication, emphasizing the diagnostic intricacies involved in distinguishing it from other pathologies.Case presentationWe present the case of a 69-year-old male with a history of hypertension, hyperuricemia, and duodenal gastric ulcer, who presented with a positive occult blood test. Lower gastrointestinal endoscopy revealed an appendiceal orifice with atypical hyperemia and edema. Subsequent imaging and biopsy results suggested an appendiceal tumor, prompting laparoscopic ileocecal resection. Intraoperative findings revealed an unremarkable appendix, but histopathological analysis unveiled appendiceal duplication, characterized by bifurcation into two lumens within a thick serosal wall. The patient was discharged without complications.ConclusionsThis case underscores the importance of recognizing appendix duplication as a rare differential diagnosis for appendiceal tumors. Surgeons should remain vigilant, especially in cases of Type A duplication, where preoperative diagnosis remains challenging. Early identification can avert potential complications and missed congenital anomalies.

Neuroendocrine Tumor of The Appendix: Retrospective 7266 analysis of Appendectomy Patients in a single center

Objective: We aim to identify appendiceal neuroendocrine tumors (NETs) seen after acute appendicitis and investigate their incidence in patients with laparoscopic or open appendectomy surgery. Here we have shaped the characteristic features of the tumor according to the 2010 classification rating of The World Health Organization (WHO) (surgical margin, invasion, grading, TNM stages) and also explored the disease-free survival in these patients. Material and Methods: This is the medical record of the patient with 7266 appendectomy retrospectively evaluated, and with these examples in histopathological analysis, a total of 43 patients were diagnosed with appendicitis NETs and included in the study. Age, gender, prediction of the surgical team during the operation, location of the tumor, WHO grade, depth of invasion, positive surgical margin, tumor size, TNM stage, and postoperative survival of these patients were analyzed retrospectively. Results: In this study, the incidence of appendiceal (NETs) was found to be 0.59% among 7,266 patients. Out of the 42 patients with appendiceal NETs, 27 were female and 16 were male. The mean age of the patients was 33.65 years (range: 11–78). The diagnosis was aided by clinical examination, ultrasound, and abdominal computerized tomography (CT) imaging. Most patients were initially diagnosed with appendicitis, and surgical interventions, including laparoscopic and conventional appendectomy, were performed. The pathological analysis confirmed appendicitis with NETs, classified according to the WHO 2010 recommendation. The majority of patients had well-differentiated NETs (G1 or G2). Surgical resection achieved negative radial margins, and no pathological signs were detected during follow-up colonoscopies. At a median follow-up of 70.2 months, all patients remained disease-free and alive. Conclusion: Appendiceal neuroendocrine tumors are rare but distinct entities that require a multidisciplinary approach for diagnosis and management. Surgical resection is the primary treatment, with systemic therapies reserved for advanced cases. The prognosis is generally favorable, with better outcomes seen in well-differentiated tumors. Long-term follow-up is crucial. Further research is needed to improve understanding and optimize treatment strategies for these tumors.

A Case of Double Primary Carcinoma was Accidentally Found During Operation: Hepatocellular Carcinoma and Appendiceal Mucinous Adenocarcinomas

Background: Synchronous double primary cancer of hepatocellular carcinoma (HCC) and appendiceal mucinous adenocarcinomas has not been reported. Liver metastasis of digestive tract tumors is so common that it is easy to ignore the existence of double primary cancer in the digestive system. The appendiceal malignant tumor itself is easy to be misdiagnosed or missed. For a better understanding of HCC and appendiceal mucinous adenocarcinomas, the information on our recent case has been summarized. Case Presentation: A 58-year-old man was admitted with repeated upper abdominal pain for one week. Imaging examination and liver biopsy pathology were diagnosed as hepatocellular carcinoma. Colonoscopy showed no obvious organic lesions in the terminal ileum and the whole large intestine. We had anatomical resection of the S5 and S6 segments of the liver. At the same time, two suspicious lesions in the right liver were ablated by microwave. But at the end of the operation, we accidentally found a hard mass about 3 cm × 3 cm in size at the end of the appendix. And the rapid pathological results showed appendiceal adenocarcinoma, so we had a right hemicolectomy. So far, the diagnosis of the patient was changed to synchronous double primary cancer consisting of HCC and appendiceal mucinous adenocarcinomas. Conclusion: It is the first time we have reported a case of synchronous double primary hepatic cancer consisting of HCC and appendiceal mucinous adenocarcinomas in the world. Due to the rarity and unspecific clinical features, it is extremely challenging to be diagnosed preoperatively. It requires a more comprehensive understanding of the patient's medical history, careful physical examination, and comprehensive exploration of the abdominal cavity during the operation to avoid missed diagnosis. Patients with HCC and appendiceal mucinous adenocarcinomas are generally associated with poor prognosis. It needs we put forward new countermeasures for the new question.

Incidence of occult appendiceal neoplasm in patients over 40 years with acute appendicitis: A single-institution review.

