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The Oncologist Sep 2017Appendiceal mucinous neoplasms (AMNs) are a rare and heterogeneous disease for which clinical management is challenging. We aim to review the literature regarding... (Review)
Review
OBJECTIVE
Appendiceal mucinous neoplasms (AMNs) are a rare and heterogeneous disease for which clinical management is challenging. We aim to review the literature regarding modalities of treatment to guide the management of AMNs.
METHODS AND REVIEW CRITERIA
We conducted a PubMed search in February 2016 for English-language publications, using the terms "appendiceal," "appendix," "carcinoma," "cancer," "mucinous," "treatment," "genes," "target," "genomic," and terms listed in the articles' subheadings. Published reports and abstracts from the American Society of Clinical Oncology meetings were also searched.
RESULTS
In this review, we summarize current data and controversies in AMN classification, clinical presentation, molecular alterations, treatment outcomes with regard to cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), and the role of systemic chemotherapy.
CONCLUSION
Appendiceal mucinous neoplasms are a heterogeneous group of tumors with a rising incidence. Treatment is based on stage and histology. Low-grade tumors are treated surgically with resection of the primary site in early stage disease, or peritoneal debulking and HIPEC in patients with advanced stage disease. Treatment of high-grade tumors requires further prospective trials, and options include debulking surgery and HIPEC with or without preoperative chemotherapy. Trials evaluating novel therapies based on the molecular profiling of AMN tumors are needed to evaluate therapeutic options in patients who are not surgical candidates.
IMPLICATIONS FOR PRACTICE
This review provides a reference to guide gastroenterologists, pathologists, surgeons, and oncologists in the management of appendiceal mucinous neoplasms (AMNs), a rare and heterogeneous disease with no consensus on histologic classification or guidelines for treatment algorithms. This review summarizes all AMN classifications and proposes a treatment algorithm based on stage and histology of disease.
Topics: Adenocarcinoma, Mucinous; Antineoplastic Combined Chemotherapy Protocols; Appendiceal Neoplasms; Appendix; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Neoplasm Staging; Practice Guidelines as Topic; Rare Diseases; Treatment Outcome
PubMed: 28663356
DOI: 10.1634/theoncologist.2017-0081 -
Deutsches Arzteblatt International Aug 2023Neoplasms of the vermiform appendix are rare. They comprise a heterogeneous group of entities requiring differentkinds of treatment. (Review)
Review
BACKGROUND
Neoplasms of the vermiform appendix are rare. They comprise a heterogeneous group of entities requiring differentkinds of treatment.
METHODS
This review is based on publications retrieved by a selective literature search in the PubMed, Embase, and Cochranedatabases.
RESULTS
0.5% of all tumors of the gastrointestinal tract arise in the appendix. Their treatment depends on their histopathologicalclassification and tumor stage. The mucosal epithelium gives rise to adenomas, sessile serrated lesions, adenocarcinomas,goblet-cell adenocarcinomas, and mucinous neoplasms. Neuroendocrine neoplasms originate in neuroectodermal tissue. Adenomasof the appendix can usually be definitively treated by appendectomy. Mucinous neoplasms, depending on their tumorstage, may require additional cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemoperfusion (HIPEC). Adeno -carcinomas and goblet-cell adenocarcinomas can metastasize via the lymphatic vessels and the bloodstream and should thereforebe treated by oncological right hemicolectomy. Approximately 80% of neuroendocrine tumors are less than 1 cm in diameterwhen diagnosed and can therefore be adequately treated by appendectomy; right hemicolectomy is recommended if the patienthas risk factors for metastasis via the lymphatic vessels. Systemic chemotherapy has not been shown to be beneficial forappendiceal neoplasms in prospective, randomized trials; it is recommended for adenocarcinomas and goblet-cell adenocarcinomasof stage III or higher, in analogy to the treatment of colorectal carcinoma.
Topics: Humans; Appendix; Appendiceal Neoplasms; Prospective Studies; Hyperthermia, Induced; Adenocarcinoma; Appendectomy
PubMed: 37282595
DOI: 10.3238/arztebl.m2023.0136 -
Acta Gastro-enterologica Belgica 2020Primary appendiceal cancer is rare and most commonly found incidentally on a surgical specimen after appendectomy for acute appendicitis. This small organ gives rise to... (Review)
Review
Primary appendiceal cancer is rare and most commonly found incidentally on a surgical specimen after appendectomy for acute appendicitis. This small organ gives rise to different subtypes which are histological and biological distinct. Historically the classification of these tumors has been confusing because of the different nomenclature that is used. This review has broadly classified them into four subgroups: colonic-type adenocarcinoma, mucinous neoplasm, goblet cell carcinoma and neuroendocrine neoplasm. Signet ring cells is not considered as a distinct subgroup but as a histologic feature that can be present in colonic-type adenocarcinoma and mucinous neoplasms. As staging and management of appendiceal tumors depend on these subtypes, an adequate classification of them is important. This review aimed to give an overview of the epidemiology, grading and staging, management and prognosis of these neoplasms. Despite its rarety, specific staging systems and treatment guidelines exist for some subtypes. For other subtypes staging systems and management is extrapolised from colorectal cancer because of the lack of randomised, prospective trials.
