Mucocoele of the appendix.

Introduction: Mucocoele of the appendix occurs in 0.2-0.7% of people in the world without any well-defined clinical symptoms. It occurs when there is an accumulation of mucous in the lumen of the appendix. Case Presentation: We present three cases: a 48-year-old male admitted to the emergency room with a one-day history of right iliac fossa pain. Abdominal examination was suggestive of acute appendicitis. The initial abdominal computerised tomography scan was reported as being unremarkable. At surgery, a firm tumour of the appendix was found, and a limited right hemicolectomy was done. Histopathology confirmed a mucocoele of the appendix with borderline mucinous histology.The second case is a 63-year-old man who presented with a one-year history of abdominal distension and weight loss. Previous abdominal ultrasound was suggestive of liver cirrhosis with significant ascitic fluid. Abdominal magnetic resonance imaging found an appendix mucocoele with infiltration of the omentum and scalloping of the liver surface suggestive of pseudomyxoma peritonei. A percutaneous biopsy of the omental mass confirmed metastatic mucinous adenocarcinoma of the appendix.The third case is a 68-year-old man who, during an annual medical check-up, had an incidental finding of a cystic right iliac fossa mass on ultrasound, confirmed on abdominopelvic computerised tomography scan to be an appendix mucocele. He had laparoscopic appendicectomy. The histopathological diagnosis confirmed a mucinous cystadenoma of the appendix. Conclusion: Preoperative diagnosis of appendiceal mucocoele is difficult and commonly discovered intraoperatively. The prognosis is good for the histologically benign type, but it is poor when malignant or peritoneal lesions are present. Funding: None declared.

Low-grade appendiceal mucinous neoplasms: Histomorphological spectrum in a tertiary care hospital.

BACKGROUND: Low-grade appendiceal mucinous neoplasms (LAMNs) are benign non-invasive epithelial proliferations of the appendix. These usually present clinically as mucoceles and these rarely exceed 2 cm in diameter. Lesions confined to the lumen are labelled as LAMN; however those in which mucin spreads outside the peritoneum are labeled as pseudomyxoma peritonei (PMP). AIMS AND OBJECTIVE: A retrospective study was conducted over a period of three years and all cases of appendectomies were studied. Twelve cases of LAMN were identified, which is a diagnostic dilemma for the pathologists and clinicians. RESULTS: LAMN was identified based on the histopathological features. Out of the 12 cases, 9 were classified as LAMN and 3 as appendiceal neoplasm with PMP. There was villous or flat proliferation of epithelial lining, loss lymphoid aggregates, and dissecting mucin within muscularis. CONCLUSION: LAMNs are rare neoplasms of the appendix, with clinical presentation similar to acute appendicitis. Mucinous collections within the appendiceal wall should be extensively searched for mucosal changes and, if found, should prompt a careful search for pushing invasion of LAMNs. A thorough and vigilant gross examination can be of great help. Appendicectomy is the treatment of benign and grossly intact mucinous neoplasm.

Schistosomal appendicitis: a case report.

BACKGROUND: Schistosomiasis is one of the endemic parasitic diseases in many developing countries. Despite this, appendicitis secondary to schistosomiasis is an uncommon condition even in some endemic areas. Schistosomal appendicitis, an incidentally discovered appendicitis associated with schistosomiasis histological findings, affects young males predominantly. Timely diagnosis and treatment, including appendectomy and anti-helminthic therapy, are crucial. CASE REPORT: A 24-year-old Sudanese male patient presented with abdominal pain. Diagnosed with acute appendicitis, he underwent appendectomy, revealing appendix inflammation with Schistosoma ova in histopathology. Abdominal ultrasound detected no complications. Weakly positive Schistosoma serology was noted, but stool and urine analysis showed no infection evidence. Prescribed praziquantel, patient had 3-year post-op follow-up without complications. CONCLUSIONS: This case report underscores the significance of including schistosomiasis in the differential diagnosis of appendicitis, particularly in regions where the disease is endemic. It underscores the necessity of histopathological evaluations for accurate diagnosis, emphasizing the potential implications for clinical practice in similar settings.

