Int J Colorectal Dis (2001) 16:408–409 DOI 10.1007/s003840100328
Gidon Almogy Yakov Fellig Kalman Paz Arie Durst Ahmed Eid
Adenocarcinoma of the appendix associated with long-standing Crohn’s disease Accepted: 1 June 2001 Published online: 28 August 2001 © Springer-Verlag 2001
Dear Editor: The association of inflammatory bowel disease (IBD) with colorectal adenocarcinoma is well established [1]. Patients suffering from longstanding Crohn’s disease (CD) are at increased risk for the development of adenocarcinomas of both the small and large bowels. Other neoplasms of the bowel, such as carcinoid tumors, do not occur in higher frequency in patients with IBD [2]. Adenocarcinoma of the appendix is a rare tumor that is usually found incidentally at the time of laparotomy. Appendiceal involvement in CD is not uncommon. We report a patient with long-standing CD who developed an adenocarcinoma of the appendix. This is the first report of an association between CD and adenocarcinoma of the appendix, and it illustrates that careful inspection is required for proper diagnosis and management. A 38-year-old man was admitted to our hospital for increasing abdominal pain 36 h after colonoscopy. The patient was diagnosed as having severe CD involving the terminal ileum and colon 10 years previously by small bowel follow-through and colonoscopy. His disease was nonresponsive to medical treatment, including 5-amino salicylic acid and steroids, and he remained with in-
LETTER TO THE EDITOR
tractable abdominal pain and diarrhea over the years. Immunosuppressive therapy failed due to intolerance to drugs. The patient’s compliance with medical advice, therapy, and follow-up was poor. He had been taking 10–20 mg prednisone daily, depending on the severity of his disease, for over 10 years. He had documented osteoporosis secondary to chronic steroid therapy. Several weeks prior to the current admission, the patient complained of worsening diarrhea and abdominal pain. He became progressively steroid dependent. Colonoscopy at another hospital showed severe Crohn’s colitis with a stricture at the splenic flexure which was pneumatically dilated. Immediately following the procedure the patient complained of abdominal pain. Plain radiography did not reveal free air, and the patient was discharged. The patient presented to our hospital 36 h later with increasing abdominal pain and fever. Plain radiography of the abdomen revealed a large amount of free air, and the patient was rehydrated and taken to the operating room for exploratory laparotomy. At laparotomy there was an inflammatory mass at the splenic flexure, without macroscopic perforation, and a hard, irregular mass at the base of the cecum, suspicious for a carcinoma. The terminal ileum was grossly diseased with fat, creeping, boggy mesentery, and thickening of the bowel wall. An extended colectomy was performed from healthy-appearing terminal ileum to the descending colon beyond the inflammatory mass, and a mucous fistula and end ileostomy were fashioned. Postoperatively the patient developed severe wound infection, necessitating drainage of the wound. The patient recovered and was discharged 13 days after surgery. Macroscopic examination of the surgical specimen, which included
the terminal ileum, colon, and appendix, revealed creeping fat and thickening of the bowel wall. There were fissures and stricture formation. The mucosa had a cobblestone appearance. There was perforation of the colon with a pericolic abscess. The appendiceal wall was replaced by a friable to solid white tissue obscuring the lumen. A mucous filled space was found in the periappendiceal fat near the appendiceal apex. Histological sections of the terminal ileum and colon showed skip lesions with architectural distortion of the crypts and cryptitis. Areas of mucosal ulceration with fissure formation and transmural inflammation with lymphoid aggregates in the muscularis propria were noted. Histological sections of the appendix showed adenocarcinoma of the appendix, moderately to poorly differentiated, with focal mucinous areas, invading through the wall and into the periappendiceal fat. A few ill-defined noncaseating granulomas composed of multinucleated giant cells surrounded by lymphocytes were found in the appendiceal serosa and subsereosa adjacent to the tumor. The multinucleated giant cells did not stain immunohistochemically for cytokeratin. The adjacent cecum was not involved with adenocarcinoma. These findings are consistent with Crohn’s terminal ileitis and colitis, possibly with appendiceal involvement, alongside with adenocarcinoma confined to the appendix. Ulcerative colitis (UC) carries a significant risk for the development of adenocarcinoma of the colon and rectum [1, 3]. The extent and duration of disease are directly related to the increased risk. Although the appendix has been shown to be involved in more than 50% of cases of pancolitis [4], only four cases of appendiceal carcinoma associated with UC have been published [5, 6, 7, 8]. All four cases had long-standing UC.