Topics: Appendectomy; Appendiceal Neoplasms; Appendicitis; Colorectal Neoplasms; Humans; Prospective Studies
PubMed: 33094592
DOI: No ID Found -
Neuroendocrinology 2016
Topics: Appendiceal Neoplasms; Europe; Humans; Neuroendocrine Tumors
PubMed: 26730583
DOI: 10.1159/000443165 -
American Society of Clinical Oncology... Mar 2021Appendiceal neoplasms include a heterogeneous group of epithelial and nonepithelial tumors that exhibit varying malignant potential. This review article summarizes... (Review)
Review
Appendiceal neoplasms include a heterogeneous group of epithelial and nonepithelial tumors that exhibit varying malignant potential. This review article summarizes current diagnostic criteria, classification systems, and optimal therapeutic strategies for the five main histopathologic subtypes of appendiceal neoplasms. In particular, the management of epithelial appendiceal neoplasms has evolved. Although their treatment has historically been extrapolated from colon cancer, improved understanding of their unique histopathologic and molecular characteristics and a growing body of published clinical data support a more nuanced approach to their management.
Topics: Appendiceal Neoplasms; Humans; Neoplasms, Glandular and Epithelial
PubMed: 33770459
DOI: 10.1200/EDBK_321009 -
International Journal of Surgery... Jun 2015Mucocoele of the appendix is rarely encountered but consultant general surgical and trainees must be aware of it as a differential diagnosis, due to the nuances... (Review)
Review
INTRODUCTION
Mucocoele of the appendix is rarely encountered but consultant general surgical and trainees must be aware of it as a differential diagnosis, due to the nuances associated with its management. We aimed to provide a comprehensive review of the current literature concerning this rare surgical pathology.
METHODS
Search terms "appendi*", "tumour", "malignancy", "mucino*" and "cystadenoma" were used in combination to identify papers from PubMed. Abstracts and full text were manually reviewed to identify suitable papers.
RESULTS
Full search results included 311 articles. Review of titles and abstracts led to further full text review of 46 articles. Of these 30 were selected for inclusion based on relevance, adequate sample size and recent publication date.
DISCUSSION
Mucocoele of the appendix describes dilatation with associated luminal mucin and can result from benign and malignant processes. It contributes 0.2-0.7% of all appendiceal pathologies. The most common presenting symptoms are abdominal pain and a palpable mass in the right iliac fossa. Computed tomography of the abdomen and pelvis is key in facilitating diagnosis, although CEA and CA19-9 also have a role. The major complication of malignant causes of mucocoele is progression to pseudomyxoma peritonei. Treatment is surgical with or without chemotherapy depending on the underlying cause. Prognosis depends on aetiology.
CONCLUSION
Mucocoele of the appendix is a rare diagnosis. However, given the possibility of neoplastic peritoneal dissemination, it should be considered as a diagnosis, especially in older females with non-specific symptoms similar to appendicitis.
Topics: Abdominal Pain; Appendiceal Neoplasms; Appendicitis; Cecal Diseases; Cystadenoma, Mucinous; Diagnosis, Differential; Female; Humans; Mucocele; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Tomography, X-Ray Computed
PubMed: 25917270
DOI: 10.1016/j.ijsu.2015.04.052 -
Current Oncology (Toronto, Ont.) May 2022Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the... (Review)
Review
Pseudomyxoma peritonei (PMP) is a rare clinical syndrome. It originates from neoplasms of the appendix and leads to the formation of peritoneal implants and the accumulation of mucinous ascites. PMP represents a spectrum of low to high-grade disease. Despite aggressive management, many PMP patients recur, leading to debilitating symptoms and few treatment options. Therefore, scientists have continued to look for ways to improve treatment and further understand disease pathogenesis. Microorganisms were previously hypothesized to play a role in PMP progression and development. Hence, antibacterial treatment was suggested by some authors, but the data were limited. In this paper, we review the current data on the role of bacteria in PMP, discuss the significance, and suggest possible solutions to the inherent challenges in these studies. Given the limitations of the discussed studies, we remain skeptical about introducing novel antibacterial treatment into clinical practice at this time; however, the available data are valuable and indicate that more research into the molecular mechanisms of PMP is needed.