MAIT cell activation and recruitment in inflammation and tissue damage in acute appendicitis.

Mucosal-associated invariant T (MAIT) cells are antimicrobial T cells abundant in the gut, but mechanisms for their migration into tissues during inflammation are poorly understood. Here, we used acute pediatric appendicitis (APA), a model of acute intestinal inflammation, to examine these migration mechanisms. MAIT cells were lower in numbers in circulation of patients with APA but were enriched in the inflamed appendix with increased production of proinflammatory cytokines. Using the patient-derived appendix organoid (PDAO) model, we found that circulating MAIT cells treated with inflammatory cytokines elevated in APA up-regulated chemokine receptors, including CCR1, CCR3, and CCR4. They exhibited enhanced infiltration of Escherichia coli-pulsed PDAO in a CCR1-, CCR2-, and CCR4-dependent manner. Close interactions of MAIT cells with infected organoids led to the PDAO structural destruction and death. These findings reveal a previously unidentified mechanism of MAIT cell tissue homing, their participation in tissue damage in APA, and their intricate relationship with mucosal tissues during acute intestinal inflammation in humans.

Immunoglobulin G4-related disease mimicking an appendiceal neoplasm: a case report.

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by chronic activation of the immune system and a tendency to form tumorous lesions. IgG4-RD is frequently characterized by the presence of tumor-like masses affecting multiple organs and is easily mistaken for a malignant neoplasm. However, IgG4-RD affecting the appendix is extremely rare, with only seven cases reported previously. We report the case of a woman in her early 60s who presented with insidious abdominal pain and radiological findings mimicking appendiceal neoplasms. After diagnosing appendiceal neoplasms, surgery was performed. The patient had a serum IgG4 concentration of <1.35 g/L, which did not satisfy one of the three revised comprehensive diagnostic criteria for IgG4-RD. A pathological examination was conducted, and the patient was diagnosed with appendiceal IgG4-RD. To the best of our knowledge, there have been no previously reported cases of IgG4-RD affecting the appendix in patients with low serum IgG4 concentrations. This report may prove beneficial for the future understanding of IgG4-RD and for the revision of diagnostic and treatment strategies.

Pathologic Analysis of Twenty-one Appendices From Children With Multisystem Inflammatory Syndrome Compared to Specimens of Acute Appendicitis: A Cross-sectional Study.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a rare, severe complication of coronavirus disease 2019, commonly involving the gastrointestinal tract. Some children with MIS-C undergo appendectomy before the final diagnosis. There are several hypotheses explaining the pathomechanism of MIS-C, including the central role of the viral antigen persistence in the gut, associated with lymphocyte exhaustion. We aimed to examine appendectomy specimens from MIS-C patients and assess their pathologic features, as well as the presence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antigens. METHODS: In this cross-sectional study we included 21 children with MIS-C who underwent appendectomy. The control group included 21 sex- and age-matched children with acute appendicitis (AA) unrelated to SARS-CoV-2 infection. Histologic evaluation of appendiceal specimens included hematoxylin and eosin staining and immunohistochemical identification of lymphocyte subpopulations, programmed cell death protein-1 (PD-1) and SARS-CoV-2 nucleocapsid antigen. RESULTS: Appendices of MIS-C patients lacked neutrophilic infiltrate of muscularis propria typical for AA (14% vs. 95%, P < 0.001). The proportion of CD20+ to CD5+ cells was higher in patients with MIS-C (P = 0.04), as was the proportion of CD4+ to CD8+ (P < 0.001). We found no proof of SARS-CoV-2 antigen presence, nor lymphocyte exhaustion, in the appendices of MIS-C patients. CONCLUSIONS: The appendiceal muscularis of patients with MIS-C lack edema and neutrophilic infiltration typical for AA. SARS-CoV-2 antigens and PD-1 are absent in the appendices of children with MIS-C. These findings argue against the central role of SARS-CoV-2 persistence in the gut and lymphocyte exhaustion as the major triggers of MIS-C.

