Appendiceal Neoplasms Research Articles

Management of the Uninvolved Uterus and Adnexa During Routine Pelvic Peritonectomy in Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Varies by Histology and Menopausal Status: An International Survey of Peritoneal Surface Malignancy Surgeons.

No guidelines exist regarding the management of the uninvolved uterus or adnexa (fallopian tubes and/or ovaries) in patients with peritoneal metastases (PM) from non-gynecologic malignancies. It is unclear whether salpingo-oophorectomy, hysterectomy, or both should be performed when a complete pelvic peritonectomy is otherwise warranted. A 25-item electronic survey was sent to 225 surgeons worldwide who routinely perform cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC). Participants were recruited through listservs of expert groups. Individual surgeon approaches to the management of the grossly uninvolved uterus and adnexa in pre- and post-menopausal women with PM from low- and high-grade appendiceal neoplasms, colorectal cancer, and peritoneal mesothelioma were collected using a 5-point Likert scale. A total of 135 complete responses (60% response rate) were obtained from surgeons practicing in 27 countries. Respondents reported a median practice of 10 years (interquartile range [IQR] 6-15 years) and a median performance of 20 (IQR 12-30) CRS/HIPEC operations per year. Rates of salpingo-oophorectomy differed by histology and a woman's menopausal status, ranging from 29 to 42% in pre-menopausal women to 71-77% in post-menopausal women (P < 0.001). Notably, the number of surgeons who would perform a hysterectomy was lower, ranging from 12 to 27% for pre-menopausal women and from 32 to 44% for post-menopausal women, dependent on histology (P < 0.001). Surgeons are overall more aggressive with adnexal resection than with hysterectomy in both pre- and post-menopausal women with PM from non-gynecologic malignancies. Further prospective studies are required to determine the best approach to optimize surgical and oncologic outcomes while also accounting for the fertility and hormonal impact.

Appendiceal Mucosal Schwann Cell Proliferation Associated with Incidentally Detected Goblet Cell Adenocarcinoma of Appendix in a Case of Invasive Adenocarcinoma of Sigmoid Colon- A Case Report

Abstract Introduction/Objective Appendiceal mucosal Schwann cell proliferation has been suggested to be a putative histologic marker of appendiceal diverticular disease. Appendiceal Goblet cell adenocarcinoma, (GCA) is amongst the most rare appendiceal tumors with a reported incidence of 0.05 cases per 100,000 population per year in United States. They have features of both neuroendocrine tumors and adenocarcinomas, but behave more similarly to adenocarcinomas. Methods/Case Report Herein, we report a case of appendiceal mucosal schwann cell proliferation associated with incidentally detected Goblet cell adenocarcinoma of appendix in a known case of invasive adenocarcinoma of sigmoid colon. A 75-year-old female presented with sigmoid colon obstruction. Intraoperatively, she was found to have gangrenous and perforated colon, as well as a large obstructing sigmoid mass, and underwent total abdominal colectomy with end-ileostomy. On gross examination, a tan bosselated tumor (up to 3.5 cm) was seen in proximal sigmoid colon. Microscopic examination showed Invasive adenocarcinoma, moderately-differentiated. Appendix was unremarkable on gross examination. Incidentally, the appendix microscopically demonstrated “Goblet cell adenocarcinoma”, histologic grade 1 out of 3 (pathologic stage pTis pN0), present in multiple foci within lamina propria of the distal third of appendix over an area measuring 1.5 cm. Additionally, there were foci of SOX10-positive bland- looking spindle cell proliferation in lamina propria of the appendix. This proliferation is rendered as “Schwann cell proliferation”. Results (if a Case Study enter NA) NA Conclusion One study found that “Schwann cell proliferation” is seen in 42% of appendiceal diverticula cases. It has never been reported in association with “Appendiceal GCA” before. Here we report an intriguing case of Schwann cell proliferation associated with this rare entity of GCA for the first time in literature. Therefore, awareness of such coexistence of these pathologic entities in the same appendix is essential to alert the pathologist to look for GCA, whenever Schwann cell proliferation is encountered in random section of an appendix. This case also emphasizes the importance of being vigilant of the possible presence of other tumors in a specimen with a known tumor.

