Appendiceal intussusception of mucinous cystadenoma presenting as acute appendicitis

Iftikhar Ahmed Khan; Khaloud Al Zain; Mahdi Saleh; Abdullah Al Dohayan

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CASE REPORT

Year : 2002 | Volume : 8 | Issue : 1 | Page : 25-27

Appendiceal intussusception of mucinous cystadenoma presenting as acute appendicitis

Iftikhar Ahmed Khan, Khaloud Al Zain, Mahdi Saleh, Abdullah Al Dohayan
Department of Surgery, King Khalid university Hospital, King Saud University, Riyadh, Saudi Arabia

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Date of Submission	16-Dec-2000
Date of Acceptance	16-Jul-2001

How to cite this article:
Khan IA, Al Zain K, Saleh M, Al Dohayan A. Appendiceal intussusception of mucinous cystadenoma presenting as acute appendicitis. Saudi J Gastroenterol 2002;8:25-7

How to cite this URL:
Khan IA, Al Zain K, Saleh M, Al Dohayan A. Appendiceal intussusception of mucinous cystadenoma presenting as acute appendicitis. Saudi J Gastroenterol [serial online] 2002 [cited 2020 Nov 28];8:25-7. Available from: https://www.saudijgastro.com/text.asp?2002/8/1/25/33381

Introduction

Mucinous cystadenoma is an uncommon tumor of the appendix. Grossly it may form appendiceal mucocele due to accumulation of mucus in the lumen of the appendix. This can act as a lead point for intussusception. However, this happens rarely^[1] . We present a case of appendiceal intussusception due to mucinous cystadenoma.

Case Report

A 21-year old male presented to the emergency room with right lower quadrant pain for four days. This was associated with anorexia and nausea. There was no vomiting and no change in bowel habits or urinary symptoms. Examination revealed tenderness and guarding in right iliac fossa. Bowel sounds were exaggerated. Per rectal examination was normal. WBC count was 9000/ mm³ . Diagnosis of acute appendicitis was made. Under general anesthesia, a mass could be felt in right lower quadrant. Exploration through gridiron incision revealed small quantity of clear fluid localized around appendix. Base of appendix was swollen and was intussuscepting into the cecum [Figure - 1]. There was no mesenteric lymphadenopathy. After extension of gridiron incision, the patient underwent limited right hemicolectomy and had uneventful post-operative recovery.

Pathological series of the specimen comprised of segment of terminal ileum, appendix and cecum. Appendix was 4cm in diameter. It contained yellowish white mucous material. The length of the ileal segment was 9cm. The ascending colon measured 8cm in length. Sections through appendix showed atypical epithelial lining with areas of mucinous differentiation. There was no infiltration of underlying structures [Figure - 2],[Figure - 3]. Histopathological diagnosis of mucinous cystadenoma arising from base of appendix was reported. Sections of ileum and colon were unremarkable. There was no evidence of metastasis in mesenteric lymph nodes.

At the first follow up visit, three weeks post the operation, he was doing well. He did not return after the first clinic visit.

Discussion

Appendiceal mucocele describes the gross enlargement of the appendix from accumulation of mucoid substance within the lumen. Mucocele of appendix is rare, encountered only in 0.2-0.3% of 43,000 appendectomies reviewed^[2] . Four histological subtypes are described. Cystadenomas occur with relative frequency of 52%. Other types are retention cyst, mucosal hyperplasia and cyst adenocarcinoma with relative frequencies of 18,20 & 10 percent respectively^[3] . The course of the disease and the prognosis are related to these subtypes^[3] .

In six percent of instances, appendiceal mucoceles may be associated with an unusual form of gelatinous ascites termed pseudomyxoma peritonei. This description is limited to ascites shown to contain epithelial cells, which may be isolated, or in clusters and excludes acellular ascites, which sometimes accompanies appendiceal mucoceles^[4] , as in our case.

Intussusception in adults is a rare occurrence, representing 1 % of patients with bowel obstruction and five percent of all intussusceptions^[5] . In contrast to intussusception in children, a demonstrable etiology is found in 70-90% of cases in the adult population. Intralumenal lesions alter normal bowel peristalsis and form leading edges for the intussusceptum. Although intussusception presents acutely in children, adults may present with acute, intermittent or chronic symptoms^[5] .

Very few cases of appendiceal intussusception associated with mucocele have been reported^{[6],[7],[8],[9]} . Majority of the cases have clinical picture of chronic or relapsing intestinal obstruction. Our case presented with features of acute appendicitis. Because the symptomatology of adult intussusception varies greatly among individuals, it is impossible to define a perfect diagnostic algorithm. Although the correct diagnosis is often based upon intra-operative findings, the modern diagnostic techniques can be of significant help in precisely identifying these lesions preoperatively^[10] .

Plain abdominal films and contrast studies showed location of intestinal obstruction. Typical aspects of appendiceal mucocele include a rim-like calcification of the mucocele wall seen on the plain abdominal film and ceceal filming defect at barium enema^[3] . Persistent intussusception of the appendix during the barium enema examination is highly suggestive of any kind of appendiceal abnormality because idiopathic intussusception often reduces spontaneously during the examination^[11] . The classic sonographic features of intussusception include the "target" and "doughnut" signs in transverse view and the "psuedokidney" sign in the longitudinal view^[12] . However, the use of ultrasound is limited due to the presence of gas in the bowel, which leads to poor transmission and difficulties in image interpretation.

The characteristic CT features of intussusception include a target mass with enveloped, excentrically located areas of low density^[13] . Appendiceal mucocele may appear as a cystic lesion with parietal calcification in contiguity with the cecum on CT scan^[3] . Fine needle aspiration (FNA) of the appendiceal mucocele is generally avoided because of fear that puncture of a distended viscus will lead to seeding of neoplastic cells and result in localized or diffused pseudomyxoma paritonei. However, a recent case report suggests that FNA of an appendiceal mucocele may be safe when a smallgauge needle and retroparitoneal approach are utilized^[14] .

As majority of intussusceptions in adults have an underlying pathology, resection without reduction is advocated^[5] . Also, treatment of appendiceal mucocele is surgical. Procedures vary from appendectomy to right hemicolectomy. If malignancy is suspected, the surgeon is best advised to proceed with right hemicolectomy^[6] . The incision for exploration should be midline. However, in case of acute symptoms, as in our case, the gridiron incision can be extended to muscle-cutting incision to allow adequate excess^[15] .

Appendectomy alone is curative for patients with benign cystadenomas developing on the distal twothirds of the appendix because the tumors never spreads to adjacent lymph nodes. However, right colectomy is indicated when the tumor involves the proximal one-third of the appendix, as in our case, or in the instance of adhesions with the terminal part of the ileum or cecum^[3] . There are no reported cases of recurrence or mortality after five years because of cystadenomas, after adequate surgical treatment^[16] .

Patients with mucinous cystadenocarcinomas with or without pseudomyxoma peritonei require aggressive initial surgical therapy in the form of right hemicolectomy. This should also include complete removal of all gelatinous masses within the abdominal cavity, resection of the greater omentum and bilateral o'ophorectomy when ovarian involvement is present^[3] . Some reports recommend second look laparotomies at six months^[17] . Appropriate postoperative follow-up is therefore recommended.

References

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