Abdominal Pain and Ileocolitis in a 51-Year-Old Woman

Abstract

Question: A 51-year-old woman was evaluated over 2 months for abdominal pain. She had a history of appendectomy several years prior for appendicitis, abdominal hysterectomy, and multiple exploratory laparoscopies with lysis of adhesions in the remote past. At presentation, she complained of right-sided, crampy abdominal pain. An abdominal CT showed symmetrical thickening of the distal ileum and serosal inflammation around the cecum, and postoperative changes of appendectomy. Colonoscopy revealed a markedly deformed cecum and inaccessible terminal ileum. A cecal biopsy demonstrated mild chronic inflammation and edema of the lamina propria. She was treated with corticosteroids for presumed Crohn’s ileocolitis. Follow-up MRI enterography showed inflammatory changes of a long segment of distal ileum extending to the ileocecal junction evidenced by mural thickening with mucosal hyperenhancement (Figure A, arrow). Owing to persistence of symptoms and these imaging findings, medical therapy was intensified with initiation of adalimumab. There was no improvement and she developed obstructive symptoms necessitating hospitalization. Upright plain radiograph of the abdomen showed multiple dilated loops of small bowel (Figure B). She underwent a laparoscopic-assisted ileocecectomy with primary stapled anastomosis owing to the refractory nature of her symptoms and to obtain a tissue diagnosis. Histopathology is shown in Figure C. What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Figure C shows a poorly differentiated adenocarcinoma ex goblet cell carcinoid (GCC), which is a subtype of the rare GCC tumor that occurs almost exclusively in the appendix.1Roy P. Chetty R. Goblet cell carcinoid tumors of the appendix: An overview.World J Gastrointest Oncol. 2010; 2: 251-258Crossref PubMed Google Scholar Histologic evaluation showed malignant glands infiltrating the muscle layer (Figure C, panel 1; original magnification, ×200), residual focal areas of GCC (Figure C, panel 2; original magnification, ×400), and tumor cells positive for chromogranin (Figure C, panel 3) and cdx2 (Figure C, panel 4). Three subtypes of GCC exist and are classified as typical GCC (group A), adenocarcinoma ex GCC signet ring cell type (group B), and poorly differentiated adenocarcinoma ex GCC (group C).2Tang L.H. Shia J. Soslow R.A. et al.Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.Am J Surg Pathol. 2008; 32: 1429-1443Crossref PubMed Scopus (221) Google Scholar Histologic examination in this case revealed a group C tumor, which is the least common, and has the highest risk of recurrence and mortality.2Tang L.H. Shia J. Soslow R.A. et al.Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.Am J Surg Pathol. 2008; 32: 1429-1443Crossref PubMed Scopus (221) Google Scholar GCC most commonly presents as acute appendicitis, but can have a various presentations including nonspecific abdominal pain as seen in our patient.2Tang L.H. Shia J. Soslow R.A. et al.Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.Am J Surg Pathol. 2008; 32: 1429-1443Crossref PubMed Scopus (221) Google Scholar Cross-sectional imaging findings are usually those of acute appendicitis or a prominent appendix, but are not reported to include abnormalities of long segments of bowel.3Lee K.S. Tang L.H. Shia J. et al.Goblet cell carcinoid neoplasm of the appendix: clinical and CT features.Eur J Radiol. 2013; 82: 85-89Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar There is scant literature describing GCC in general, with group C lesions representing only 7 cases in a large series of 63 patients by Tang et al2Tang L.H. Shia J. Soslow R.A. et al.Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix.Am J Surg Pathol. 2008; 32: 1429-1443Crossref PubMed Scopus (221) Google Scholar in 2008. This case of ex GCC likely arose from the appendiceal stump and to our knowledge has never been described. GCC tumors are rare, malignant lesions primarily of the appendix and may infiltrate the cecum and ileum mimicking Crohn’s ileocolitis. This case underscores the importance of considering the differential diagnosis of inflammatory thickening of the ileum and cecal regions particularly when there are atypical histologic features and lack of response to medical therapy.