C0672 - A Rare Case of Appendiceal Goblet Cell Adenocarcinoma With Peritoneal Carcinomatosis

Syed Hamaad Rahman, DO¹, Nihal Ijaz Khan, MBBS², Abdul Arham, MBBS³, Abu Hurairah, MD^4
1Methodist Dallas Medical Center, Dallas, TX; ²Allama Iqbal Medical College, Sarnia, ON, Canada; ³Allama Iqbal Medical College, Mississauga, ON, Canada; ⁴AdventHealth Orlando, Orlando, FL

Introduction: Primary appendiceal cancers are an extremely rare entity, representing approximately 1% of gastrointestinal malignancies. Of those, appendiceal goblet cell carcinoma (GCC) is one of the most rare subtypes with a reported incidence of 0.05 cases per 100,000 population per year in the United States. These tumors consist of both glandular and neuroendocrine elements containing goblet cells. We present a unique case of appendiceal GCC with peritoneal carcinomatosis presenting as a small bowel obstruction.

Case Description/Methods: A 43-year-old male presented for 2 months of diffuse, intermittent abdominal pain which acutely worsened into a constant and colicky abdominal pain. On examination, he was afebrile with elevated blood pressure of 196/89 mmHg and had a distended, diffusely tender abdomen with sluggish bowel sounds. CT abdomen revealed small bowel obstruction and focal density in the right lower quadrant abutting the small bowel loops with associated edema and omental infiltration. Colonoscopy was performed which revealed an extremely tortuous colon and was complicated by a serosal tear and internal hemorrhage which required surgical intervention. Intraoperatively, omental caking and peritoneal carcinomatosis of the mesentery, peritoneal surfaces, distal jejunum, sigmoid colon and terminal ileum were noted. Biopsy and immunohistochemistry of the omentum and mesenteric nodules were consistent with a diagnosis of metastatic goblet cell adenocarcinoma with lymphovascular invasion. Once stabilized, he was started on FOLFOX chemotherapy regimen with outpatient heme/onc follow up.

Discussion: Appendiceal GCC is one of the rarest GI malignancies documented. It is often asymptomatic and diagnosed incidentally; however it can also present as chronic abdominal pain, acute appendicitis, and even bowel obstruction. Treatment consists of resection of the colon and chemotherapy in localized disease, and in cases of peritoneal carcinomatosis, cytoreductive therapy and heated intraperitoneal chemotherapy (HIPEC) are utilized. Not only is it rare, but it’s also more aggressive than other appendiceal cancers. One study showed that the average survival rate of patients with peritoneal carcinomatosis treated with cytoreductive surgery and HIPEC was 18.5 months. In cases of acute appendicitis, it is crucial to biopsy the surgical specimen so this diagnosis is not missed. Our case highlights an interesting and complicated presentation of this rare disease.


Disclosures:


Syed Hamaad Rahman, DO¹, Nihal Ijaz Khan, MBBS², Abdul Arham, MBBS³, Abu Hurairah, MD⁴. C0672 - A Rare Case of Appendiceal Goblet Cell Adenocarcinoma With Peritoneal Carcinomatosis, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.