Risk of appendiceal neoplasm in patients with appendix disorders

The most common appendicular disease is acute appendicitis, with a lifetime risk of 7%–8%. Complicated cases, which can occur in 2%–7% of patients, can significantly impact the severity of the condition and may require different management approaches. Nonoperative management with possible delayed appendectomy has been suggested for selected patients, however, there is a non-negligible risk of missing an underlying malignancy, which is reported to be as high as 11%. Diagnostic work-up is paramount to achieve optimal treatment with good results.

Association between preoperative appendiceal histology grade and Pseudomyxoma peritonei grade offer a solution to avoid right hemicolectomy during cytoreductive surgery and HIPEC

Introduction& objectives: Treatment of PMP consists of appendectomy, cytoreductive surgery (CRS) and HIPEC. Right-sided hemicolectomy is necessary only when PMP is high grade, given the lymphatic invasion risk. To date, no single preoperative factor was identified as predictive of PMP grade.Materials & methods: Preoperative factors of a prospective cohort study on PMP were retrospectively analysed, in order to identify situations linked with high or low grade appendiceal PMP. The main outcome was PMP grade on definitive histology after CRS. Resultsn=105. In univariate analysis, the grade of the appendiceal tumor, systematically reviewed in an expert center, showed an OR of 25.00 (95% CI: 3.30–189.27; p = 0.001) and an NPV of 93.75 [85.36, 100]. Peritoneal biopsy demonstrated an OR of 19.80 (95% CI: 2.30–170.71; p = 0.002) and a PPV of 90 [71.41, 100]. In multivariate analysis, these two factors remained significantly associated with PMP grade. ConclusionWhenever appendiceal tumor is low grade on preoperative histology, the colon has to be spared unless completeness of CRS is compromised, which is a high-grade feature in fact. In case of high grade appendiceal tumor and/or peritoneal biopsy, right-sided hemicolectomy is warranted. If no histology is available preoperatively, adapt to intraoperative lesions as no preoperative factors seem to be predictive.

S2924 An Unexpected Discovery During a Surveillance Colonoscopy: Mucinous Appendiceal Neoplasm

S2924 An Unexpected Discovery During a Surveillance Colonoscopy: Mucinous Appendiceal Neoplasm

S3004 A Rare Case of Mucinous Appendiceal Neoplasm Presenting in a Young Adult Male

S3004 A Rare Case of Mucinous Appendiceal Neoplasm Presenting in a Young Adult Male

Appendiceal Neoplasm in Conservatively Managed Acute Appendicitis: A Retrospective Observational Study.

Introduction Appendiceal neoplasms are more prevalent in patients ≥ 40 years old who present with complicated appendicitis, especially if managed conservatively. Routine interval appendicectomy is not recommended. Follow-up bowel screening using both a CT scan and colonoscopy is recommended. Following the COVID-19 pandemic, many units have increased their utilization of non-operative management of acute appendicitis (NOM).This provides an optimal population sample to study the incidence of unexpected appendiceal malignancy compared to a similar cohort that underwent operative management. The primary outcomes of interest include the incidence of appendiceal malignancy following NOM, efficacy of bowel screening, and rates of re-admission. Methods A retrospective, observational study on patients admitted with acute appendicitis from January 2020 to December 2021. All patients diagnosed with acute appendicitis aged 40 years and older were included in the study, while those under 40 or without a diagnosis of acute appendicitis were excluded. Results We had 211 cases of acute appendicitis. 125 (59%) of which were managed operatively, while 86 cases (41%) were managed NOM. We found six cases (7%) of appendiceal malignancy in the NOM cohort, compared to two cases (1.6%) in the operatively-managed cohort. A routine follow-up colonoscopy failed to reveal any sinister pathology. All six cases underwent interval appendicectomy through which the malignancy was detected. 39 cases (45%) of NOM had at least one episode of re-admission, with 32 (37%) of them being in the first year. Conclusion NOM of acute appendicitis in adults ≥ 40 years old is associated with an increase in unexpected appendiceal malignancy, none of which were detected on follow-up colonoscopy.We emphasize the need for closer surveillance and potentially more aggressive follow-up strategies, including routine interval appendicectomy, for older patients undergoing NOM of acute appendicitis.

