Atypical Presentation of Appendiceal Adenocarcinoma – Case Study and Review of Literature

Andrew Nowakowski, OMS, Venkata K Kella, MD, Eric Balzano, MD, Hardev S Parihar, MD, FACS, Jasbir S Makar, MD. Weirton Medical Center, Weirton, WV 26062.

 INTRODUCTION: Adenocarcinoma of the appendix is very rare and represents less than 0.5% of all gastrointestinal neoplasms. Primary signet ring cell carcinoma accounts for less than 4% of all neoplasms of appendix. It usually presents as acute appendicitis or palpable mass and the unusual presentations include peritoneal carcinomatosis, hematuria, hydronephrosis. Here, we present a case study of mucinous adenocarcinoma of appendix presenting as retroperitoneal abscess and its management.

CASE STUDY: A 75-year-old white male presented with constant sharp right flank pain of 4 weeks duration. His medical history include hypertension, hypothyroidism, hypercholesterolemia, hyperuricemia, and degenerative joint disease.

Physical examination revealed stable vital signs and tender fullness in the right flank region. CT scan of abdomen showed a large retroperitoneal abscess due to perforated retrocecal appendicitis. Percutaneous drainage of the abscess was performed, cultures grew E Coli and treated with Ciprofloxacin. 3 weeks later, catheterogram performed for persistant residual cavity, revealed fistulous communication with cecum and ascending colon. Hemorrhage into the drain prompted surgical intervention and bilateral ureteric stents were placed. At laparoscopy, which was converted to laparotomy, hard large mass was found in the right lower quadrant infiltrating the posterior abdominal wall with mesenteric lymph nodes. Frozen section confirmed carcinoma. Right hemicolectomy was performed. Pathology confirmed high grade invasive mucinous adenocarcinoma of the appendix pT3No. His post-operative course was uneventful.

Patient received 6 cycles of FOLFOX treatment followed by Exploratory laparotomy, radical intraabdominal tumor debulking, omentectomy, peritoniectomy, wide local excision of retroperitoneal mass involving iliac muscle down to bone, wedge resection of liver lesion and hyperthermic intraperitoneal chemoperfusion with oxaloplatin for 2 hours at 42 degree Celsius, at a tertiary care center, 7 months after the primary surgery. At 1 year, the patient is well without evidence of recurrence on PET CT scan.

CONCLUSION: Non carcinoid carcinoma of appendix is rare, more so is the primary signet ring type. This case study highlights the atypical presentation and multidisciplinary management of mucinous adenocarcinoma of the appendix with very gratifying results.

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