Search Results (6)

Background: Low-grade appendiceal mucinous neoplasms (LAMNs) are rare lesions of the vermiform appendix and characterized by mucinous epithelial proliferation, extracellular mucin, and the absence of destructive invasion. Appendiceal diverticulum (AD) is also an uncommon condition that may be challenging to differentiate from acute appendicitis when it is superimposed by diverticulitis or perforation. Some recently published studies emphasized that complicated AD with mucosal hyperplasia can be confused with LAMNs, leading to overdiagnosis. The present study aimed to determine the histopathological features which can be used in the differential diagnosis of LAMNs and ADs, particularly complicated diverticula, in a large cohort. Methods: Cases comprising LAMNs and ADs diagnosed between 2011 and 2021 were included in the study. All cases were evaluated for the epithelial lining, the wall of the lesions, and the presence of cellular or acellular mucin, with its localization in terms of level and site of involvement within the appendix also recorded. Results: The hypermucinous epithelium characteristic of LAMNs, fibrosis, and calcification in the wall and the absence of lamina propria and muscularis mucosa proved to be the most discriminatory features in the differential diagnosis of LAMNs and ADs. Conclusions: The distinction between mucinous neoplasia and its mimics is critically important, since mucinous neoplasia requires surveillance imaging and potential surgery or chemotherapy depending on the extent of the disease, whereas non-neoplastic lesions are treated by an appendectomy and require no future intervention or surveillance. Full article

Appendiceal mucocele is a rare entity first described by Carl von Rokitansky, characterized by cystic dilatation of the appendiceal lumen due to obstruction, epithelial proliferation or inflammation and accumulation of mucoid material. The cause can be either neoplastic or non-neoplastic. Patients with appendiceal mucocele can be asymptomatic or present with right lower quadrant pain which may mimic acute appendicitis. We present the case of a 68-year-old male, who presented to the Emergency Room with a two-day history of right lower quadrant pain, nausea, vomiting and loss of appetite. Abdominal examination revealed tenderness over the Mc Burney point with localized guarding and laboratory results showed leukocytosis with neutrophilia. The abdominal computed tomography revealed a cystic dilated appendix, with a length of 130 mm and a diameter of 75 mm, situated ascending retrocecal and associating peri-appendicular inflammatory changes. The patient underwent right hemicolectomy with side-to-side ileo-colonic anastomosis, due to a wide intraluminal communication between the appendix and the cecum and the inflammation of both structures. Histopathological examination showed both high-grade and low-grade appendiceal mucinous neoplasm. Full article

We report a rare case of low-grade appendiceal mucinous neoplasm (LAMN) causing ileocolic intussusception. The case underscores the importance of considering ileocolic intussusception in differential diagnoses for nonspecific gastrointestinal symptoms. Early diagnosis via contrast-enhanced CT and scrupulous surgical intervention are crucial for favorable outcomes. Full article

There is no clear evidence on the prevalence and clinical presentation of appendiceal mucinous neoplasm (AMN) among patients with inflammatory bowel disease (IBD), so a systematic review was performed to investigate the diagnosis, management and treatment of AMN in these patients. PubMed, Medline, Scopus and the Cochrane Library were searched for articles published up to September 2023. Twenty-three studies reporting data about 34 AMN patients were included. UC patients had a median age of 52 years and a median length of disease of 10 years; CD patients had a median age of 40.5 years and a median length of disease of 5 years. A pre-operative diagnosis was achieved in 44% of patients. Most patients were symptomatic (82.6%) and showed moderate–severe disease activity (61%). Surgical procedures were performed: laparoscopic appendectomy, ileocecal resection, right hemicolectomy and colectomy/proctocolectomy. Of the patients, 73.5% were diagnosed with low-grade mucinous neoplasm (LAMN) and nine with adenocarcinoma. Synchronous colorectal dysplasia/carcinoma was present in 23.5% of patients. IBD patients with long-standing disease should be routinely screened, not only for colorectal cancer but also for AMN, during gastro-enterologic follow-up. Laparoscopic appendectomy of unruptured LAMN as well as right hemicolectomy of non-metastatic adenocarcinoma are safe procedures in IBD patients. Full article

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. Full article

Background: Pseudomyxoma peritonei (PMP) is a rare peritoneal condition where mucus-secreting tumorous cells progressively produce a thick, gelatin-like substance. The prognosis of patients with PMP is determined by the degree of cellularity within the mucin (low-grade (LAMN) vs. high-grade (HAMN) histologic features) and by the extent of the disease. Methods: Prognostic relevance of tumor markers CA19-9 and CEA, gender, Peritoneal Cancer Index (PCI), and completeness of cytoreduction (CC) after cytoreductive surgery were evaluated on 193 consecutive PMP patients, based on a retrospective analysis of prospectively gathered data from a German tertial referral center. Results: We demonstrated that low PCI, CC0 status, low-grade histology, and female gender were independent positive prognostic factors for both overall survival (OS) and progression-free survival (PFS). Furthermore, LAMN patients with achieved CC0 status show significantly better OS and PFS compared to those with CC1 status (p = 0.0353 and p = 0.0026 respectively). In contrast, the duration and drug of hyperthermic intraperitoneal chemotherapy (HIPEC) were not prognostic in any comparison. Increased CA19-9 and CEA levels were significantly associated with HAMN cases, but also predicted recurrence in patients with low-grade histologies. Conclusion: Our study confirmed the prognostic role of tumor markers and emphasized the importance of CC status and PCI in a large cohort of PMP- and LAMN patients. Full article