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The appendix was involved in the inflammatory process in only one of these cases [5]. Adenocarcinoma of the appendix is a rare tumor that is usually found incidentally at the time of laparotomy performed for signs and symptoms of acute appendicitis. CD has been shown to mimic both acute appendicitis and adenocarcinoma of the appendix [9]. In this case, laparotomy was performed for iatrogenic colonic perforation, and the appendiceal mass was an incidental and unrelated finding. This case represents the first published report of an adenocarcinoma of the appendix in a patient with long-standing CD. A careful and thorough exploration of the small and large intestine was required in order to reach the diagnosis and initiate proper surgical management. Odze at al. [5] have reported a patient with long-standing UC who developed appendiceal adenocarcinoma in histologically affected appendix. They concluded that dysplasia in the involved appendix eventually led to malignant transformation. However, it is well established that patients with CD can develop adenocarcinomas of the colon in unaffect-
ed segments of the large bowel [3]. Since the appendix is composed of colonic-type epithelium, it is reasonable to believe that patients with CD can develop adenocarcinoma of the appendix more frequently. In summary, this case shows an association between CD and appendiceal adenocarcinoma which has not yet been reported. A careful search should be made for this pathology in all patients with longstanding IBD.
References 1. Heimann TM, Oh SC, Martinelli G, et al (1992) Colorectal cancer associated with ulcerative colitis: a study of prognostic indicators. Am J Surg 164:13–17 2. Greenstein AJ, Balasubramanian S, Harpaz N, Rizwan M, Sachar DB (1997) Carcinoid tumors and inflammatory bowel disease: a study of eleven cases and review of the literature. Am J Gastroenterol 92:682–685 3. Ribeiro MB, Greenstein AJ, Sachar DB, et al (1996) Colorectal adenocarcinoma in Crohn’s disease. Ann Surg 223:186– 193 4. Scott IS, Sheaff M, Coumbe A, et al (1998) Appendiceal inflammation in ulcerative colitis. Histopathology 33:168– 173
5. Odze RD, Medline P, Cohen Z (1994) Adenocarcinoma arising in an appendix involved with chronic ulcerative colitis. Am J Gastroenterol 89:1905– 1907 6. Zannoni U, Masci C Bazzocchi R, et al (1997) Cancer of the appendix in longstanding ulcerative colitis: a case report. Tumori 83:958–959 7. Kashani M, Levy M (1983) Primary adenocarcinoma of the appendix with bilateral Krukenberg ovarian tumors. J Surg Oncol 22:101–105 8. Moreira VF, Milicua JM, Sanchez AG, et al (1979) Colitis ulcerosa, pericolangitis y carcinoma appendicular. Rev Clin Esp 154:237–241 9. Scott MJ (1991) Primary adenocarcinoma of the vermiform appendix masquerading as Crohn’s disease: case report. Eur J Surg 157:153–154 G. Almogy (✉) · A. Durst · A. Eid Department of Surgery, Hadassah Hebrew University Hospital, P.O. Box 12000, Jerusalem, Israel e-mail:
[email protected] Tel.: +972-2-6777111 Fax: +972-2-6417997 Y. Fellig Department of Pathology, Hadassah Hebrew University Hospital, Jerusalem, Israel K. Paz Division of Gastroenterology, Department of Medicine, Shaare-Zedek Hospital, Jerusalem, Israel