EP452 - Outcomes of Appendiceal Cancer Over 10 Years in a District General Hospital

Abstract Aims To evaluate outcomes of appendiceal tumour patients at a district general hospital. Methods A retrospective review was performed to detect patients (16 years or older) with positive histology findings of appendiceal cancers post appendicectomy or colectomy between January 2014 to January 2024. Data was collected from a prospectively maintained electronic patient hospital record (Meditech, Version 6, USA). Results 13 patients were diagnosed to have appendiceal tumours between January 2014 – January 2024. Median age of presentation was 53 (IQR 43).Total Patient Number13Male30.8%Female69.2%Appendiceal Tumour TypePercentage of Appendiceal Tumour PatientsCarcinoid (Neuroendocrine)76.9%Adenocarcinoma7.7%Low Grade Mucinous15.4%High Grade Mucinous0% Median length of stay was 2.5 days (IQR 2.25). Emergency presentations made up 76.9% of admissions. Appendicectomy was the primary operation for 92.3%. 75% of tumours were &amp;lt;1cm, with 25% of tumours &amp;gt;1cm. Location of tumour was predominantly involving the tip in 61.5% of cases, with body and base involvement being 23.1% and 15.4% respectively. Tertiary referral was made for 84.6% of patients. 15.4% had evidence of metastatic disease. Subsequently 15.4% of patients had hyperthermic intraperitoneal chemotherapy (HIPEC), with 7.7% having HIPEC and systemic chemotherapy and radiotherapy. Conclusions Carcinoid (neuroendocrine) was the most common type of appendiceal tumour, mostly presenting as an emergency appendicitis. It was more commonly diagnosed in females. While appendiceal cancers are very rare, outcomes remained good with 100% five-year survival, demonstrating the importance of prompt tertiary referral if further treatment is required.

The incidence of incidental neoplasia in pathology samples of patient who underwent appendectomy due to acute appendicitis. A single center experience: 6446 cases.

Appendectomy is the standard treatment of acute appendicitis. However, recent advancements have introduced medical treatment as a potential alternative. Within this context, it's important to recognize the rare occurrence of appendiceal tumors, often incidentally discovered during histopathological examination following appendectomy. Consequently, there's a concern about the possibility of overlooking appendiceal neoplasms, particularly in cases where conservative treatment for acute appendicitis is considered. The files of patients who underwent surgery for acute appendicitis between January 2015 and December 2023 and were diagnosed with neoplasia in their pathology reports were retrospectively registered. Demographic characteristics of the patients, laboratory and imaging features, details of the surgery performed, preoperative and intraoperative suspicion of neoplasm and data from pathology reports were collected and analyzed. A total of 6446 patients were operated for acute appendicitis. Neoplasia was detected in the histopathological examination of 93 patients (1.44%). Acute appendicitis was diagnosed in 51.6% of the patients by computed tomography. Neoplasm suspicion was present in 9.7% of patients in preoperative imaging methods and in 6.5% of patients intraoperatively. Only appendectomy was performed in 94.5% of patients. The most common appendiceal neoplasm is neuroendocrine tumors (40.9%), followed by appendiceal mucinous neoplasm (29%), sessile serrated adenoma (15%), and adenocarcinoma (6.5%). Although appendiceal neoplasms are relatively rare in proportion, they represent a significant numerical value due to the density of cases. Therefore, this information should be taken into consideration when evaluating treatment options for acute appendicitis.

Carcinoid Heart Disease.