Pathological Assessment of the Appendix in Appendectomies Performed in Children.

BACKGROUND: Acute appendicitis is known as the most common diagnosis of acute abdomen leading to surgery. Therefore, timely diagnosis is of special importance. This study was conducted with the aim of pathological assessment of the appendix in appendectomies performed in children to determine the rate of negative appendectomies and the predictors of negative appendectomy and to evaluate the paraclinical tools used in the diagnosis of acute appendicitis. METHODS: This is a cross-sectional descriptive study. All children who underwent appendectomy at Shahid Motahari Hospital in Urmia from March 2021 to March 2022 were examined. The required data including demographic, paraclinical, and final pathology characteristics were collected and recorded. The investigated cases were classified into positive and negative appendectomy categories for comparison. RESULTS: Among 234 pathology samples of the appendix, 22 cases were related to accidental appendectomy. In addition, 11.3% of cases were negative appendectomy and 88.7% were positive appendectomy. The age range of 8 to 14 years and male gender were associated with a lower negative appendectomy rate (both P<0.001). Inflammatory (49.5%) and gangrenous appendicitis (30.2%) were the most commonly reported histopathologies. Sonography had a sensitivity of 84%, a specificity of 79%, and an overall diagnostic accuracy of 83%. CONCLUSION: A relatively significant number of accidental and negative appendectomies are performed. More careful investigation and the use of expectant and medical treatment instead of surgery, especially in females and young children, can be effective in improving diagnostic accuracy and preventing negative appendectomies.

Nodular image in the appendix observed on ultrasound: endometriosis or neuroendocrine neoplasia?

Objective: To evaluate the association between clinical and imaging with surgical and pathological findings in patients with suspected neuroendocrine tumor of appendix and/or appendix endometriosis. Methods: Retrospective descriptive study conducted at the Teaching and Research Institute of Hospital Israelita Albert Einstein, in which medical records and databases of patients with suspected neuroendocrine tumor of appendix and/or endometriosis of appendix were analyzed by imaging. Results: Twenty-eight patients were included, all of which had some type of appendix alteration on the ultrasound examination. The pathological outcome of the appendix found 25 (89.3%) lesions compatible with endometriosis and three (10.7%) neuroendocrine tumors. The clinical findings of imaging and surgery were compared with the result of pathological anatomy by means of relative frequency. Conclusion: It was possible to observe a higher prevalence of appendix endometriosis when the patient presented more intense pain symptoms. The image observed on ultrasound obtained a high positive predictive value for appendicular endometriosis.

Occult appendix pathology in patients undergoing colorectal cancer resection and prophylactic appendectomy.

BACKGROUND: The risk-benefit balance of prophylactic appendectomy in patients undergoing left colorectal cancer resection is unclear. The aim of this report is to assess the proportion of histologically abnormal appendices in patients undergoing colorectal cancer resection in a unit where standard of care is appendectomy, with consent, when left-sided resection is performed. METHODS: A retrospective study on a prospectively collected database was conducted in a single tertiary-care center. Overall, 717 consecutive patients undergoing colorectal cancer resection between January 2015 and June 2021 were analyzed. The primary outcome was the proportion of histologically abnormal appendix specimens at prophylactic appendectomy. The secondary outcome was complications from prophylactic appendectomy. RESULTS: Overall, 576/717 (80%) patients had appendectomy at colorectal cancer surgery. In total, 234/576 (41%) had a right-/extended-right hemicolectomy or subtotal colectomy which incorporates appendectomy, and 342/576 (59%) had left-sided resection (left-hemicolectomy, anterior resection or abdominoperineal excision) with prophylactic appendectomy. At definitive histology, 534/576 (92.7%) had a normal appendix. The remaining 42/576 (7.3%) showed abnormal findings, including: 14/576 (2.4%) inflammatory appendix pathology, 2/576 (0.3%) endometriosis, 8/576 (1.4%) hyperplastic polyp, and 18/576 (3.1%) appendix tumors, which encompassed six low-grade appendiceal mucinous neoplasms (LAMNs), three carcinoids, and nine serrated polyps. In the 342 patients who had prophylactic appendectomy, 10 (2.9%) had a neoplasm (two LAMN, three carcinoids, and five serrated polyps). There were no complications attributable to appendectomy. CONCLUSION: Occult appendix pathology in patients undergoing colorectal cancer resection is uncommon when prophylactic appendectomy was performed. However, approximately 3% of patients had a synchronous appendix neoplasm.

