Tumors Of Appendix Research Articles

The risk of developing pseudomyxoma peritonei from a non-perforated low grade appendiceal mucinous neoplasm found at appendicectomy

IntroductionLow-grade appendiceal mucinous neoplasms (LAMNs) are classified as non-perforated (pTis, pT3) or perforated (pT4), and considered precursors of pseudomyxoma peritonei (PMP). This study aims to quantify the risk of developing PMP from pTis and pT3 LAMNs. Materials and methodsRetrospective analysis of a prospectively collected database identified LAMN patients referred to a specialist centre from 2004 to 2019. pT4 LAMNs and other appendix tumours were excluded. All patients had specialist review of their pathology, operation note, and a CT scan (at least 6 weeks post-operatively). Surveillance CTs were then performed at 6, 12, 24, 36, 48, & 60 months, with tumour markers (CEA, CA19-9, CA125). Results193 pT3/pTis LAMN patients were included (pTis = 153, pT3 = 40). Median follow-up = 6.45 (3.91–22.13) years, M:F ratio = 1:1.57, and median age = 57 (23–83) years. Initial surgery included: appendicectomy (67 %), appendicectomy + visceral resection (6 %), and right hemicolectomy (27 %). R1 resections were identified in 5/193 patients (2.5 %). 3 R1 patients underwent re-operation (2 caecal pole excision and 1 ileocecectomy), none of which had residual tumour. 8/193 patients (4 %) were lost to follow up. None of the remaining 185 developed PMP. ConclusionThis is the largest reported series of pTis/pT3 LAMNs with standardised follow-up in the literature. LAMNs correctly classified as pT3/pTis (after careful specialist review of pathology, operation note, and a baseline post-operative CT) have negligible risk of developing PMP and should have low intensity surveillance. If completely excised, further surgery is not indicated. R1 resections should be considered on an individual basis at a specialist centre.

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.

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

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

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.

Mucinous carcinoma of the appendix: a case report

Mucinous carcinoma of the appendix is ​​​​a rare malignant tumor of the appendix. Incidence ranges about 0.01-0.2% in cases of malignancy in the gastrointestinal tract. Clinically, the symptoms appear to resemble acute appendicitis. The diagnosis was established at intraoperative time. Studies on this case are still few. A 69-year-old man complained of lower right abdominal pain since 6 days before going to Sanjiwani hospital. The patient also complained of fever, nausea, decreased appetite and constipation. Examination results temperature 37.6 0C, McBurney sign (+), rebound tenderness (+), localized defense on lower right abdomen. Ultrasound examination of the abdomen with an impression of the end tubular structure, a peristaltic diameter of 0.87 cm accompanied by a fluid collection, suspicious of appendicitis with perforation. Inflamed appendix and localized mucin were found at ileocaecal junction intraoperatively. The patient underwent a right hemicolectomy. Histopathological examination revealed a morphological picture suitable for mucinous carcinoma of the appendix. Mucinous carcinoma of the appendix is ​​​​a rare malignant tumor of appendix. Patients can have the same symptoms as appendicitis. Ultrasound investigations can find a picture like appendicitis. Mucinous carcinoma of the appendix is ​​more frequently found intraoperatively. Histopathological examination is necessary to determine a definitive diagnosis of mucinous carcinoma of the appendix. In high grade appendiceal mucinous neoplasm (HAMN) a right hemicolectomy can be performed and continued with adjuvant chemotherapy.

Primary appendiceal lymphoma managed through videolaparoscopic appendectomy in an Amazonian center: Case report and review of literature

Introduction: Primary gastrointestinal lymphoma is rare and appendix involvement is rarer. Most cases of appendiceal lymphoma present as clinical acute appendicitis and are generally diagnosed intra-operatory. Some computed tomography findings in preoperative evaluation may suggest appendix neoplasm rather than appendicitis, such as appendix diameter larger than 15 mm, abdominal lymphadenopathy and aneurysmal dilatation of the lumen. Case Report: We report a case of a 26-year-old Brazilian man first diagnosed with acute appendicitis and submitted to videolaparoscopic approach with appendectomy and posterior chemotherapy after histopathological reveal of primary appendiceal lymphoma. Conclusion: Surgical resection followed by chemotherapy remains as standard management choice when possible. Adequate investigation and mandatory histopathological analysis in appendectomies are fundamental for diagnosis and proper treatment.