Topics: Appendiceal Neoplasms; Appendix; Humans; Neoplasm Recurrence, Local; Peritoneal Neoplasms; Pseudomyxoma Peritonei
PubMed: 35621679
DOI: 10.3390/curroncol29050289 -
Indian Journal of Cancer Oct 2023Histopathological examination of appendectomy specimens may reveal malignancies. Based on these results, either appendectomy is sufficient or sometimes a further...
BACKGROUND
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.
METHODS
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.
RESULTS
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.
Topics: Humans; Appendiceal Neoplasms; Appendicitis; Retrospective Studies; Appendectomy; Neuroendocrine Tumors
PubMed: 38159212
DOI: 10.4103/ijc.IJC_450_20 -
Clinics (Sao Paulo, Brazil) 2022The objective of this systematic review is to provide efficacy and safety data in the application of Intra-Abdominal Hyperthermia Chemotherapy (HIPEC) and Cytoreductive... (Review)
Review
Efficacy and safety in the use of intraperitoneal hyperthermia chemotherapy and peritoneal cytoreductive surgery for pseudomyxoma peritonei from appendiceal neoplasm: A systematic review.
The objective of this systematic review is to provide efficacy and safety data in the application of Intra-Abdominal Hyperthermia Chemotherapy (HIPEC) and Cytoreductive Surgery (CRS) in patients with Peritoneal Pseudomyxoma (PMP) of origin in the cecal appendix. The databases Medline and Central Cochrane were consulted. Patients with PMP of origin in the cecal appendix, classified as low grade, high or indeterminate, submitted to HIPEC and CRS. The results were meta-analyzed using the Comprehensive Metanalysis software. Twenty-six studies were selected to support this review. For low-grade PMP outcome, 60-month risk of mortality, Disease-Free Survival (DFS), and adverse events was 28.8% (95% CI 25.9 to 32), 43% (95% CI 36.4 and 49.8), and 46.7% (95% CI 40.7 to 52.8); for high-grade PMP, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 55.9% (95% CI 51.9 to 59.6), 20.1% (95% CI 15.5 to 25.7) and 30% (95% CI 25.2 to 35.3); PMP indeterminate degree, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 32.6% (95% CI 30.5 to 34.7), 61.8% (95% CI 58.8 to 64.7) and 32.9% (95% CI 30.5 to 35.4). The authors conclude that the HIPEC technique and cytoreductive surgery can be applied to selected cases of patients with PMP of peritoneal origin with satisfactory results.
Topics: Appendiceal Neoplasms; Combined Modality Therapy; Cytoreduction Surgical Procedures; Humans; Hyperthermia, Induced; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Pseudomyxoma Peritonei; Retrospective Studies
PubMed: 35576869
DOI: 10.1016/j.clinsp.2022.100039 -
BMC Surgery Feb 2021At present, the term mucocele is outdated, and mucinous appendiceal neoplasm is preferred. Mucinous appendiceal neoplasm is an uncommon pathology that occurs... (Review)
Review
BACKGROUND
At present, the term mucocele is outdated, and mucinous appendiceal neoplasm is preferred. Mucinous appendiceal neoplasm is an uncommon pathology that occurs predominantly in middle-aged women. Its classification and management have been the subject of debate in recent decades. The aim of this study was to analyse the incidence, clinical management and survival of these tumours diagnosed in our centre in the last 10 years.
METHODS
This was a retrospective observational study of patients with a diagnosis of appendiceal neoplasms between 2009 and 2018 in our centre. Variables such as sex, age, tumour type, clinical status, diagnosis, treatment and survival were collected. All data were analysed using the statistical program IBM SPSS Statistic® version 25.
RESULTS
Twenty-nine patients with a diagnosis of appendiceal neoplasm were identified, and 24 corresponded to neoplastic appendiceal mucinous lesions (85.7%). The average age was 59.7 ± 17.6 years. Most patients were women (15 cases; 62.5%). Most of them presented with chronic abdominal pain (37.5%), and the diagnosis was performed by computed tomography (CT) (50%). The treatment was surgical in all cases. The surgical technique depended on the findings and histology of the tumour.
CONCLUSION
Mucinous appendiceal neoplasms are an uncommon entity, and their pathological classification and management have recently changed.
Topics: Adult; Aged; Appendiceal Neoplasms; Appendix; Female; Humans; Middle Aged; Mucocele; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 33573654
DOI: 10.1186/s12893-021-01091-9