Carcinoid heart disease (CHD) is a rare cardiac complication that occurs most commonly in patients with advanced neuroendocrine tumors and is a known sequela of carcinoid syndrome. Neuroendocrine tumors most widely associated with CHD include tumors in the small bowel, followed by lung, large bowel, pancreatic, appendiceal, and ovarian neoplasms. Carcinoid syndrome is a paraneoplastic syndrome caused by the release of serotonin and other substances from neuroendocrine tumors. It results in a spectrum of symptoms, including diarrhea, flushing, bronchospasm, and symptoms of congestive heart failure. Without treatment and for patients with advanced heart failure, the prognosis of CHD can be less than a year. Management of CHD is often challenging as patients typically present late, and the disease can progress rapidly. Therefore, optimal management of these patients requires close collaboration among various specialties to quantify disease burden, delay the progression of valvular disease, and determine the most effective surgical and medical management strategies depending on the cardiac manifestations to improve quality of life and reduce mortality. This involves a collaborative team, including cardiology and oncology, and often involves many other disciplines, including hepatobiliary and cardiovascular surgeons, endocrinologists, anesthesiologists, and gastroenterologists.

A Low-Grade Appendiceal Mucinous Neoplasm Mimicking a Hydrosalpinx: A Case Report and Review of the Literature

Introduction: A Low-Grade Mucinous Neoplasm (LAMN), formerly known as “mucocele” or “cystadenoma,” is a non-invasive and rare appendiceal tumor, representing 0.2-0.7% of all appendix specimens. Case Report: A 31-year-old female patient who was suffering from chronic right lower quadrant abdominal pain was submitted to an exploratory laparotomy for a presumed right hydrosalpinx. A low-grade mucinous neoplasm of the appendix was discovered and the patient underwent an appendectomy. Discussion: A review of the current literature was performed regarding LAMNs. Conclusion: Further evidence is required in order to address the controversial issues of the pre-operative diagnosis of a LAMN, the extent of surgery performed and the questions regarding fertility preservation in young women.

Postoperative 68Ga-DOTATATE positron emission tomography has a low yield in incidental appendiceal neuroendocrine tumours.

Rarely, appendiceal neuroendocrine tumours (NET) are an incidental finding when an appendicectomy is undertaken for suspected appendicitis. The role of further imaging in this setting is poorly defined. Positron emission tomography (PET) using 68Ga-DOTATATE is requested to evaluate post-surgical status, however, there is little evidence to guide how it should be employed. The aims of this project are to: (i) characterize 68Ga-DOTATATE PET findings in patients with incidental appendiceal NETs and (ii) discuss how these data might inform post-surgical imaging with PET. We reviewed 47 PET scans in 30 patients, undertaken from 2009 to 2018. Scintigraphic findings, histopathological characteristics of the initial appendiceal lesion and medical records were reviewed. Most patients (n = 15) had small (<10 mm) appendiceal NETs with low grade (Ki67 < 2%) features. Eight patients had tumours between 10 and 20 mm, and seven had tumours >20 mm. Goblet cell features were identified in two patients. Three positive PET scans were reported in one patient with an index tumour measuring 40 mm and Ki67 < 2%. The remaining 29 patients had 44 negative scans. Clinical outcome data were available in 27 patients (mean follow-up time 57 months; range 6-123 months). There was no evidence of recurrent neuroendocrine disease at the time of the last follow-up. These data indicate that in most cases, post-surgical 68Ga-DOTATATE PET is negative in patients with incidentally detected appendiceal NETs. Clinical outcome data suggest that 68Ga-DOTATATE PET should be reserved for patients with large tumours (>20 mm) or those displaying goblet cell features.

Pathological Features Associated with Lymph Node Disease in Patients with Appendiceal Neuroendocrine Tumors.

This study aimed to evaluate the role of pathological features beyond tumor size in the risk of lymph node metastasis in appendiceal neuroendocrine tumors. Analyzing data from the national cancer database, we found that among 5353 cases, 18.8% had lymph node metastasis. Focusing on tumors smaller than 2 cm, a subject of considerable debate in treatment strategies, we identified lymphovascular invasion as one of the strongest predictors of lymph node disease. Interestingly, extension into the subserosa and beyond, a current factor in the staging system, was not a strong predictor. These findings suggest that careful interpretation of pathological features is needed when selecting therapeutic approaches using current staging systems.

The Optimal Approach to Surgical Management of Goblet Cell Carcinoid of the Appendix: A Systematic Review.

