Appendiceal adenocarcinoma, an exceedingly rare malignancy, sparks debate on the optimal surgical approach-appendectomy or right hemicolectomy-for early-stage cases. This study aims to investigate the impact of these two surgical methods on the survival prognosis of patients with early appendiceal adenocarcinoma.

Utilizing a multicenter medical database, we gathered data from 168 patients diagnosed with T1 stage appendiceal adenocarcinoma admitted between January 2008 and January 2015. This study aims to compare the impact of different treatment modalities on the prognosis of appendiceal adenocarcinoma in these two groups.

In patients diagnosed with T1 appendiceal adenocarcinoma, the survival prognosis was not significantly improved with right hemicolectomy compared to appendectomy. Out of one hundred twenty-seven patients undergoing right colon resection, only three exhibited lymphatic metastasis, resulting in a rate of 2.3%.

Simple appendectomy can fulfill the objective of achieving radical tumor resection, rendering right hemicolectomy unnecessary.

Appendiceal mucinous lesions' classification and nomenclature has been modified several times along the last decades, reflecting their great heterogeneity and making difficult to compare results and draw conclusions. Despite its nearby origin, appendiceal mucinous lesions have a distinctive behaviour compared to colorectal cancer, including their molecular and genetic markers. Due to their low frequency, their management is not well standardised. However, surgery is considered the cornerstone of treatment. Their indolent behaviour has encouraged surgeons to apply more aggressive treatments, such as cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC), that may extend overall survival. Chemotherapy is reserved for unresectable and/or disseminated disease and could play a role in the adjuvant and neoadjuvant setting. Pressurised intraperitoneal aerosol chemotherapy (PIPAC) is recently emerging as a possible alternative for treatment in advanced disease although its results in long-term survival are lacking Hereby, we review the available evidence in the management of appendiceal mucinous lesions, including localised and disseminated disease, with a special emphasis on the oncological perspective, focusing on the lights and shadows of the systemic treatments.

We recently read with great interest a study by Zhang et al in the World Journal of Gastroenterology. In our practice, we focus specifically on examining appendiceal mucinous neoplasms (AMNs) with endoscopic ultrasound (EUS) using different scopes. AMNs are rare neoplastic lesions characterized by an accumulation of mucin inside a cystic dilatation of the appendix. Clinically, they can present as nonspecific acute appendicitis. AMNs can turn into a life-threatening condition, termed pseudomyxoma peritonei, in which the ruptured appendix causes accumulation of mucin in the abdomen. Therefore, accurate and rapid diagnosis of AMN is essential. EUS is able to confirm and stage AMNs; although, EUS examination was once limited to the rectal and anal regions due to the conventional oblique-view scopes. With the emergence of new forward-view linear echoendoscopes and instruments like EUS miniprobes and overtubes, the scope of examination is changing. Herein, we discuss the feasibility of using the curved linear array echoendoscopes to examine cecal and appendiceal orifice lesions.

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).

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.

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.

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.

Epithelial-mesenchymal transition (EMT), angiogenesis, cell adhesion and extracellular matrix (ECM) interaction are essential for colorectal cancer (CRC) metastasis. Low grade mucinous neoplasia of the appendix (LAMN) and its advanced state low grade pseudomyxoma peritonei (lgPMP) show local aggressiveness with very limited metastatic potential as opposed to CRC. To better understand the underlying processes that foster or impede metastatic spread, we compared LAMN, lgPMP, and CRC with respect to their molecular profile with subsequent pathway analysis. LAMN, lgPMP and (mucinous) CRC cases were subjected to transcriptomic analysis utilizing Poly(A) RNA sequencing. Successfully sequenced cases (LAMN n = 10, 77%, lgPMP n = 13, 100% and CRC n = 8, 100%) were investigated using bioinformatic and statistical tests (differential expression analysis, hierarchical clustering, principal component analysis and gene set enrichment analysis). We identified a gene signature of 28 genes distinguishing LAMN, lgPMP and CRC neoplasias. Ontology analyses revealed that multiple pathways including EMT, ECM interaction and angiogenesis are differentially regulated. Fifty-three significantly differentially regulated gene sets were identified between lgPMP and CRC followed by CRC vs. LAMN (n = 21) and lgPMP vs. LAMN (n = 16). Unexpectedly, a substantial enrichment of the EMT gene set was observed in lgPMP vs. LAMN (FDR=0.011) and CRC (FDR=0.004). Typical EMT markers were significantly upregulated (Vimentin, TWIST1, N-Cadherin) or downregulated (E-Cadherin) in lgPMP. However, MMP1 and MMP3 levels, associated with EMT, ECM and metastasis, were considerably higher in CRC. We show that the different tumor biological behaviour and metastatic spread pattern of midgut malignancies is reflected in a different gene expression profile. We revealed a strong activation of the EMT program in non-metastasizing lgPMP vs. CRC. Hence, although EMT is considered a key step in hematogenous spread, successful EMT does not necessarily lead to hematogenous dissemination. This emphasizes the need for further pathway analyses and forms the basis for mechanistic and therapy-targeting research.

Appendiceal mucinous adenocarcinoma, one kind of specific colorectal cancer, is lowly prevalent and rarely diagnosed in clinical practice. In addition, there have been limited standard treatment strategies established for patients with appendiceal mucinous adenocarcinoma, especially with metastatic disease. The regimens for colorectal cancer, which were adopted in appendiceal mucinous adenocarcinoma, usually resulted in limited effectiveness.

Herein, we presented a case of chemo-refractory patient with metastatic appendiceal mucinous adenocarcinoma harboring ATM pathological mutation of exon 60, c.8734del, p.R2912Efs*26, and who has achieved a persistent response to salvage treatment of niraparib, with disease control time that reached 17 months and still in extension.

