Low-grade Appendiceal Mucinous Neoplasm Research Articles

An Incidental Finding of Low-Grade Appendiceal Mucinous Neoplasm in a Case of Borderline Brenner Tumor of the Ovary

An Incidental Finding of Low-Grade Appendiceal Mucinous Neoplasm in a Case of Borderline Brenner Tumor of the Ovary

Critical reappraisal of prognostic indicators for 949 mucinous appendiceal neoplasm patients.

The standard of care for treatment of an appendiceal mucinous neoplasm with peritoneal dissemination is cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC). These two treatments are combined in the operating room. A crucial requirement for benefit long-term is proper patient selection. Clinical and histopathologic prognostic indicators are used, along with the patient's fitness for surgery, to select patients to receive CRS and HIPEC. This study seeks to identify the reliable prognostic indicators for four different groups of patients. They are (1) the low-grade appendiceal mucinous neoplasms (LAMN) with a complete CRS, (2) the mucinous appendiceal adenocarcinomas (MACA) with complete CRS, (3) MACA with lymph node metastases (MACA-LN) with complete CRS, and (4) all histologic subtypes with incomplete cytoreduction. The prognostic indicators were evaluated for their impact on overall survival in these four groups of patients. The completeness of cytoreduction (CC) score statistically significantly showed survival differences in all three histologic subtypes. The peritoneal cancer index (PCI) showed significance with LAMN and MACA-LN but not with MACA and not with incomplete CRS. The prior surgical score (PSS) was a prognostic indicator that predicted the outcome with LAMN, MACA-LN, and incomplete CRS patients but not with the MACA group. Patients who were symptomatic or who had extensive systemic chemotherapy before CRS had a significantly reduced survival. The utility of prognostic indicators varied greatly within our four different groups of appendiceal mucinous neoplasms. CC score was always a reliable prognosticator. Surprisingly, PCI was not.

What is the optimum post treatment surveillance imaging protocol for low grade appendiceal mucinous neoplasms and pseudomyxoma peritoneii?

Appendiceal mucinous neoplasms are rare and can be associated with the development of disseminated peritoneal disease known as pseudomyxoma peritonei (PMP). Mucinous tumours identified on appendicectomy are therefore followed up to assess for recurrence and the development of PMP. In additional, individuals who initially present with PMP who are treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) are followed up to assess for recurrence. However, despite the concerted efforts of multiple expert groups, the optimal imaging follow-up protocol is yet to be established. The purpose of this paper is to review the available evidence for imaging surveillance in these populations to identify the optimum post-resection imaging follow-up protocol.

Closure of a Gastropleural Fistula Using Advanced Endoscopy.

Gastropleural fistulas are rare complications with significant mortality and morbidity. There are limited reports on the successful management of gastropleural fistulas with advanced endoscopic procedures. The following case of a 75-year-old woman with a history of recurrent pseudomyxoma peritonei secondary to ruptured low-grade appendiceal mucinous neoplasm status post cytoreductive surgery highlights the successful treatment of a gastropleural fistula with endoscopic suturing.

Low-grade appendiceal mucinous neoplasm; a subtle presentation during colonoscopy.

Low-grade appendiceal mucinous neoplasm; a subtle presentation during colonoscopy.

Beyond acute appendicitis: a single-institution experience of unexpected pathology findings after 989 consecutive emergency appendectomy.

Appendiceal neoplasms (ANs) are rare, with an estimated incidence of around 1%: neuroendocrine tumours (NETs) and low-grade appendiceal mucinous neoplasms (LAMNs) comprise most cases. Most tumours are cured by appendectomy alone, although some require right hemicolectomy and intra-operative chemotherapy. The aim of the present study is to evaluate our institution's experience in terms of the prevalence of AN, their histological types, treatment and outcomes in adult patients undergoing emergency appendectomy. Single-centre retrospective cohort analysis of patients treated for acute appendicitis at a large academic medical centre. Patients with a diagnosis of acute appendicitis (AA) where further compared with patients with acute appendicitis and a histologically confirmed diagnosis of appendiceal neoplasm (AN). A diagnosis of acute appendicitis was made in 1200 patients. Of these, 989 patients underwent emergency appendectomy. The overall incidence of appendiceal neoplasm was 9.3% (92 patients). AN rate increased with increasing age. Patients under the age of 30 had a 3.8% (14/367 patients) rate of occult neoplasm, whereas patients between 40 and 89years and older had a 13.0% rate of neoplasm. No difference was found in clinical presentations and type of approach while we found a lower complicated appendicitis rate in the AN group. ANs are less rare with respect to the literature; however, clinically, there are no specific signs of suspicious and simple appendicectomy appears to be curative in most cases. However, age plays an important role; older patients are at higher risk for AN. ANs still challenge the non-operative management concept introduced into the surgical literature.