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

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. 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. 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. 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.

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. 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. 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. 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.

A Case of Mucinous Neoplasm of Appendix with Pseudomyxoma Peritonei and Ovarian Metastasis

A Case of Mucinous Neoplasm of Appendix with Pseudomyxoma Peritonei and Ovarian Metastasis

Childhood Neuroendocrine Tumors of Appendix: Suggested Approach and Management

Childhood Neuroendocrine Tumors of Appendix: Suggested Approach and Management

Epidemiological and Pathological Profile of Cutaneous Adnexal Tumors, Central Laboratory of Pathological Anatomy and Cytology of Ibn Sina Hospital, Rabat Morocco: Report of 35 Cases

Skin appendix tumors are rare and highly varied tumors. Are mostly benign characters, rarely malignant and dominated from the morphological point of view by their lesional polymorphism. These tumors may present with follicular, apocrine/eccrine, sebaceous and sometimes mixed differentiation, posing diagnostic problems. The aim of this study is to describe the epidemiological profile and the different anatomopathological aspects of this group of tumors and to compare them with data from the literature. We carried out a descriptive study with a retrospective stage, of 35 cases of cutaneous adnexal tumors collected at the service of the Central Laboratory of Pathological Anatomy and Cytology of Ibn Sina Hospital, CHU of Rabat during a period of 3 years, from 2019, 2020 and 2021. The result of this study shows the peak frequency of these lesions, which is located between 60 years and older, occupying 34.2% of all cases; the average age of the patients studied is 41 years and we find the predominance of the female sex which corresponds to 62.9% of the cases; the predilection seat of the lesions is the region of the head and neck, which is occupied by 51.4%; tumor differentiation is divided into apocrine/eccrine which represents 68.6% of cases; follicular in 20% of cases and sebaceous in 11.4% of cases; the most common histological type is poroma 25.7% followed by pilomatricoma 14.2% and papilliferous syringocystadenoma 11.4%; benign tumors are frankly in the majority, occupying 91.5%, against 8.5% of malignant tumors.

Incidental appendiceal neoplasms: Single-centre results.

Histopathological examination of appendectomy specimens may reveal malignancies. Based on these results, either appendectomy is sufficient or sometimes a further treatment protocol can be needed. In this study, malignancy-diagnosed cases on appendectomy specimen were examined. Patients who underwent appendectomy between January 2013 and December 2018 with a pre-diagnosis of acute appendicitis were evaluated retrospectively and those cases with malignancy were included in the study. Patients' age, sex, tumor type, tumor diameter, tumor grade, tumor localization, surgical margin, Ki-67 index, state of lymphovascular invasion, state of peri-neural invasion, and follow-up period duration were recorded. On examination of 2336 appendectomy specimens, 16 patients (0.7%) were found to have neuroendocrine tumors (NET), 11 patients (0.5%) were found to have low-grade mucinous neoplasm (LAMN), and five patients (0.2%) were found to have primary appendix carcinomas. Appendix tumors usually present with acute appendicitis symptoms. Despite re-operation with right hemicolectomy (RHC) being required in the treatment of adenocarcinoma cases, appendectomy provides adequate treatment in most cases with NET and LAMN. With these tumors, which usually have a benign prognosis, it is important to perform the necessary screening in the postoperative period and not to interrupt follow-up.

Incidental Neuroendocrine Tumors of Appendix: Study from a Tertiary Care Hospital

Incidental Neuroendocrine Tumors of Appendix: Study from a Tertiary Care Hospital