Goblet cell carcinoid (GCC) is a rare and poorly understood appendiceal neoplasm, exhibiting mixed histological and aggressive clinical features. Current guidelines recommend right hemicolectomy in all cases, although there is conflicting evidence that appendicectomy alone may be sufficient. This review aims to identify the optimal surgical management for appendiceal GCC. A systematic review was performed by searching MEDLINE, Embase, Scopus and the Cochrane Register of Controlled Trials. Randomised controlled trials, cohort studies or large case series (>5 patients) reporting clinical outcomes for patients undergoing surgical management of GCC of the appendix were included. Outcomes extracted included participant and tumour characteristics, type of surgery and survival data. A total of 1341 studies were retrieved. After duplicate removal, 796 titles were screened for relevance prior to abstract and full text review. A total of six studies were included for analysis, comprising 3177 patients-1629 females and 1548 males. The median age ranged from 51 to 72 years. A total of 2329 patients underwent right hemicolectomy, while 824 were treated with appendicectomy only. Overall, the included studies report increased survival in patients undergoing right hemicolectomy compared to appendicectomy alone. A meta-analysis was not possible due to insufficient data reported in the published literature to date. There is no consensus regarding the optimal surgical management of appendiceal GCC, as outcomes-based data comparing surgical interventions are lacking. It is possible that some patients with favourable features are overtreated. The absence of robust evidence to support a more conservative approach means that right hemicolectomy remains the standard of care for all patients, in keeping with current international guidelines. The rarity of this condition and limited data in the published studies remain barriers to evidence-based best clinical practice.

Appendiceal goblet cell adenocarcinoma with perineural invasion extending into the ileocecal lesion

BackgroundAppendiceal goblet cell adenocarcinoma (GCA) is a rare subtype of primary appendiceal adenocarcinoma with an incidence of 1–5 per 10,000,000 people per year. Appendiceal tumors are often diagnosed after appendectomy for acute appendicitis. Notably, however, there is currently no standard treatment strategy for GCA, including additional resection. We report a case of appendiceal GCA with perineural extension into the cecum, in which ileal resection was considered effective.Case presentationA 41-year-old man was diagnosed with acute appendicitis and underwent appendectomy. Histopathological findings revealed GCA (T3, Pn1). He was referred to our hospital for additional resection. Preoperative examination indicated a diagnosis of GCA cT3N0M0. Laparoscopic ileocecal resection and D3 lymph node dissection were performed 2 months after initial appendectomy. The patient had a good postoperative course and was discharged 8 days after surgery. Histopathological findings showed a GCA invading the cecum, despite an intact appendiceal stump, no lymph node metastasis, no vascular invasion, and no horizontal extension into the submucosa. Direct invasion of the tumor through the serosa was not observed, but perineural extension was conspicuous in the cecum, suggesting that the GCA extended into the cecum via perineural invasion. The resection margins were negative. The patient has survived free of recurrence for a year after ileocecal resection.ConclusionsThe current patient was diagnosed with appendiceal GCA following appendectomy for acute appendicitis. Despite intact of appendiceal stump and no evidence of lymph node or distant metastasis, he underwent laparoscopic ileocecal resection and D3 lymph node dissection 2 months after initial appendectomy, with a favorable outcome. Despite the detection of perineural invasion, the patient declined adjuvant therapy. This case suggests that extensive resection may be required in patients with appendiceal GCA, but the role of adjuvant therapy remains unclear.

Comparison of EPIC Versus HIPEC in the Treatment of Colorectal Peritoneal Metastases and Appendix Tumors Using Inverse Probability of Treatment Weighting.