Low-grade Appendiceal Mucinous Neoplasms: A Case Report

Low-grade Appendiceal Mucinous Neoplasms: A Case Report

Appendicular Mucocele Complicated by Peritoneal Pseudomyxoma

Appendiceal mucocele (AM) or Appendiceal mucinous neoplasms is a rare but potentially dangerous condition. In the latter, a spontaneous or iatrogenic rupture of the mucocele can lead to mucinous intraperitoneal ascites, a syndrome known as pseudomyxoma peritonei. We present the case of a 76-year-old female patient who reported right iliac fossa pain with RIF tenderness on clinical examination, and underwent an abdominal CT scan showing an appendiceal mucocele associated with a small peritoneal effusion suspecting a ruptured mucocele, related to peritoneal pseudomyxoma. The patient underwent appendectomy with peritoneal lavage and aspiration of all mucin found. Anatomopathological analysis confirmed the diagnosis of appendiceal mucocele (a low-grade appendiceal mucinous neoplasm).

Incidental findings during donor liver assessment: Single center experience

Intraoperative consultation of donor liver is an important part of transplant evaluation and determination of liver eligibility. In this study, we describe incidental pathologic findings discovered during the pretransplant evaluation of liver donors in our Institution from 1/2010 to 12/2022. During this 13-year period 369 intraoperative consultations from 262 liver donors were performed. Of those cases, incidental findings were identified in 22 cases (5.9 %) from 19 donors (7.3 %); two donors had more than one lesion. The median age of this subset of patients was 53 years (range: 18–70) and females predominated (63 %). Sixteen of the donors had abnormal findings in the liver: 6 bile duct hamartoma (BDH), 5 hyalinized nodule with Histoplasma capsulatum, 5 focal nodular hyperplasia (FNH), 2 bile duct adenomas (BDA), 1 biliary cyst and 1 hemangioma. One donor had both FNH and a BDH. One BDH and 1 BDA case was misdiagnosed as malignancy during the frozen section evaluation. Three donors had extrahepatic pathologies: a pancreatic tail schwannoma, a low-grade appendiceal mucinous neoplasm, and a lymph node with metastatic endometrial endometrioid adenocarcinoma. Of the 19 livers, the final organ disposition was available for 9: 6 were transplanted (67 %) and 3 were discarded (33 %). Two of the 3 discarded organs were misdiagnosed BDH and BDA cases, and one was incorrectly reported as having 90 % microvesicular steatosis during the frozen assessment. We present the clinicopathologic characteristics of liver donors with incidental findings during the pre-transplant evaluation which could lead to unwarranted graft dismissal if misdiagnosed. Additionally, incidental fungal infections can have implications for immunosuppressive therapy and the decision to use or reject the graft.

Low-grade Appendiceal Mucinous Neoplasm in a Middle-aged Female: A Case Report

Mucinous Appendiceal Neoplasms (MANs) are rare tumours where more than 50% of the tumour volume is composed of extracellular mucin. Low-grade Appendiceal Mucinous Neoplasm (LAMN) is a rare condition with symptoms that vary depending on clinical manifestations. It can manifest as an unruptured mucin-filled appendix, transmural invasion of the primary tumour, or present with peritoneal metastases post-rupture. The prognosis of LAMN depends on the presence or absence of neoplastic epithelium outside the appendix. Here, the authors describe a case of 35-year-old female who visited the OPD of Emergency Medicine with only complain of pain in abdomen for three days. She exhibited right iliac fossa tenderness with no guarding during the abdominal examination. She underwent an open appendectomy and was diagnosed with LAMN upon histopathological examination. She was advised to follow up after six months post-discharge to monitor for any metastatic spread. Due to its malignant potential, mucinous lesions of the appendix are uncommon yet significant entities. They are more prevalent in women and can range from mucinous adenocarcinomas to straightforward retention cysts. This case illustrates the need for extreme caution when dealing with appendiceal tumours and the importance of selecting the appropriate course of action, be it surgical or medical.