The microbiome of the appendix differs in patients with and without appendicitis: A prospective cohort study.

BACKGROUND: Appendicitis seems to be a disease of infectious origin, but the detailed pathogenesis is unknown. We aimed to investigate the microbiome of the appendix lumen in patients with and without appendicitis, including a comparison of the subgroups of complicated versus uncomplicated appendicitis. METHODS: This prospective observational cohort study included adult patients undergoing laparoscopic appendectomy for suspected appendicitis. According to histopathologic findings, the investigated groups consisted of patients with and without appendicitis, including subgroups of complicated versus uncomplicated appendicitis based on the surgical report. A swab of the appendix lumen was analyzed for genetic material from bacteria with shotgun metagenomics, and outcomes included analyses of microbiome diversity and differential abundance of bacteria. RESULTS: A total of 53 swabs from patients with suspected appendicitis were analyzed: 42 with appendicitis (16 complicated) and 11 without appendicitis. When comparing patients with and without appendicitis, they were equally rich in bacteria (alpha diversity), but the microbiome composition was dissimilar between these groups (beta diversity) (P < .01). No consistent bacterial species were detected in all patients with appendicitis, but a least 3 genera (Blautia, Faecalibacterium, and Fusicatenibacter) and 2 species, Blautia faecis and Blautia wexlerae, were more abundant in patients without appendicitis. For the subgroups complicated versus uncomplicated appendicitis, both measures for microbiome diversity were similar. CONCLUSION: The appendix microbiome composition of genetic material from bacteria in adult patients with and without appendicitis differed, but the microbiome was similar for patients with complicated versus uncomplicated appendicitis. Trial registration NCT03349814.

Acute diverticulitis of the appendix.

Acute diverticulitis of the appendix (ADA), though uncommon, often presents similarly to acute appendicitis but carries a higher risk of complications such as perforation and malignancy. We report the case of a male patient in his 50s with acute right iliac fossa abdominal pain, diagnosed via CT scan with ADA. Urgent laparoscopic appendicectomy was performed, and the patient was discharged without further issues. This case highlights the importance of promptly identifying and managing such conditions to minimise complications and improve outcomes. Despite the overlap in symptoms between appendiceal diverticulitis and acute appendicitis, accurate diagnosis is crucial for appropriate treatment. Healthcare providers should maintain a high index of suspicion, particularly in older patients presenting with an acute appendicitis, like clinical picture to ensure timely intervention and optimal patient care.

Ultrasound Assessment in Children With Suspected Appendicitis: Time to Revise Diagnostic Criteria: A Prospective Cohort Study.

Background: Although ultrasound is considered the gold standard for the evaluation of children with suspected appendicitis, there is still much debate about the most accurate ultrasound findings. The purpose of this study was to define the best ultrasound signs that could ultimately improve the diagnostic accuracy of ultrasound for diagnosing pediatric acute appendicitis, and to differentiate between simple appendicitis and complicated appendicitis. Patients and Methods: After approval by our Institutional Review Board, a prospective study was carried out from January 1, 2022, to July 31, 2023, in a pediatric emergency department. We included all patients aged under 14 years with suspected appendicitis and ultrasound-visualized appendix. Results: A total of 550 patients presented with suspected appendicitis during the study period. Of these children, 411 had an ultrasound-visualized appendix. Our patients' mean age was 9.4 years. The best positive predictive value of ultrasound was found for appendiceal diameter ≥7.5 mm. The combination of an appendiceal diameter <6 mm and the lack of peri-appendiceal free fluid on ultrasound rules out the diagnosis of appendicitis. The best diagnostic accuracy of ultrasound, which was 92%, was achieved for appendix diameters ≥6.5 mm. The sonographic sign giving the best ultrasound accuracy for diagnosing complicated appendicitis was an appendix diameter ≥9 mm. Conclusions: In conclusion, our present study demonstrated that appendiceal diameter ≥6.5 mm is the gold standard for diagnosing appendicitis in pediatric patients. The combination of an appendiceal diameter <6 mm and the lack of peri-appendiceal free fluid on ultrasound would rule out appendicitis.