The selection of hyperthermic intraperitoneal chemotherapy (HIPEC) or early postoperative intraperitoneal chemotherapy (EPIC) for peritoneal metastases from colorectal cancer or appendiceal neoplasms following cytoreductive surgery (CRS) depends on the surgeon's discretion. This study was designed to compare postoperative and oncologic outcomes of HIPEC and EPIC using inverse probability of treatment weighting (IPTW). This study included 175 patients who received HIPEC or EPIC following CRS at a single tertiary university hospital between December 1999 and December 2020. Inverse probability of treatment weighting analysis was performed to control for pretreatment characteristics between the two groups. Multivariate analysis was performed to determine factors associated with postoperative and survival outcomes. After IPTW, no significant differences in baseline demographics and tumor characteristics were observed between the two groups. The HIPEC group had a significantly longer operation time than the EPIC group. The EPIC group showed a significantly higher postoperative mortality rate than the HIPEC group. Operation time (odds ratio [OR] 1.01; 95% confidence interval [CI] 1.01-1.02; p < 0.001), bowel anastomosis (OR 7.25; 95% CI 1.16-45.2; p = 0.034), neoadjuvant chemotherapy (OR 7.62; 95% CI 1.85-31.4; p = 0.005), and EPIC (OR 8.76; 95% CI 2.16-35.5; p = 0.002) were independent risk factors for major surgical complications. No association was observed between intraperitoneal chemotherapy type and major hematologic toxicity, overall survival, progression-free survival, or peritoneal progression-free survival. EPIC was a risk factor for major surgical complications. Survival outcomes were similar between the two types of intraperitoneal chemotherapy.

Minimum number of lymph nodes to maximize survival in non-metastatic appendiceal adenocarcinoma

IntroductionIn non-metastatic appendiceal adenocarcinoma, the relationship between the number of lymph nodes harvested and overall survival is unknown. The objective of this study was to determine whether the number of lymph nodes harvested impacts survival in patients with mucinous and non-mucinous appendiceal cancer. MethodsRetrospective cohort analysis was conducted in patients who underwent surgery for appendiceal adenocarcinoma in the SEER database from 2000–2019. Exclusion criteria included patients without the number of regional nodes harvested/node positivity, missing survival data, stage IV disease, and certain subtypes of appendiceal tumors. The primary outcome was 10-year overall survival. ResultsA total of 1651 patients were identified, 1101 with mucinous and 550 with non-mucinous cancer. The majority of patients were male (54 %) and between the ages of 50–74 (52 %). Mean ± SD number of lymph nodes harvested was 17.04 ± 10.41. The ten-year survival rates for patients with mucinous appendiceal cancer were 43–74 % and 25–68 % for non-mucinous cancer, depending on AJCC stage. Multivariate Cox-regression analysis displayed that the number of lymph nodes harvested was an independent predictor of survival (p < 0.0001). The optimal threshold predicted by Cox-regression for maximal survival benefit was harvest of a minimum of 15 nodes for mucinous and 12 for non-mucinous cancer. Additionally, for mucinous cancer, per each additional 3 nodes harvested past 15, the 10-year risk of mortality decreased by 7 % (HR 0.93 (0.88 to 0.98), p < 0.001). ConclusionsThe number of lymph nodes harvested is an independent predictor of survival for patients with appendiceal adenocarcinoma. To maximize this survival benefit, a minimum of 15 regional lymph nodes must be harvested for mucinous and 12 for non-mucinous cancer.

Survival among 148 patients with an incidentally detected appendiceal tumours at surgery for acute appendicitis: a population-based cohort follow-up study.

