scholarly journals Clinicopathological Features of Low-Grade Appendiceal Mucinous Neoplasms Confined to the Appendix

2021 ◽  
Vol 11 ◽  
Author(s):  
Yiyan Lu ◽  
Fang Li ◽  
Ruiqing Ma ◽  
Lan Fang ◽  
Changhai Qi

ObjectiveTo investigate the clinicopathological features and follow-up of low-grade appendiceal mucinous neoplasms (LAMNs) confined to the appendix.MethodsThe clinicopathological features, pathological primary tumor (pT) staging and follow-up of 22 patients with LAMNs confined to the appendix were analyzed retrospectively.ResultsOf 22 patients with LAMNs, 14 were pTis (eight pTism and six pTisf), six were pT3, and two were pT4a. The appendiceal diameter was significantly larger for pTisf than for pTism. The interval between first symptoms and surgery was longer for pTisf than for pTism, but not significantly different. No significant differences were found between the pT stages and appendiceal diameter or in the interval between the first symptoms and surgery. Pathomorphologically, the epithelial structures were mainly flat (100%), undulating or scalloped (82%); a few showed filiform villous hyperplasia (46%), and seven (32%) had serrated lesions in the background. Diverticula may be associated with LAMNs, and the location of acellular mucin caused by diverticula affected the pT stage of the LAMNs. The immunohistochemistry information showed the same pattern with cytokeratin 7 (CK7) negative, cytokeratin 20 (CK20) positive and caudal type homeobox 2 (CDX-2) positive. No lymph node metastasis was found. The lack of treatment guidelines for LAMNs confined to the appendix and different acceptances of patients of preventive intervention led to varied clinical treatments. However, we found no short-term benefits of prophylactic extended resection or hyperthermic intraperitoneal chemotherapy.ConclusionLAMNs confined to the appendix are rare and must be differentiated from serrated lesions and diverticula. LAMNs with different pT stages have inert biological behavior. Determining the long-term effects of preventive treatment on survival and recurrence requires more data and a longer follow-up.

2019 ◽  
Vol 28 (3) ◽  
pp. 252-258 ◽  
Author(s):  
Mary Wong ◽  
Brad Barrows ◽  
Alexandra Gangi ◽  
Stacey Kim ◽  
Richard B. Mertens ◽  
...  

Background. In this single-institution study, we applied the current (eighth edition) American Joint Committee on Cancer pathologic staging criteria to 64 low-grade mucinous neoplasms of the appendix (LAMNs), examined their histopathologic features, and studied the patients’ clinical outcomes. Design. Sixty-four LAMNs, with a median follow-up of 52 months, were reviewed. Results. The distribution of pathologic stages was pTis (n = 39), pT3 (n = 1), pT4a (n = 5), pT4aM1a (n = 8), and pT4aM1b (n = 11). Recurrence was observed in only 2 patients (both with pT4aM1b disease), one of whom died of disease. All remaining patients were disease-free after a median clinical follow-up of 60 months. Conclusions. Our study confirms that pTis LAMNs have an excellent prognosis and suggests that pT4a and pT4aM1a LAMNs may also have a low risk of developing progressive disease.


2020 ◽  
Author(s):  
xinyu ren ◽  
Yin Cheng ◽  
Tao Lu ◽  
Junliang Lu ◽  
Yan Wu ◽  
...  

Abstract AIM Low grade appendiceal mucinous neoplasm (LAMN) and serrated lesions are sometimes hard to differentiate from morphology. We try to characterize them from the immunohistochemical and molecular perspective and to reflect the difference between them. METHODS 25 appendix neoplasm including 13 LAMNs, 12 serrated lesions were selected from January 2013 to December 2014. Immunohistochemical analyses for cytokeratin 20, MUC6, MUC5AC, MUC1, Ki-67, P53 and mismatch repair (MMR) proteins including MLH1, PMS-2, MSH-6, MSH-2 were performed. Microsatellite instability (MSI) status was also evaluated. Besides, we detect K-RAS, B-RAF and GNAS gene mutation status of these lesions. RESULTS Immunochemically, 83.3% serrated lesions showed scattered CK20 staining in the deep crypt, which was less so for LAMNs. As for mucin expression, MUC5AC had slightly higher positive rate in LAMNs and than in serrated lesions.MUC1 was significantly higher expressed in LAMNs than in serrated lesions. 46.1% LAMNs have P53 expression in deep crypt, while P53 was negative in the deep crypt of serrated lesions. 58.3% serrated lesions had deficient MMR protein expresion pattern compared to 23.1% of LAMNs. B-RAF mutation was detected in 3 cases, all were serrated lesions. K-ras and GNAS mutation was detected in both LAMNs and serrated lesions. CONCLUSION Immunohistochemical panel comprising markers such as CK20, MUC5AC, MUC1, Ki-67 and P53, with genotyping covering hotspots of the KRAS, BRAF and GNAS genes can help the differential diagnosis of low grade appendix neoplasm.