Expression of Calretinin in the Cecal Muscularis Interna: Observation and Hypothetical Relevance to Appendicitis.

BACKGROUND: The unexpected observation of calretinin immunoreactivity in smooth muscle cells in the muscularis propria of the cecum led to a more detailed examination of calretinin expression and its possible relationship to propulsive contractile activity around the vermiform appendix. METHODS: Immunohistochemistry and RNA in situ hybridization were performed to analyze calretinin expression in intestinal samples from 33 patients at ages ranging from mid-gestation fetuses to adults, as well as in some potentially relevant animal models. Dual immunolabeling was done to compare calretinin localization with markers of smooth muscle and interstitial cells of Cajal. RESULTS: Calretinin expression was observed consistently in the innermost smooth muscle layers of the muscularis interna in the human cecum, appendiceal base, and proximal ascending colon, but not elsewhere in the intestinal tract. Calretinin-positive smooth muscle cells did not co-express markers located in adjacent interstitial cells of Cajal. Muscular calretinin immunoreactivity was not detected in the ceca of mice or macaques, species which lack appendices, nor in the rabbit cecum or appendix. CONCLUSIONS: Localized expression of calretinin in cecal smooth muscle cells may reduce the likelihood of retrograde, calcium-mediated propulsive contractions from the proximal colon and suppress pro-inflammatory fecal stasis in the appendix.

The role of advanced endoscopy in appendiceal polyp management and outcomes.

BACKGROUND: Appendiceal orifice lesions are often managed operatively with limited or oncologic resections. The aim is to report the management of appendiceal orifice mucosal neoplasms using advanced endoscopic interventions. METHODS: Patients with appendiceal orifice mucosal neoplasms who underwent advanced endoscopic resections between 2011 and 2021 with either endoscopic mucosal resection (EMR), endoscopic mucosal dissection (ESD), hybrid ESD, or combined endoscopic laparoscopic surgery (CELS) were included from a prospectively collected dataset. Patient and lesion details and procedure outcomes are reported. RESULTS: Out of 1005 lesions resected with advanced endoscopic techniques, 41 patients (4%) underwent appendiceal orifice mucosal neoplasm resection, including 39% by hybrid ESD, 34% by ESD, 15% by EMR, and 12% by CELS. The median age was 65, and 54% were male. The median lesion size was 20 mm. The dissection was completed piecemeal in 49% of patients. Post-procedure, one patient had a complication within 30 days and was admitted with post-polypectomy abdominal pain treated with observation for 2 days with no intervention. Pathology revealed 49% sessile-serrated lesions, 24% tubular adenomas, and 15% tubulovillous adenomas. Patients were followed up for a median of 8 (0-48) months. One patient with a sessile-serrated lesion experienced a recurrence after EMR which was re-resected with EMR. CONCLUSION: Advanced endoscopic interventions for appendiceal orifice mucosal neoplasms can be performed with a low rate of complications and early recurrence. While conventionally lesions at the appendiceal orifice are often treated with surgical resection, advanced endoscopic interventions are an alternative approach with promising results which allow for cecal preservation.

Evaluation of immunohistochemical expression of novel neuroendocrine marker INSM1 and histological tumor growth pattern in well-differentiated neuroendocrine tumors of the appendix: 15-year single tertiary center experience.