To investigate the incidence of occult appendiceal neoplasm in patients aged 40 years and over who underwent appendicectomy for appendicitis. The clinical coding electronic database was used to identify patients aged 40 years and over who were diagnosed with appendicitis from September 2010 to September 2022. Patients were included if they were managed operatively. Retrospective cohort study. Modified Monash category 3 (large rural town). Patients aged 40 years and over undergoing appendicectomy for appendicitis. Incidence of appendiceal neoplasm within appendicectomy specimen. A total of 279 patients aged 40 years and over underwent appendicectomy, with a median age of 53 years (range 40-95). Nineteen patients (7%) were found to have a primary neoplastic lesion within the appendix: seven neuroendocrine neoplasms (37%), six sessile serrated lesions (32%), two colonic-type adenocarcinoma (11%), two goblet cell adenocarcinoma (11%) and two appendiceal mucinous neoplasms (11%). Additionally, one patient had a metastatic adenocarcinoma of pancreaticobiliary aetiology. Occult appendiceal neoplasm was higher than reported in the literature in our cohort. This would support appendicectomy as the treatment of choice for patients aged 40 years and over with acute appendicitis and caution against nonoperative management in this demographic.

Mucocele of the Appendix Discovered Golestan Hospital of Ahwaz: A Case Report

Introduction: One rare cause of appendicitis is mucocele, which may have a neoplastic or non-neoplastic source. Simple appendectomy or right hemicolectomy may be necessary; sometimes, medication may be prescribed to manage symptoms. In this study, we reported a rare case with mucocele of the appendix managed by antibiotic therapy and a simple appendectomy. Case Presentation: A 60-year-old female with a seven-month history of right lower quadrant pain. A physical exam showed tenderness and rebound tenderness in the McBurney’s point. Her CT-scan tubular mass, measured 105 * 37 mm in RLQ, is suggested for mucocele appendicitis. Appendectomy was performed through a McBurney incision, and the pathology was a mucocele of the appendix with mucin-secreting mucosal hyperplasia. Conclusions: Malignancy of the appendix is extremely rare. Appendiceal tumors have a low prevalence, but due to the nature of these tumors, which can also be malignant, they should be given importance, and careful follow-up is mandatory. Patients must remain informed and proactive as the surgical community continues to learn more about this condition. The mucocele appendix can be successfully managed and treated with timely diagnosis and treatment.

Incidence, Clinicopathological Features and Oncologic Outcome of Appendiceal Neoplasms: A Single-Center Cohort Study

Appendiceal tumors represent a large amalgam of different tumor lineages. The continuous evolution in their pathological classifications has led to some variable recommended attitudes over time. The aim of this study is to review the incidence, clinicopathological characteristics, therapeutic approach and oncological results in this type of tumor at our institution. This is a single-centre retrospective cohort study. Every pathologic report catalogued as an appendiceal specimen was reviewed for a time period of 5 years (2013–2017) at our institution. Demographic, clinical, pathological and oncologic follow-up data were recorded. A descriptive study of the sample was completed. A total of 1434 appendiceal specimens was analyzed. Appendiceal neoplasms incidence was 3.2%. Epithelial tumors were the predominant histological subtype, making up 68% of the cases. Low-grade appendiceal mucinous neoplasia and neuroendocrine tumors were the most frequent neoplasms with malignant potential, with 13 and 6 cases, respectively. In more than 80% of neoplasia cases, the definitive treatment was appendectomy. Mortality cases were related to tumors with a very poor prognosis and an advanced stage. All patients had adequate oncological follow-up. Although it is still quite rare, the incidence of appendiceal tumors is increasing with an epidemiological change in favor of mucinous neoplasms currently predominating. Therefore, it is necessary to know and use an updated anatomo-pathological classification in order to provide correct treatment in the first or second surgical stage, as well as the correct follow-up of patients.

Low-Grade Appendiceal Mucinous Neoplasm: What Is the Best Treatment?

Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon tumor of the appendix that is usually diagnosed incidentally after surgery. Although LAMN may be asymptomatic, it can rupture and seed mucin and neoplastic epithelium into the peritoneum, causing pseudomyxoma peritonei (PMP). Data from 53 patients were retrospectively analyzed. Age, sex, tumor size, margin status, peritoneal carcinomatosis index, surgical procedures, postoperative results with histologic diagnosis, T stage, recurrence, and mortality of the patients were evaluated. Appendectomy was performed in 37 patients, right hemicolectomy in nine patients, cytoreductive surgery in one patient, and cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in six patients. Recurrence occurred in four patients. Of the patients who developed recurrence, one patient had stage T4a disease, and the other three patients had T4b disease (p<0.001). Eighteen patients had acellular mucin in the serosa and four of these patients developed recurrence (p=0.004). Twelve patients had appendix perforation, and three of these patients had PMP on exploration (p<0.001). The mean survival time was 93.3 months in patients without recurrence and 32 months in patients with recurrence (p=0.021). Low-grade appendiceal mucinous neoplasms are rare appendiceal tumors. The appropriate management of this tumor is controversial. T stage, appendix perforation, presence of acellular mucin on the serosa, and surgical margins are risk factors for the development of PMP. Appendectomy is considered sufficient when there are no risk factors for Tis (LAMN) and T3 disease. Right hemicolectomy may be sufficient if there are no risk factors for T4a disease, but cytoreductive surgery and hyperthermic intraperitoneal chemotherapy seem to be the most appropriate treatments in the presence of the stated risk factors for T4b disease.