376 Neoplasms of the Appendix- Are They Managed Appropriately?

Abstract Aim Appendix neoplasms are rare. Half are neuroendocrine tumours (NET). Incidence of NET is 0.4 per hundred thousand and of low-grade appendix mucinous neoplasm (LAMN) is 0.2-0.3 per hundred thousand. Most are diagnosed incidentally on appendicectomy specimens. The aim of this study was to ascertain the incidence of appendix neoplasms managed in a tertiary referral centre and to ascertain compliance with international guidelines. Method A prospectively maintained gastrointestinal oncology database was interrogated over a six-year period (July 2016-July 2022). Histologically confirmed primary appendix neoplasms were included. Data collected included patient demographics, clinical presentation, management and follow up (median 38 months). Histopathological staging was according to the AJCC 8th edition. Institutional review board permission was not required. Results Incidence of appendix neoplasm over the study period is 1.3% (16/1259). These included 8 NET, 5 LAMNs, 2 invasive adenocarcinoma and 1 adenocarcinoid. A majority were managed with definitive appendicectomy. One patient underwent right hemicolectomy following discussion at tumour board meeting. Four patients were enrolled on a surveillance programme. A further four with LAMN underwent appendicectomy only and one had appendicectomy and partial caecectomy. Surveillance was not deemed necessary in any of the LAMN cases. Conclusions Incidence of appendix neoplasm in our centre is comparable to that reported in larger series (2%). All cases were discussed at tumour board meeting. Management in all cases was in keeping with current guidelines. While most patients had adequate margin clearance with appendectomy alone, a small number required further surgery and/or surveillance.

A Comparative Study between Pre-Operative and Intra-Operative Findings among Patients with Acute Appendicitis

Background: Acute appendicitis could be due to a bacterial, viral or parasitic infection in the digestive tract, which can enlarge the tissue of the appendix wall, stools causing a blockage in the tube between the large intestine and the appendix tumors, inflammatory bowel disease, injury or trauma to the abdomen. It is a sudden and severe inflammation of the appendix. The symptoms of acute appendicitis occur suddenly and are usually severe. In severe cases, appendicectomy is often needed. Aim of the study: The aim of this study was to compare between pre-operative and intra-operative findings among patients with acute appendicitis. Methods: This cross-sectional study was conducted in the department of Surgery, North East Medical College Hospital, Sylhet, Bangladesh during the period from July 2008 to June 2009. In total 100 clinically diagnosed cases of acute appendicitis were enrolled in this study as study subjects. Proper written consents were taken from all the participants before data collection. All the pre-operative and intra-operative findings among patients were recorded and analyzed. All data were processed, analyzed and disseminated by using SPSS version 16.0 program as per necessity. Results: In this study, among total 100 cases, clinically acute appendicitis was found in 78% and burst appendix in 22% of cases. Appendix was found retrocaecal in 67%, pelvic in 30% and pracaecal, subcaecal and postileal each comprises 1% of the patients. Finally, we found a significant correlation of pre-operative diagnosis with luminal contents (P&lt;0.0001). Besides this, the findings of this study indicated that, the clinical diagnosis was concordance with intra-diagnosis in 84% of cases and discordant in 16% of cases. Conclusion: In this current study, pre-operative clinical diagnosis was concordance with intra-operative diagnosis in majority of the cases. So, we can conclude that, the surgeon should be careful about the pre-operative assessment of the cases in ........

The Vermiform Appendix and Its Pathologies.

The vermiform appendix is a muscular cylindrical structure originating near the junction of the cecum and ileum, averaging 9 cm (5-35 cm) in size. As the most mobile viscera, it can adopt several positions, the most common being the retrocecal position. Perceived as an atavistic organ lacking physiological relevance, the vermiform appendix appears to be involved in immune function, serving in the maturation of B lymphocytes and the production of immunoglobulin A, in endocrine function, excreting amines and hormones in the 2-3 mL of mucus secreted daily, and in digestive function, by storing beneficial bacteria from where they can recolonize the colon. With a lumen of about 6 mm, the vermiform appendix has a reduced storage capacity, so any blockage of the appendix with fecoliths (fecaliths), seeds derailed from the colon, or enlarged lymph nodes prevents drainage and intraluminal accumulation of secreted mucus. Unable to relax, the appendix wall severely limits its intraluminal volume, so mucus accumulation leads to inflammation of the appendix, known generically as appendicitis. In addition, the vermiform appendix may be the site of the development of neoplastic processes, which may or may not involve mucus production, some of which can significantly affect the standard of living and ultimately lead to death. In general, mucinous tumors may have a better prognosis than non-mucinous tumors. This review takes a comprehensive path, starting by describing the anatomy and embryology of the vermiform appendix and further detailing its inflammatory pathologies, pathologies related to congenital anomalies, and appendix tumors, thus creating an up-to-date framework for better understanding, diagnosis, and treatment of these health problems.

