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Gastrointestinal Stromal Tumor of the Appendix with associated Mucocele Mimicking Acute Perforated Appendicitis Treated with Laparoscopic Appendectomy

Elizabeth A Verrico, DO1, Justin Sargent, DO1, Lindsay Tse, DO2, German Costa, MD1, Steven Shikiar, MD1. 1Hackensack Meridian Health Palisades Medical Center, 2Houston Methodist Hospital

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract and most often present in the stomach and small bowel although they may also present in the large bowel or esophagus. GIST of the vermiform appendix, however, is extremely rare, and represents <1% of all GISTs.

This is a case of a 54-year-old male who presented with acute right lower quadrant abdominal pain with associated nausea. His medical history was only significant for hypertension and his surgical history included a laparoscopic right inguinal hernia repair. On physical exam he was tender in the right lower quadrant and suprapubic region with mild localized guarding without generalized peritoneal signs. Laboratory results were only significant for a mildly elevated white blood cell count. CT was performed and demonstrated an inflamed appendix with periappendiceal inflammatory change and a 2.3x4cm rim enhancing fluid collection consistent with perforated appendicitis. The patient was given a course of intravenous antibiotics and taken to the operating room for laparoscopic appendectomy. Significant inflammation was noted in the right lower quadrant with mild peri-appendiceal adhesions and fluid. Successful laparoscopic appendiceal resection was completed. Post-operative course was unremarkable. Pathology revealed focal spindle cell proliferation at the distal portion of the appendix positive for CD 117 and CD 34, consistent with GIST, and focal mild luminal distention with luminal mucoid material and extravasation into the wall/periappendiceal tissue consistent with mucocele.

GIST of the vermiform appendix is infrequently reported in the literature with varying presentations from incidental diagnosis during surgery for another pathology or at autopsy, to signs and symptoms similar to acute appendicitis. Due to the variability of presentation and the uncommon pathological finding of GIST of the appendix, it is very difficult to diagnose in the preoperative period. Although rare, obstruction of the appendiceal lumen can lead to secondary inflammation and thus acute appendicitis-like symptoms. Therefore, appendiceal GIST should be considered in patients presenting with acute right lower quadrant abdominal pain, especially if imaging findings are concerning for possible mass of the appendix. Appendectomy should be performed in order to obtain appropriate pathological examination including immunohistochemical staining and evaluation of malignant potential so that treatment can be tailored on an individual basis.


Presented at the SAGES 2017 Annual Meeting in Houston, TX.

Abstract ID: 95763

Program Number: P029

Presentation Session: Poster Session (Non CME)

Presentation Type: Poster

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