We supposed that appendiceal mucinous adenocarcinoma patients harboring ATM pathological mutations may respond to the treatment of niraparib, even without a homologous recombination deficiency (HRD) status; however, it needs further confirmation in a larger cohort.

Appendiceal mucinous neoplasms (AMNs) are rare adenomatous primary tumors of the appendix. Although of low malignant potential, these neoplasms can cause serious potentially fatal complications such as bowel obstruction and pseudomyxoma peritonei, making prompt identification and removal of utmost importance. AMNs often present with nonspecific gastrointestinal symptoms or are asymptomatic and found incidentally.

A patient aged 72 years presented with generalized weakness and appeared on imaging to have acute appendicitis complicated by rupture. On colonoscopy, the patient was found to have an inverted appendix that after appendectomy was revealed to harbor a perforated low-grade AMN.

Although AMNs are rare, physicians should still consider it when imaging suggests appendicitis. Having AMNs as part of the differential diagnosis is especially necessary in cases, such as this one, in which the patient has appendiceal inversion, is aged > 50 years, and has concurrent colorectal neoplasms.

Low-grade appendiceal mucinous neoplasms (LAMNs) are generally treated by surgical resection, but posttreatment surveillance protocols are not well-established. The objectives of this study were to characterize posttreatment surveillance and determine the risk of recurrence following surgical resection of LAMN.

Patients who underwent surgical resection of localized LAMNs in an 11-hospital regional healthcare system from 2000 to 2019 were identified. Posttreatment surveillance regimens were characterized, and rates of disease recurrence were evaluated.

A total of 114 patients with LAMNs were identified. T-category was pTis for 92 patients (80.7%), pT3 for 7 (6.1%), pT4a for 14 (12.3%), and pT4b for 1 (0.9%). Two patients (1.8%) had a positive resection margin. Posttreatment surveillance was performed for 39 (34.2%) patients and consisted of office visits for 32 (82%) patients, computerized tomography imaging for 30 (77%), magnetic resonance imaging for 5 (13%), colonoscopy for 15 (38%), and serum tumor marker measurement for 12 (31%). After a mean follow-up duration of 4.7 years, no patients experienced tumor recurrence.

Posttreatment surveillance is common among patients with LAMNs. However, no patients experienced tumor recurrence, regardless of T-category or margin status, suggesting that routine surveillance following surgical resection of LAMN may be unnecessary.

Background: The COVID-19 pandemic has put enormous pressure on hospital resources, and has affected all aspects of patient care. As operative volumes decrease, cancer surgeries must be triaged and prioritized with careful thought and attention to ensure maximal benefit for the maximum number of patients. Peritoneal malignancies present a unique challenge, as surgical management can be resource intensive, but patients have limited non-surgical treatment options. This review summarizes current data on outcomes and resource utilization to help inform decision-making and case prioritization in times of constrained health care resources. Methods: A rapid literature review was performed, examining surgical and non-surgical outcomes data for peritoneal malignancies. Narrative data synthesis was cross-referenced with relevant societal guidelines. Peritoneal malignancy surgeons and medical oncologists reviewed recommendations to establish a national perspective on case triage and mitigating treatment strategies. Results and Conclusions: Triage of peritoneal malignancies during this time of restricted health care resource is nuanced and requires multidisciplinary discussion with consideration of individual patient factors. Prioritization should be given to patients where delay may compromise resectability of disease, and where alternative treatment options are lacking. Mitigating strategies such as systemic chemotherapy and/or surgical deferral may be utilized with close surveillance for disease stability or progression, which may affect surgical urgency. Unique hospital capacity, and ability to manage the complex post-operative course for these patients must also be considered to ensure patient and system needs are aligned.

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.

No guidelines exist for surveillance following cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) for appendiceal and colorectal cancer. The primary objective was to define the optimal surveillance frequency after CRS/HIPEC.

The U.S. HIPEC Collaborative database (2000-2017) was reviewed for patients who underwent a CCR0/1 CRS/HIPEC for appendiceal or colorectal cancer. Radiologic surveillance frequency was divided into two categories: low-frequency surveillance (LFS) at q6-12mos or high-frequency surveillance (HFS) at q2-4mos. Primary outcome was overall survival (OS).

Among 975 patients, the median age was 55 year, 41% were male: 31% had non-invasive appendiceal (n = 301), 45% invasive appendiceal (n = 435), and 24% colorectal cancer (CRC; n = 239). With a median follow-up time of 25 mos, the median time to recurrence was 12 mos. Despite less surveillance, LFS patients had no decrease in median OS (non-invasive appendiceal: 106 vs. 65 mos, p < 0.01; invasive appendiceal: 120 vs. 73 mos, p = 0.02; colorectal cancer [CRC]: 35 vs. 30 mos, p = 0.8). LFS patients had lower median PCI scores compared with HFS (non-invasive appendiceal: 10 vs. 19; invasive appendiceal: 10 vs. 14; CRC: 8 vs. 11; all p < 0.01). However, on multivariable analysis, accounting for PCI score, LFS was still not associated with decreased OS for any histologic type (non-invasive appendiceal: hazard ratio [HR]: 0.28, p = 0.1; invasive appendiceal: HR: 0.73, p = 0.42; CRC: HR: 1.14, p = 0.59). When estimating annual incident cases of CRS/HIPEC at 375 for non-invasive appendiceal, 375 invasive appendiceal and 4410 colorectal, LFS compared with HFS for the initial two post-operative years would potentially save $13-19 M/year to the U.S. healthcare system.

Low-frequency surveillance after CRS/HIPEC for appendiceal or colorectal cancer is not associated with decreased survival, and when considering decreased costs, may optimize resource utilization.