2010 ◽  
Vol 28 (1) ◽  
pp. E12 ◽  
Author(s):  
Wolfgang K. Pfisterer ◽  
Ronald A. Nieman ◽  
Adrienne C. Scheck ◽  
Stephen W. Coons ◽  
Robert F. Spetzler ◽  
...  

Object The goal in this study was to determine if proton (1H) MR spectroscopy can differentiate meningioma grade and is associated with interpretations of biological behavior; the study was performed using ex vivo high-resolution spectra indicating metabolic characteristics. Methods Sixty-eight resected tissue samples of meningiomas were examined using ex vivo 1H MR spectroscopy. Of these meningiomas, 46 were WHO Grade I, 14 were WHO Grade II, and 8 were WHO Grade III. Fifty-nine were primary meningiomas and 9 were recurrences. Invasion of adjacent tissue (dura mater, bone, venous sinus, brain) was found in 32 cases. Thirty-nine meningiomas did not rapidly recur (as defined by expansion on MR imaging within a 5-year follow-up period), whereas rapid recurrence was confirmed in 24 meningiomas, and follow-up status was unknown in 5 cases. Results The absolute concentrations of total alanine and creatine were decreased in high-grade compared with low-grade meningiomas, as was the ratio of glycine to alanine (all p < 0.05). Additionally, alanine and the glycine/alanine ratio distinguished between primary and recurrent meningiomas (all p < 0.05). Finally, the absolute concentrations of alanine and creatine, and the glycine/alanine and choline/glutamate ratios were associated with rapid recurrence (p < 0.05). Conclusions . These data indicate that meningioma tissue can be characterized by metabolic parameters that are not typically identified by histopathological analysis alone. Creatine, glycine, and alanine may be used as markers of meningioma grade, recurrence, and the likelihood of rapid recurrence. These data validate a previous study of a separate group of Grade I meningiomas.


Author(s):  
kathleen McGrath ◽  
Mairéad Casserly ◽  
Freda O’mara ◽  
Jurgen Mulsow ◽  
Conor Shields ◽  
...  

Abstract Objective To improve the number of patients receiving annual computed tomography (CT) scan and tumour markers, who are diagnosed with low-grade mucinous neoplasms (LAMN). Design A pre-/post-intervention design was employed using Lean Six Sigma methods to identify gaps in the screening system and to develop and implement solutions for a more robust, auditable screening programme. Setting The patients diagnosed with LAMN of the appendix referred to the acute hospital and are enrolled in the screening service. Participants Consultant colorectal surgeons, cancer nurse specialist, colorectal medical team and quality improvement staff. Interventions Diagnostic tools identified gaps in the current process. A set of improvements were implemented to standardize the pathway for referral and surveillance of patients, provide information on the condition and treatment and standardize and track information received by patients and their referring hospital. Main Outcome Measure(s) Pre and post-intervention outcome measures were taken for the number of patients who receive an annual CT of thoracic, abdomen and peritoneum and tumour markers and number of patients who receive information and contact details. Results At baseline, of the 28 patients that met the inclusion criteria only 61% had a correct follow-up. Following the implementation of improvements, 78% of patients had correct follow-up and 90% had received information. Conclusions Gaps in the current cancer screening system were identified and improvements implemented a reduced number of patients having an incorrect follow-up. Findings are applicable across all precancerous screening systems irrespective of the type of malignancy. The methods used empowered patients and fostered an interdisciplinary team approach to care.