Landscape of Genetic Mutations in Appendiceal Cancers.

In appendiceal cancers, the most frequently mutated genes are (i) KRAS, which, when reactivated, restores signal transduction via the RAS-RAF-MEK-ERK signaling pathway and stimulates cell proliferation in the early stages of tumor transformation, and then angiogenesis; (ii) TP53, whose inactivation leads to the inhibition of programmed cell death; (iii) GNAS, which, when reactivated, links the cAMP pathway to the RAS-RAF-MEK-ERK signaling pathway, stimulating cell proliferation and angiogenesis; (iv) SMAD4, exhibiting typical tumor-suppressive activity, blocking the transmission of oncogenic TGFB signals via the SMAD2/SMAD3 heterodimer; and (v) BRAF, which is part of the RAS-RAF-MEK-ERK signaling pathway. Diverse mutations are reported in other genes, which are part of secondary or less critical signaling pathways for tumor progression, but which amplify the phenotypic diversity of appendiceal cancers. In this review, we will present the main genetic mutations involved in appendix tumors and their roles in cell proliferation and survival, and in tumor invasiveness, angiogenesis, and acquired resistance to anti-growth signals.

Mucinous tumors of the peritoneum

Disseminated peritoneal seeding of mucin-forming tumors is arare malignant disease with variable prognosis. Histomorphological criteria are instrumental in the prognostic assessment. The past 10years have led to astandardization of nomenclature and subsequently to the establishment of therapeutic standards. This article aims to provide the current status of the pathological classification, staging, and grading. Selective literature search in PubMed and Medline RESULTS: The vast majority of disseminated peritoneal mucinous diseases that correspond to the clinical presentation of pseudomyxoma peritonei (PMP) arise from mucinous tumors of the vermiform appendix. Here are to be distinguished: 1)low-grade appendiceal mucinous neoplasms (LAMN), 2)(very rare) high-grade appendiceal mucinous neoplasms (HAMN), 3)mucinous adenocarcinoma without signet ring cells (G2) and 4)mucinous adenocarcinoma with signet ring cells or signet ring cell carcinoma (G3). Other primary tumors only rarely induce PMP. Terms such as mucocele or mucinous cystadenoma of the appendix correspond to LAMN and should no longer be used. Prognostic distinctions are further made between low-grade PMP, which usually arises from LAMN, and the prognostically less favorable high-grade PMP, which usually arises from mucinous/signet ring cell adenocarcinoma or the rare HAMN. Disseminated peritoneal mucinous disease/PMP must then be further distinguished from prognostically excellent local mucin formation of the peri-appendix region. The currently valid nomenclature, as it has emerged from consensus meetings and in parts has also found its way into the current WHO 2019, has significantly contributed to the fact that the prognosis of patients today can be better estimated and effective forms of treatment could be developed.

Incidental finding of synchronous neuroendocrine tumor of appendix and rectal adenocarcinoma. A case report and literature review

Abstract Objectives Colorectal cancers represent the predominant malignancies affecting the gastrointestinal tract. In contrast, appendiceal tumors occur less frequently. Among them, appendiceal neuroendocrine tumors (ANETs) account for approximately 50 % of cases and are often detected incidentally during unrelated procedures. Case presentation We present an extremely rare case of an incidental neuroendocrine tumor of the appendix discovered during surgery for rectal adenocarcinoma and provide a review of the relevant literature. A 70-year-old patient with radiologically and endoscopically diagnosed rectal cancer underwent a low anterior resection with total mesorectal excision and a temporary diverting ileostomy following preoperative chemotherapy and radiation therapy. Intraoperatively, a small appendiceal mass was discovered and excised. Histopathological examination confirmed a well-differentiated NET of the appendix measuring 0.4 cm in diameter. Conclusions This case report emphasizes the importance of thorough intraoperative examination during colorectal surgeries and highlights the need for increased awareness of appendiceal NETs among surgeons.

Mucinous Neoplasm of Appendix: Treatment

Mucinous Neoplasm of Appendix: Treatment