This article has Open Peer Review reports available.
Chronic abdominal pain, appendiceal mucinous neoplasm, and concurrent intestinal endometriosis: a case report
© Kurogochi et al.; licensee BioMed Central Ltd. 2012
Received: 8 May 2012
Accepted: 18 August 2012
Published: 26 September 2012
Although both appendiceal tumor and intestinal endometriosis have been reported as rare causes of abdominal pain, the coexistence of appendiceal mucinous neoplasm and ileal endometriosis has not previously been reported.
A 41-year-old Japanese woman presented with a positive fecal occult blood test and a 3-year history of menstruation-related lower abdominal pain. A colonoscopy demonstrated extrinsic compression of the cecum, suggesting a mass arising from the appendix or adjacent structures. Abdominal imaging showed a 6-cm cystic mass with intraluminal thick fluids originating from the appendix. At ileocecal resection for an appendiceal tumor, a 2-cm mass in the terminal ileum was incidentally found, which was included in the surgical specimen. Microscopic examination confirmed a diagnosis of a mucinous neoplasm of the appendix with endometriosis of the terminal ileum.
To avoid urgent surgery for subsequent serious events associated with disease progression, appendiceal tumor and intestinal endometriosis should be ruled out in patients with chronic abdominal pain.
KeywordsChronic abdominal pain Intestinal endometriosis Mucinous neoplasm of the appendix
Appendiceal tumor and intestinal endometriosis have been reported as rare causes of chronic abdominal pain. In a 10-year-old boy, chronic right lower abdominal pain every 3 to 6 weeks for the past year prior to presentation was attributed to a mucinous neoplasm of the appendix. Surgical intervention is still the gold standard of making a tissue-based diagnosis of intestinal endometriosis, because only one third of symptomatic patients were suspected of having intestinal endometriosis preoperatively. Of the symptoms related to intestinal endometriosis, chronic abdominal pain may be more common than expected. We present a case of appendiceal mucinous neoplasm with incidental intestinal endometriosis presenting with chronic abdominal pain. To the best of our knowledge, it appears that this is the first case of appendiceal mucinous neoplasm in concurrence with intestinal endometriosis.
Approximately 25% of appendiceal mucinous neoplasms are asymptomatic and found incidentally on abdominal imaging or at surgery. Rare clinical manifestations of these tumors are intestinal obstruction, intussusception, gastrointestinal bleeding and extrinsic ureteral compression. In a series of 141 patients who underwent urgent appendectomy for suspected acute appendicitis, 4 patients were found to have a mucinous neoplasm of the appendix on final pathology. The classification and nomenclature of appendiceal mucinous neoplasms have not been confirmed. Classical division of these tumors into cystadenoma and cystadenocarcinoma is not necessarily suitable because Prayson and colleagues reported a series of 19 patients with pseudomyxoma peritonei, confirmed by malignant epithelium in peritoneal implants, where greater than 75% had only mucinous cystadenomas of the appendix as the primary lesion. These findings were repeated in another report, in which a mucinous cystadenoma without atypical epithelium was accompanied by pseudomyxoma peritonei. Recently, Pai and colleagues stratified appendiceal mucinous neoplasms into four distinct groups based on reassessment of pathologic features and long-term follow-up of 116 patients who underwent resection of appendiceal neoplasms. According to their classification, the low-grade noninvasive mucinous neoplasms, all of which have been called cystadenoma, were split into three groups. Group 1 is mucinous adenoma that is confined to the appendix; Group 2 is mucinous neoplasm with low-grade dysplasia that is confined to the appendix associated with extra-appendiceal acellular mucin; and Group 3 is mucinous neoplasm with low-grade dysplasia with extra-appendiceal neoplastic epithelium. Group 4 is mucinous cystadenocarcinoma with high-grade cytology, complex architecture, or invasion. After a long-term follow-up, mucinous adenoma (Group 1) never recurred and tumors in Group 2 rarely recurred irrespective of the types of surgery, whereas in Group 3 the 5-year disease-free survival rate was 25% and in Group 4 it was 20%. Our case was classified as Group 2, low-grade mucinous neoplasm without extra-appendiceal spread. For appendiceal mucinous neoplasms, various surgical procedures such as simple appendectomy, appendectomy with partial resection of the cecum, ileocecal resection and right hemicolectomy have been performed using an either open or laparoscopic approach. The principle of surgery is complete excision of the tumor without any spillage of mucus-containing tumor cells in the peritoneal cavity. Although the size of the tumor is not likely to help in the differential diagnosis of mucinous neoplasms, the procedure of choice seems dependent on tumor size. We chose ileocecal resection rather than right hemicolectomy because even if the tumor is diagnosed as a cystadenocarcinoma on subsequent histopathological examination, it rarely spreads by the lymphatics. Since synchronous appendiceal mucinous neoplasms and colon cancer or adenoma are not uncommon, examination of the large intestine should be done with full colonoscopy. Recently, the coexistence of ileal endometriosis and carcinoid tumor of the appendix was reported, but a case of appendiceal mucinous neoplasm with concurrent ileal endometriosis has not been reported.