2019 ◽  
Vol 07 (09) ◽  
pp. E1130-E1134
Author(s):  
Jean-Michel Gonzalez ◽  
Diane Lorenzo ◽  
Jean-Philippe Ratone ◽  
Adrian Culetto ◽  
Frédérique Maire ◽  
...  

Abstract Introduction Branch duct intraductal papillary mucinous neoplasms (BD-IPMNs) require follow-up to detect worrisome features (WF). Data are missing about endoscopic pancreatic sphincterotomy (PS) for symptomatic IPMN. Patients and methods This was a retrospective multicenter study in four expert centers. Patients treated with endoscopic PS for symptomatic (painful) BD-IPMN without WF were included. Age, sex, follow-up time, characteristics of IPMNs and endoscopic retrograde cholangiopancreatographies (ERCPs), and indications for surgery were recorded. Results In total, 21 patients were included (median age 68 years, range 45 – 87 years). The median number of cysts was 2 (range 1 – 10), located in the head (59 %), body/tail (17 %), or multifocal (24 %). ERCP including PS was completed in all of the cases, with biliary sphincterotomy in 33 %. Clinical efficacy after one session was 81 % (17/21). Among the failures, one had a second successful PS and three were operated. The final efficacy was 86 % (18/21). Seven patients were operated after a mean of 19 months: four for WF, three for pain. The histopathology showed four low grade dysplasia, one high grade dysplasia, and two no dysplasia. No adenocarcinoma occurred during a follow-up of 99 months (range 14 – 276 months). Conclusions Endoscopic PS for symptomatic IPMN without WF is effective in more than 80 % of cases, without increasing the risk for adenocarcinoma.


Author(s):  
Giada Munari ◽  
Gianluca Businello ◽  
Paola Mattiolo ◽  
Gianmaria Pennelli ◽  
Marta Sbaraglia ◽  
...  

Abstract Purpose Non-neuroendocrine neoplasms of the appendix are a phenotypically heterogeneous group of lesions; a comprehensive molecular characterization of these tumors is still lacking. Methods A total of 52 samples taken from 49 patients was evaluated: 18 sessile serrated lesions (SSL; 3 with dysplasia), 2 high-grade tubular adenomas, 1 tubulo-villous adenoma,1 hyperplastic polyp, 18 low-grade appendiceal mucinous neoplasms (LAMN), 3 high-grade appendiceal mucinous neoplasms (HAMN) and 9 mucinous adenocarcinomas. Hotspot mutational profiling of the RNF43, SMAD4, KRAS, NRAS, BRAF and PIK3CA genes was performed. Expression of p53, MLH1, PMS2, MSH2, and MSH6 was evaluated by immunohistochemistry. Results KRAS was the most frequently mutated gene (53.9% of cases), followed by RNF43 (15.4%), and BRAF (13.5%). In particular: KRAS was mutated in 44.4% of adenocarcinomas, 66.7% of HAMNs, 61.1% of LAMNs, 53.3% of SSL without dysplasia and in 66.7% of SSL with dysplasia; RNF43 was mutated in 33.3% of adenocarcinomas, 66.7% of HAMNs, 11.1% of LAMNs and in 6.7% of SSL without dysplasia; BRAF was mutated in 11.1% of adenocarcinomas, 26.7% of SSL without dysplasia and in 5.6% of LAMNs. Only a case of high-grade tubular adenoma showed mismatch repair deficiency, while immunohistochemical expression of p53 was altered in 21.1% of cases. Conclusions The histological phenotypic similarities between appendicular mucinous lesions and serrated colon lesions do not reflect a similar genetic landscape. Mismatch repair deficiency is a rare event during appendiceal mucinous carcinogenesis.