To investigate the long-term prognosis of appendiceal tumours incidentally detected at appendicectomy for suspicion of benign appendicitis. A retrospective register-based single centre cohort study was carried out, using data from the local acute appendicectomy quality register of cases operated on at the Department of Surgery, South General Hospital, Stockholm, Sweden. The local colorectal cancer register was also used to identify appendix tumours. The study period was between January 2004 and January 2023. Survival was calculated according to the Kaplan-Meier method. A total of 11,888 patients were registered in the acute acute appendicectomy register, 54% males and 46% females, median age 32 (Q1 = 21, Q3 = 47) (with 33.7% were 41 years or older). From the appendicectomy and colorectal registers 148 (1.2% of the total cohort) appendiceal tumours were found; 60% in females and 40% in males, median age 56 (Q1 = 43, Q3 = 70) (with 78.4% being 41 years or older). Tumours found were: Low grade Appendiceal Mucinous Neoplasms (LAMN, N = 64); Neuroendocrine Tumours (NET N = 24); adenocarcinomas or other form of carcinomas (N = 57); and adenomas (N = 3). The overall 5-year survival in patients operated for LAMN was 96.8%, for NET 93.3% and for adenocarcinoma 69.7%. The overall 5-year survival for all tumour patients was 85.7%. For the younger patients (< 51 years) with LAMN and NET, almost all survived to the end of follow-up. Survival of patients in the carcinoma group was statistically significantly lower than for the LAMN and NET groups, especially in females 51 years or older. In the group of tumour patients undergoing surgery (n = 146), primary surgery was laparoscopic in 47% and open in 52%. Two patients did not undergo surgery due to widespread disease. In 64% of cases operation was acute, whereas it was delayed and/or planned in 34%. Most procedures were laparoscopic appendicectomy 36%, followed by open appendicectomy 30%, right-sided hemicolectomy 14.6% (open 11.6% and laparoscopic 3%, acute operation 5.5%), ileocaecal resection 5% (acute operation 3.4%), and staging laparoscopy 7%. In 38% of the operated patients the tumour was discovered incidentally at histopathology examination. Two patients had CRS and HIPEC as the initial operation. Forthy-three per cent of the 146 tumour patients operated underwent a second procedure: CRS and HIPEC in 23.3% and right-sided hemicolectomy in 13.6% (laparoscopic 8.2% open 5.4%). Survival was high for patients with incidentally detected appendiceal LAMN or NET, but not so for carcinoma. Survival was lower in the carcinoma group older than 50 years, especially those sick and females.

Appendiceal goblet cell adenocarcinoma newly classified by WHO 5th edition: a case report (a secondary publication)

BackgroundAppendiceal goblet cell adenocarcinoma (AGCA) is a newly proposed cancer type in the 5th edition of the WHO Classification of Tumours in 2019. We experienced this rare form of appendiceal primary neoplasm.Case presentationAn 85-year-old male presented a positive fecal occult blood test. A series of imagings revealed a type 1 tumor, located on the appendiceal orifice. The subsequent biopsy made the diagnosis of signet-ring cell carcinoma. Consequently, he underwent the laparoscopic-assisted ileocecal resection. Initially, the tumor was suspected to be a Goblet cell carcinoid (GCC). There was a discrepancy between the histological and immunostaining findings: the tumor cells exhibited morphological similarities to GCCs, however displayed limited staining upon immunostaining. Ultimately, we concluded that the tumor should be classified as AGCA, by following WHO 5th Edition. AGCA represents a newly categorized subtype of adenocarcinomas. Because of our preoperative suspicion of malignancy, we performed tumor resection with regional lymph node dissection, despite the fact that most appendiceal malignant tumors are typically identified after an appendectomy.ConclusionWe experienced a case that provides valuable insights into the comprehension of AGCA, a recently established pathological entity in the WHO 5th Edition. This article is an acceptable secondary publication of a case report that appeared in Azuma et al. (J Jpn Surg Assoc 83:1103–1108, 2022)

Clinical Significance of Circulating Tumor Cells in Epithelial Appendiceal Neoplasms with Peritoneal Metastases.

Appendiceal tumors are uncommon and, at times, discovered incidentally during histological examination. The histopathological classification of the disease is complex and has generated some controversy. The analysis of circulating tumor cells can be used for the early detection of metastatic potential. The aim of the present study was to examine the prognostic value of circulating tumor cells in patients with appendiceal tumors and peritoneal metastases. To our knowledge, this is the first study to examine CTCs in appendiceal tumors. We performed a prospective cohort study of consecutive patients treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy between 2015 and 2019 at a HIPEC referral center. In total, 31 patients were included in the analysis, and circulating tumor cells were detected in 15 patients (48%). CTC positivity was not associated with overall or recurrence-free survival, nor was it correlated with PCI score or histopathological grading. Surprisingly, however, CTCs were found in almost half the patients. The presence or quantities of these cells did not, on their own, predict systemic metastatic potential during the observed time, and they did not appear to significantly correlate with the oncological outcomes recorded.