Intestinal endometriosis may be more frequent than expected, but the involvement of the terminal ileum is rare. There were 163 cases of bowel endometriosis (5.4%) among 3037 laparotomies for endometriosis, and resections of the ileum and/or cecum were performed in 11 cases, whereas a colectomy was needed in 30 cases. In another report on patients with intestinal endometriosis, 72.4% had involvement of rectosigmoid, 13.5% of the rectovaginal septum, and 7% of the distal ileum. The classical symptom of dysmenorrhea due to endometriosis may be absent if the bowel is exclusively involved. The superficial intestinal endometriosis may be asymptomatic, but the clinical features of a subset of intestinal endometriosis are progressive, leading to subsequent serious complications such as intestinal obstruction[13, 14] and perforation requiring emergency surgery.
Both appendiceal tumor and intestinal endometriosis can cause chronic abdominal pain, which may be an omen of subsequent severe events associated with disease progression. Careful history taking and relevant abdominal imaging are needed when encountering a patient complaining of chronic abdominal pain.
Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Blecha MJ, Gupta A, Hoover JD: Chronic abdominal pain secondary to a mucous cystadenoma of the appendix in a 10-year-old boy. J Pediatr Surg. 2005, 40: 1792-1794. 10.1016/j.jpedsurg.2005.07.065.View ArticlePubMedGoogle Scholar
- Kaufman LC, Smyrk TC, Levy MJ, Enders FT, Oxentenko AS: Symptomatic intestinal endometriosis requiring surgical resection: clinical presentation and preoperative diagnosis. Am J Gastroenterol. 2011, 106: 1325-1332. 10.1038/ajg.2011.66.View ArticlePubMedGoogle Scholar
- Aho AJ, Heinonen R, Lauren P: Benign and malignant mucocele of the appendix: histological types and prognosis. Acta Chir Scand. 1973, 130: 392-400.Google Scholar
- Sieren LM, Collins JN, Weireter LJ: The incidence of benign and malignant neoplasia presenting as acute appendicitis. Am Surg. 2010, 76: 808-811.PubMedGoogle Scholar
- Prayson RA, Hart WR, Petras RE: Pseudomyxoma peritonei: a clinicopathologic study of 19 cases with emphasis on site of origin and nature of associated ovarian tumors. Am J Surg Pathol. 1994, 18: 591-603.PubMedGoogle Scholar
- Kusuyama T, Fujita M: Appendicieal mucinous cystadenoma associated with pseudomyxoma peritonei and multicystic peritoneal mesothelioma: report of a case. Surg Today. 1995, 25: 745-749. 10.1007/BF00311493.View ArticlePubMedGoogle Scholar
- Pai RK, Beck AH, Norton JA: Appendiceal mucinous neoplasms: clinocopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol. 2009, 33: 1425-1439. 10.1097/PAS.0b013e3181af6067.View ArticlePubMedGoogle Scholar
- Lee J, Gardiner KR, Wilson BG: Incidental mucinous cystadenocarcinoma of the appendix. Postgrad Med J. 1996, 72: 55-56. 10.1136/pgmj.72.843.55.View ArticlePubMedPubMed CentralGoogle Scholar
- Fujiwara T, Hizuta A, Iwagaki H: Appendiceal mucocele with concomitant colonic cancer. Report of two cases. Dis Colon Rectum. 1996, 39: 232-236. 10.1007/BF02068082.View ArticlePubMedGoogle Scholar
- Azordegan N, Yazdankhah A, Moghadasian MH: A rare case of coexistence of carcinoid tumor of the appendix vermicularis and ileal endometriosis. Arch Gynecol Obstet. 2009, 279: 183-187. 10.1007/s00404-008-0660-1.View ArticlePubMedGoogle Scholar
- Weed JC, Ray JE: Endometriosis of the bowel. Obstet Gynecol. 1987, 69: 727-730.PubMedGoogle Scholar
- MacAffee CHG, Greer HLH: Intestinal endometriosis: a report of 29 cases and a survey of the literature. J Obstet Gynecol Br Empire. 1960, 67: 539-555. 10.1111/j.1471-0528.1960.tb09211.x.View ArticleGoogle Scholar
- De Ceglie A, Bilardi C, Blanchi S: Acute small bowel obstruction caused by endometriosis: a case report and review of the literature. World J Gastroenterol. 2008, 14: 3430-3434. 10.3748/wjg.14.3430.View ArticlePubMedGoogle Scholar
- Ridha JR, Cassaro S: Acute small bowel obstruction secondary to ileal endometriosis: report of a case. Surg Today. 2003, 33: 944-947. 10.1007/s00595-003-2613-4.View ArticlePubMedGoogle Scholar
- Deeker D, Konig J, Wardelmann E: Terminal ileitis with sealed perforation–a rare complication of intestinal endometriosis: case report and short review of the literature. Arch Gynecol Obstet. 2004, 269: 294-298.Google Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.