Low-grade Appendiceal Mucinous Neoplasm Masquerading as Acute Appendicitis: A Case Report

Appendiceal mucinous neoplasms, particularly Low-grade Appendiceal Mucinous Neoplasms (LAMN), are rare but important diagnostic entities as they are potential causes of a surgical abdomen. The clinical manifestations of this lesion are obscure and ill-defined, and these lesions are commonly misdiagnosed as acute appendicitis, adnexal masses, or retroperitoneal tumours. This lesion can rupture and seed mucin and neoplastic epithelium into the peritoneum, leading to Pseudomyxoma Peritonei (PMP), a serious complication with a high morbidity and mortality rate. Therefore, timely identification and treatment of LAMN are crucial for reducing the risk of PMP and improving prognosis and outcomes. Ultrasonography (USG) and Computed Tomography (CT) scans are useful methods for diagnosis; however, the diagnosis is often incidental or found intraoperatively during resection for suspected acute appendicitis. Clinical awareness of the misleading presentations of LAMN should be present in cases of a surgical acute abdomen to prevent performing a dissimilar treatment intervention. Considering the rarity of this lesion and its varied presentation, it is important to study and document this type of neoplasia in the literature. Hereby, authors report a rare case of LAMN in a 33-year-old male patient with a primary diagnosis of acute appendicitis. This case highlights the importance of having a high index of clinical suspicion of appendiceal malignancy and mucocele rupture in patients planned for appendectomy. This also emphasises the fact that all excised appendicectomy tissues should be sent for histopathological examination as they can harbor pathological changes like LAMN.

Two cases of low-grade appendiceal mucinous neoplasm (LAMN) and LAMN with appendiceal endometriosis that were difficult to distinguish from right ovarian and fallopian tube tumors before laparoscopic surgery

Two cases of low-grade appendiceal mucinous neoplasm (LAMN) and LAMN with appendiceal endometriosis that were difficult to distinguish from right ovarian and fallopian tube tumors before laparoscopic surgery

Long-term Postoperative Follow-up for 60 Months in Three Patients with Low-grade Appendiceal Mucinous Neoplasm

Long-term Postoperative Follow-up for 60 Months in Three Patients with Low-grade Appendiceal Mucinous Neoplasm

Low-grade Appendiceal Mucinous Neoplasm with Appendiceal Reduction and Re-expansion Over a Two-year Period: A Case Report.

A 71-year-old woman was found to have submucosal tumor-like lesion on colonoscopy (CS) before gastric surgery, and computed tomography (CT) showed a 12-mm structure at the base of the appendix. The lesion could not be clearly detected on CT nine months later, but it had enlarged again on CT one year later; therefore, CS and endoscopic ultrasound (EUS) were performed. The lesion was determined to be cystic with viscous contents, and laparoscopic appendicectomy was performed. This is the first report of low-grade appendiceal mucinous neoplasm (LAMN) diagnosed by a histopathologic examination of a resected specimen showing shrinkage and re-expansion of the appendix.

Unraveling the Unusual Pathological Findings of Appendiceal Lesions: A Cross-sectional Study from Tertiary Care Centre in Chengalpattu, Tamil Nadu, India

Introduction: The appendix, once considered a vestigial structure, is now recognised as a site of diverse pathologies beyond simple appendicitis through a systematic analysis of published literature and case studies. The diagnosis of appendiceal lesions is still a clinical phenomenon and a constant struggle. The gold standard for diagnosing appendiceal lesions, however, is meticulous histopathological examination. Aim: To determine the histopathological distribution of all the appendiceal lesions and to identify the rare and unusual histopathological findings in appendectomy specimens received at a tertiary care Centre. Materials and Methods: A cross-sectional study was conducted in the Department of Pathology at Karpaga Vinayaga Institute of Medical Sciences and Research Centre, Chengalpattu, Tamil Nadu, India for a duration of 10 years, from August 2013 to August 2023. From the histopathological records of 832 appendectomies, 40 specimens with unusual histopathological findings were identified and analysed in this study. Relevant recorded data from all the appendiceal specimens reported during the study period, with special reference to age, sex, and histopathological findings, were retrieved and evaluated retrospectively. Corresponding slides and specimens were reviewed. The data were presented in the form of frequency tables and percentages. The analysis was done using Statistical Package for Social Sciences (SPSS) software version 20.0. Results: Out of 832 appendectomy specimens, there were 485 (58.29%) cases of acute appendicitis, 244 (29.33%) cases of chronic appendicitis, 59 (7.09%) cases of subacute appendicitis, and 4 (0.48%) cases of gangrenous appendicitis. Unusual histopathology findings were noted in 40 cases (4.81%). These include 17 cases of appendiceal neuroma, eight cases of Xanthogranulomatous appendicitis, seven cases of Enterobius vermicularis, three cases of Low-grade Appendiceal Mucinous Neoplasm (LAMN), one case of appendiceal Neuroendocrine Tumour (NET), one case of Goblet Cell Adenocarcinoma (GCA), one case of metastatic squamous cell carcinomatous deposit in the appendix, one case of urothelial rests in the appendix, and one case of appendiceal lipoma. Conclusion: In this study, 4.81% of patients had atypical appendiceal lesions. A complete histopathological examination of appendectomy specimens increases the chances of discovering unusual lesions of the appendix. Ultimately, this comprehensive analysis will contribute to future improvements in clinical management and prognostication, fostering better patient outcomes.

