Abstract

Appendicectomy is one of the most performed surgical procedures. Neoplasms of the appendix are identified in approximately 1% of appendiceal specimens, and the incidence seems to be rising. The aim of this review was to summarize the current data on these neoplasms, focusing on the recent classification, clinical manifestations, management, and follow-up of such patients, which remain under debate. A literature search was performed using the database PubMed. The keywords used were related to appendiceal neoplasms, mucocele, pseudomyxoma peritonei and treatment. Records without abstracts, case reports, opinion articles and experimental studies were excluded. The appendix tumors can be classified between epithelial or nonepithelial. The epithelial variant is composed by mucinous neoplasms, nonmucinous adenocarcinoma, and signet ring cell tumors; instead, neuroendocrine tumors, lymphoma, and sarcoma are included in nonepithelial neoplasia. Goblet cell carcinoids share characteristics of both epithelial and nonepithelial tumors. The clinical presentation can be variable and the algorithm for evaluation and treatment is complex. Treatment is based on stage and histology. Our intention is to clarify some questions and help the surgeon in operative decisions, treatment strategies, and patient counseling. Doi: 10.28991/SciMedJ-2021-0303-9 Full Text: PDF

Highlights

  • The appendix was first described in literature in 1522 by Jacopo Berengario da Carpi [1, 2]

  • The epithelial variant is composed by mucinous neoplasms, nonmucinous adenocarcinoma, and signet ring cell tumors; instead, neuroendocrine tumors, lymphoma, and sarcoma are included in nonepithelial neoplasia

  • Even neuroendocrine tumors with 1-2cm may be treated with an appendectomy alone, if the margin is clear, there is less than 3 mm of mesoappendiceal invasion, multifocality is not present, and there is no involvement of regional nodal or isolated liver [29, 39]

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Summary

Introduction

The appendix was first described in literature in 1522 by Jacopo Berengario da Carpi [1, 2]. During the three centuries, its role in the inflammation of the right lower quadrant was not completely understood [3, 4] It was just in 1735, that Claudius Amyand performed the first appendicectomy that was popularized, as we know it today, by McBurney in 1889 [5]. Most of the neoplasms of the appendix are discovered incidentally in the pathological analysis; on the other hand, some can be identified intraoperatively [8]. Goblet cell carcinoids share characteristics of both epithelial and nonepithelial tumors

Epidemiology
Pathology
Classification
Nonepithelial Tumors
Goblet Cell
Clinical Manifestations
Diagnosis and Staging
Surgical Management
Appendectomy
Right Hemicolectomy
Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy
Preoperative Systemic Chemotherapy
Follow-up
Prognosis
10. Conclusions
11.1. Author Contributions
Findings
12. References
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