INTRODUCTION: Well-differentiated neuroendocrine tumors of the appendix (A-WDNET) are the most common neoplasms of the appendix but are rare and incidental. INSM1 is a novel transcription factor marker with high sensitivity and specificity for neuroendocrine differentiation. It may display architecturally diverse tumor growth patterns including solid, nest, insular trabecular, and acinar. AIMS: The aim of this study was to determine the staining expression of INSM1 in A-WDNETs and detail the morphological tumor growth patterns. MATERIALS AND METHODS: INSM1 immunohistochemistry was performed on 35 A-WDNET patients. Tumors were histologically classified according to their growth patterns. RESULTS: Thirty-five patients, 60% of whom were male, had a mean age of 30 years at diagnosis and a mean tumor size of 0.9 cm. Eight percent of the tumors invaded the mucosa/submucosa, 34.3% showed invasion into the muscularis propria, 57.1% showed invasion into the subserosa or mesoappendix, LN metastasis was observed in two patients, lymph-vascular invasion in two patients, and perineural invasion in five patients. Ninety-four percent of the tumors were grade 1, the mitotic rate was >2% in two cases, and Ki-67 PI was >3% in two cases. INSM1 was positive in all cases (100%), 1+ 8.6%, 2+ 5.7%, 3+ 17.1%, 4+ 68.6%, weak staining in 11.4%, moderate staining in 22.9%, and strong staining in 67.7%. Type A histological growth pattern was observed in 54.3%, type B in 31.4%, and type C in 14.3% architecturally. CONCLUSIONS: INSM1 was positive in all A-WDNET morphological patterns and was 100% sensitive. INSM1 IHC can be used as an alternative to traditional neuroendocrine markers or in combination with the diagnosis of neuroendocrine tumors.

Transverse colon diverticulitis mimicking acute appendicitis.

Diverticulitis in a solitary transverse colon diverticulum is uncommon, with only a handful of cases documented in the literature. There are various clinical manifestations of the disease, which make clinical and radiological diagnosis rather challenging. Herein, we present a case of a premenopausal female patient in her late 40s who presented to the emergency department, complaining of right lower quadrant abdominal pain, nausea, anorexia and fever. Following clinical, biochemical and radiological tests, the patient was prepared for surgical operation, with the presumed diagnosis of acute appendicitis. An appendicectomy was planned via a McBurney incision. Notably, no inflammation of the appendix was discovered. However, on further exploration, an inflammatory mass was identified in the transverse colon, which was subsequently excised and sent for histological examination. The histology results confirmed the presence of a ruptured solitary transverse colon diverticulum, accompanied by an adjacent mesenteric abscess. The patient's postoperative recovery was uneventful.

Nonneoplastic pathology of the appendix: A review article, based on the recent literature.

OBJECTIVES: The appendix, although considered a vestigial organ, is of considerable clinical importance because acute appendicitis is a common medical problem. There are also other disease processes involving the appendix. The appendix is among the first specimens that the pathologist (and surgeon) cuts one's teeth on. Thus, there may be a tendency to underestimate the clinically and prognostically significant appendiceal pathologies. METHODS: We provide a vade mecum of the pathologic features of a wide range of nonneoplastic appendiceal pathologies, with an emphasis on developing a practical approach to grossing, microscopy, and reporting-all with clinical and therapeutic implications. Much of this is based on literature on MEDLINE with reference to years 2008 to 2023, as well as on personal experiences and interpretations. RESULTS: The appendix can harbor a myriad of nonneoplastic pathologies, including infections, inflammations of varying etiologies (including interval appendectomy), endometriosis, diverticulosis, and so on. Chronic appendicitis, Crohn disease, and clinical audit are recurring themes while COVID-19 is a new entity. CONCLUSIONS: Most importantly, all pathologists should appreciate that the appendix is not as "routine" a specimen as one would want to believe.

'It's just a mucocele': a case report of a massive appendiceal mucocele presenting as a left upper quadrant mass.

Tumours of the appendix are very rare tumours that can and often present with a mucocele. This is a case report highlighting the associated pathology of appendix tumours and the management of a large mucocele. Specifically, how a right hemicolectomy is very rarely needed in these cases regardless of size and local anatomical relationships and some important considerations for the practicing surgeon in the non-tertiary centre that encounters a case like this.