Active surveillance for low-grade appendiceal mucinous neoplasm (LAMN).

Due to the scarcity of low-grade appendiceal mucinous neoplasm (LAMN), there is an absence of systematized guidelines concerning its management, especially after incidental finding on an appendiceal specimen. In this study, we evaluate the active surveillance (AS) strategy adopted for a series of patients diagnosed with LAMN on resection specimens who were considered to have a low risk of pseudomyxoma progression. Thirty patients were included between April 2014 and July 2021, with a female majority and a median follow-up period of 3.1 years. The inclusion criteria were as follows: LAMN diagnosis on appendiceal specimens, confirmed in an expert center, limited extra-appendiceal mucin resected and localized around the appendix, normal biology (CEA, CA199, CA125) and normal abdominopelvic MRI. AS included physical exam (trocar scar), biology and MRI, 6months postoperatively, then yearly for 10 years. As an initial surgery, 77 % had an appendectomy as their initial intervention, 17 % had a cecectomy, and 6 % had a right colectomy. After follow-up, 87 % of patients showed no sign of disease progression by MRI, while 13 % progressed to PMP. MRI performed in the first postoperative year predicted the disease prognosis in 97 % of patients. The AS strategy, based on MRI, is a valid option after incidental LAMN diagnosis.

Goblet Cell Adenocarcinoma in the Background of a Ruptured Appendiceal Diverticulum Mimicking Low-grade Appendiceal Mucinous Neoplasm: A Case Report and Literature Review

Goblet Cell Adenocarcinoma in the Background of a Ruptured Appendiceal Diverticulum Mimicking Low-grade Appendiceal Mucinous Neoplasm: A Case Report and Literature Review

Non-inflamed appendix with LAMN causing closed-loop small bowel obstruction

Introduction: Low grade appendiceal mucinous neoplasms are generally encountered incidentally in appendicectomies for suspected appendicitis. This case is a documentation of a rare occurrence of LAMN presenting with small bowel obstruction. Case Report: An 83-year-old male presented to the Emergency Department with a three day history of nausea, vomiting, and obstipation and profoundly dehydrated. Computed tomography revealed a high grade small bowel obstruction with 2 transition points in the right iliac fossa, concerning for closed loop obstruction, necessitating an urgent laparotomy. Conclusion: While acute appendicitis is a common cause of emergency abdominal pathology, when coupled with small bowel obstruction, a neoplastic cause should be considered as part of the differential diagnosis.

Long term survival outcomes of surgery combined with hyperthermic intraperitoneal chemotherapy for perforated low-grade appendiceal mucinous neoplasms: Amulticenter retrospective study.

Low-grade appendiceal mucinous neoplasms (LAMN) are very rare, accounting for approximately 0.2%-0.5% of gastrointestinal tumors. We conducted a multicenter retrospective study to explore the impact of different surgical procedures combined with HIPEC on the short-term outcomes and long-term survival of patients. We retrospectively analyzed the clinicopathological data of 91 LAMN perforation patients from 9 teaching hospitals over a 10-year period, and divided them into HIPEC group and non-HIPEC group based on whether or not underwent HIPEC. Of the 91 patients with LAMN, 52 were in the HIPEC group and 39 in the non-HIPEC group. The Kaplan-Meier method predicted that 52 patients in the HIPEC group had 5- and 10-year overall survival rates of 82.7% and 76.9%, respectively, compared with predicted survival rates of 51.3% and 46.2% for the 39 patients in the non-HIPEC group, with a statistically significant difference between the two groups (χ2=10.622, p=0.001; χ2=10.995, p=0.001). Compared to the 5-year and 10-year relapse-free survival rates of 75.0% and 65.4% in the HIPEC group, respectively, the 5-year and 10-year relapse-free survival rates of 48.7% and 46.2% in the non-HIPEC group were significant different between the two outcomes (χ2=8.063, p=0.005; χ2=6.775, p=0.009). The incidence of postoperative electrolyte disturbances and hypoalbuminemia was significantly higher in the HIPEC group than in the non-HIPEC group (p=0.023; p=0.044). This study shows that surgery combined with HIPEC can significantly improve 5-year and 10-year overall survival rates and relapse-free survival rates of LAMN perforation patients, without affecting their short-term clinical outcomes.

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.