Appendiceal Neoplasms
Primary appendiceal neoplasms are rare and most commonly diagnosed incidentally, upon histological analysis of the appendectomy specimen. The timing and circumstances of the diagnosis, tumor histology, staging, required resection margin, and the probabili
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Abbreviations AJCC DPAM ENETS HIPEC LAMN PCI PMAC PMCA PMP
American Joint Commission on Cancer Disseminated peritoneal adenomucinosis European Neuroendocrine Tumor Society Hyperthermic (or heated) intraperitoneal chemotherapy Low grade appendiceal mucinous neoplasms Peritoneal carcinomatosis index Peritoneal mucinous adenocarcinomatosis Peritoneal mucinous carcinomatosis Pseudomyxoma peritonei
Key Concepts • Although appendectomy for appendicitis is the most common emergency operation performed by general surgeons, primary neoplasms of the vermiform appendix are rare, and each individual general surgeon will have limited personal experience in the management of such lesions. • Most primary neoplasms of the appendix are not associated with specific signs or symptoms and are incidentally diagnosed after pathological analysis of the appendectomy specimen, or detected incidentally on imaging such as computed tomography (CT) done for other indications. • Primary neoplasms of the appendix can generally be divided into epithelial, non-epithelial, and mixed lesions. Epithelial lesions include adenoma and adenocarcinoma. Non-epithelial tumors include neuroendocrine tumors (carcinoids), lymphoma, leiomyoma, leiomyosarcoma, and other even rarer rarities. Goblet cell carcinoids are mixed lesions with features of carcinoid as well as mucinous adenocarcinoma.
• Epithelial tumors, and specifically mucinous adenocarcinomas, are the most common primary appendiceal neoplasms. • Pseudomyxoma peritonei is the result of a perforation and peritoneal dissemination of a mucin-producing epithelial neoplasm, most commonly originating from the appendix or the ovaries. In select patients, cytoreductive surgery with HIPEC should be considered. • A mucocele is a morphologic cystic manifestation of an epithelial appendiceal neoplasm. Perforation leads to pseudomyxoma peritonei. Therefore, intact removal enbloc is of utmost importance. • Appendiceal carcinoids are rarely associated with carcinoid syndrome or multicentricity. • The newest tumor staging guidelines distinguish appendiceal tumors from colon cancer, and separate between epithelial and non-epithelial lesions. • The extent of surgical resection depends on the cell type, preoperative staging, the ability to achieve negative resection margins, and the probability of nodal disease. • Surgery is the primary treatment for localized disease, whereas its role in metastatic disease needs to be individually analyzed and weighed against systemic chemotherapy.
Introduction The appendix vermiformis is commonly regarded as the organ that will introduce a surgical trainee to the art of his or her chosen specialty. Inflammation of this organ, namely appendicitis, is the disease process which will be instrumental in “teaching” the fundamentals of history taking, physical examination and differential diagnosis of the acute abdomen to medical students and surgical residents. Appendectomy is the most frequent emergency operation
© Springer International Publishing 2016 S.R. Steele et al. (eds.), The
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