2021 ◽  
pp. 216770262097977
Author(s):  
Kristof Hoorelbeke ◽  
Nathan Van den Bergh ◽  
Rudi De Raedt ◽  
Marieke Wichers ◽  
Ernst H. W. Koster

Previous studies suggest that cognitive control training (CCT) shows potential as a preventive intervention for depression. In this study, the first to examine long-term preventive effects of CCT, we examined effects on (a) task-specific cognitive transfer at 1-year follow-up, (b) recurrence of depression, and (c) functioning over the course of a year. Each of 92 remitted depressed patients were randomly assigned to a CCT condition or an active control condition (ACT). Effects of training were monitored using weekly assessments of emotion regulation, cognitive complaints, depressive symptoms, and resilience (brief weekly questionnaire). At 1-year follow-up, participants completed a structured clinical interview, cognitive transfer task, and questionnaires. We observed task-specific cognitive transfer ( p < .001, d = 1.23) and lower recurrence rates in the CCT condition ( p = .04; odds ratio = 0.38). However, no long-term beneficial effects of training were observed on the weekly ratings of functioning, and groups did not differ in performance on the self-report questionnaires at 1-year follow-up.


2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
A Alamassi

Abstract Introduction Appendiceal torsion is an uncommon precipitating factor for acute appendicitis. It is found to be in association with appendiceal mucocele. Case presentation A 38-year-old lady presented with acute peritonitis. CT Scan showed tubular fluid collection. This was suggested to be a torsion/volvulus of the appendix. The intraoperative finding were a distended appendix filled with mucus and twisted 720° at the base. Appendectomy was done. The histopathology showed low grade mucinus neoplasia. Discussion: Approximately, 50% of mucoceles are asymptomatic and incidentally discovered, whilst 40% present with acute appendicitis. There are two suggested pathogenesis: one being an obstructive mechanism causing high pressure in the appendix, resulting into gross mucinous accumulation and second being a neoplastic process. Appendiceal mucinous neoplasms are classified into LAMN, HAMN and mucinous appendiceal adenocarcinoma. It is important to differentiate between the pathological subtypes as the risk of occurrence of PMP may vary according to that. This would affect the treatment modalities; follow up planning, prognosis and risk of recurrence. It is important that extra precautions should be taken whilst handling the specimens intraoperatively to avoid spillage of the content as that might lead to implantation of neoplastic cells in the peritoneum and that might affect the overall survival rate.


2017 ◽  
Vol 24 (7) ◽  
pp. 1778-1782 ◽  
Author(s):  
Catarina Tiselius ◽  
Csaba Kindler ◽  
Jayant Shetye ◽  
Henry Letocha ◽  
Kenneth Smedh

2021 ◽  
Author(s):  
Yiyan Lu ◽  
Changhai Qi ◽  
Hongbin Xu ◽  
Mulan Jin

Abstract Purpose: To identify clinicopathological features for the differential diagnosis of appendiceal serrated lesions and polyps (SPs) and low-grade appendiceal mucinous neoplasm (LAMN) for the purpose of avoiding over‐diagnosis.Methods: Clinical data and pathological features of 66 patients with SPs diagnosed at the Aerospace Center Hospital between January 2013 and January 2021 were collected and compared to 22 cases of LAMN.Results: SPs, compared with LAMN, are likely to be associated with acute inflammation (SPs 53.0% vs. LAMN 18.2%), and may be located in the appendix partly, although with smaller diameter (average 9.6 vs. 27.2 mm); epithelial structures of serrated (100% vs. 22.7%) and filiform villous (47.0% vs. 18.2%) were often found in SPs. SPs occasionally show attenuated or flattened morphology (16.7% vs. 100%) and undulating or scalloped (7.6% vs. 40.9%) structures, and can also be accompanied by diverticulum (18.2% vs. 18.2%) and acellular mucin in the appendiceal wall (16.7% vs. 54.5%), which causes confusion with LAMN. The key point of the differential diagnosis is to observe whether the muscularis mucosa exists (loss, 0% vs. 100%) and fibrosis of the appendiceal wall (0% vs. 100%). SMA immunohistochemistry can assist in the diagnosis. Calcification is also indicative of LAMN.Conclusions: The epithelial structure of SPs can appear flattened and focally scalloped, and can be accompanied by mucin in the appendiceal wall, which may appear as complex lesions, easily over-diagnosed as LAMN. Key differential diagnostic features are identifying the structure of lamina propria, determining whether the muscularis mucosa exists, and whether the appendiceal wall is fibrotic.


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