Shantanu Kumar Sahu, Shikhar Agarwal, Sanjay Agrawal, Shailendra Raghuvanshi , Nadia Shirazi, Saurabh Agrawal and Uma Sharma
Neoplasms of the appendix are rare, accounting for less than 0.5% of all gastrointestinal malignancies and found incidentally in approximately 1% of appendectomy specimen. Carcinoids are the most common appendicular tumors, accounting for approximately 66%, with cystadenocarcinoma accounting for 20% and adenocarcinoma accounting for 10%. Appendiceal adenocarcinomas fall into one of three separate histologic types. The most common mucinous type produces abundant mucin, the less common intestinal or colonic type closely mimics adenocarcinomas found in the colon, and the least common, signet ring cell adenocarcinoma, is quite virulent and associated with a poor prognosis. Adenocarcinoma of appendix is most frequently perforating tumour of gastrointestinal tract due to anatomical peculiarity of appendix which has an extremely thin subserosal and peritoneal coat and the thinnest muscle layer of the whole gastrointestinal tract. In addition to the risk of perforation, mucinous adenocarcinoma of appendix have peculiar tendency for fistula formation. Many of unusual presentations reported for primary appendicular carcinoma are the results of fistula formation into the adjacent viscera such as the urinary bladder, bowel or vagina as well as extraperitoneally into retroperitoneal tissues or directly to the skin surface.
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