MUCINOUS CYSTADENOMA OF APPENDIX.
Appendicular mucocele is rare. Clinical presentation is varied with more than half being asymptomatic. Patients may present with clinical symptoms suggestive of "acute appendicitis" or other non specific abdominal complaints. A case having pain in the right lower abdomen diagnosed as retrocecal mucocele of appendix on imaging studies. Surgery revealed a giant retrocecal mucocele measuring 14 cm in length and 4 cm in diameter. The final pathologic diagnosis was mucocele caused by mucinous cyst adenoma.
Keywords: Appendix, Mucinous cyst adenoma, Retroperitoneal.
Appendicular mucocele is a cystic dilatation of the lumen of the vermiform appendix. It is a rare condition. Pre operative diagnosis based on history, clinical examination aided by investigations is important for proper management of the case.
A 52 year old female was admitted in the surgical ward because of pain in right lower abdomen for the last two days associated with nausea. She had three episodes of pain at the same location but of low intensity in the last four months for which he was treated by different general practioners. Physical examination revealed mild bulge in the right lower abdomen with soft, tender mass which moves on palpation. Her TLC was 11.6A-109/l. On abdominal ultrasound there was a solid hypoechoic mass measuring 5.3A-4.8A-13.8 cm with central hyperechoic band in the right para umbilical region extending up to umbilicus. CT scan showed well defined, rounded, homogenous area in the right iliac fossa measuring 14.0A-5.0 cm, arising from medial aspect of caecum, extending superio medially upto lower pole of right kidney with scattered foci of calcification in its wall. Patient was operated through Grid Iron incision that was later converted into Rutherford Morrison.
There was cystic mass in the retrocecal location which was freed en mass and found meeting at the base of caecum at the confluence of tinea coli (fig-I). It was diagnosed as mucocele of the appendix. Base was ligated, excision was done and about 14 cm cystic mass was removed. On dissection, thick whitish secretion was seen coming out of it. The specimen turned out to be mucinous cyst adenoma of the appendix on histopathology. Patient had an uneventful recovery and was discharged on second post op day.
Appendicular mucocele was first described as a pathological entity by Rokitansky in 1842 and definitively named by Ferenin 18761. It is found in 0.2-0.3% of all appendicectomies and accounts for around 8-10% of all appendicular tumours2.
It is a gross descriptive term referring to the dilatation of the lumen of the vermiform appendix caused by an abnormal accumulation of mucous3. It may be an incidental finding, simulate acute appendicitis or a right iliac fossa mass1. Mucosal hyperplasia, retention cyst, mucinous cyst adenoma, and mucinous cyst adenocarcinoma of the appendix are four different types of appendicular mucocele2. The most common cause of appendiceal mucocele is mucinous cystadenoma, seen in 50% of appendiceal mucocele. Retrocecal location of mucinous cyst adenoma is rare3.
Mucinous cyst adenoma is a rare cystic neoplasm of the vermiform appendix characterized by villous adenomatous changes of the appendicular epithelium associated with mucin filled lumen. The most common presentation is right iliac fossa pain, similar to that of an acute appendicitis, however, about 25% of patients are asymptomatic and the condition is found incidentally on imaging or at the time of surgery3. Complications include intestinal obstruction, intussusception, gastrointestinal bleeding and extrinsic ureteral compression. The most fearful complication in a case of mucinous cyst adenocarcinoma is pseudomyxoma peritonei secondary to spontaneous or iatrogenic rupture of the appendix and consequent spillage of neoplastic cells and mucin into the peritoneal cavity4.
A correct preoperative diagnosis is thus important to help in the choice of surgical tactics since appendicectomy is adequate for cystadenoma while cystadenocarcinoma requires a right hemicolectomy and to avoid iatrogenic rupture and peritoneal spillage of mucin during surgery5. As illustrated in our patient; both ultrasound and CT scan help in the preoperative diagnosis of mucinous cyst adenoma of the appendix. Recently, laparoscopic approach has been used for the management of mucocele of appendix, however care should be taken while retrieving the specimen to avoid spillage of contents by using endo bag6. Irfan Skukar has described a case which presented with huge abdominal mass occupying almost whole of the abdominal cavity and constipation, an unusual presentation that was managed by laparotomy and excision of the mass signifying that it can grow to a very large size7.
CONFLICT OF INTEREST
This study has no conflict of interest to declare by any author.
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2. Deans GT, Spence RAJ. Neoplastic lesions of the appendix. Br J Surg. 1995; 82: 299306.
3. Rampone B, Roviello F, Marrelli D, Pinto E. Giant appendiceal mucocele: report of a case and brief review. World J Gastroenterol.2005; 11: 4761-3.
4. Teixeira M R, Qvist H, Giercksky K E, Bahler P J, Helm S. Cytogenetic analysis of several pseudomyxoma peritonei lesions originating from mucinous cystadenoma of the appendix. Cancer Genetics and Cytogenetics 1997; 93(2): 157-9.
5. Iswariah H, Metcalfe M, Lituri D, Maddern GJ. Mucinous cystadenoma of appendix. Australian and New Zealand J Surg 2004; 74(10): 918-19.
6. Rangarajan M, Palanivelu C, Kavalakat AJ, Parthasarathi R. Laparoscopic appendectomy for mucocele of the appendix. Report 8 cases. Indian J Gastroenterol 2006. 25 (5): 25657.
7. Shukar I. Mucinous cyst adenoma of appendix, of unusual size in a young man. Pak Armed Forces Med J 2006; 56(2): 201-3.
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|Publication:||Pakistan Armed Forces Medical Journal|
|Date:||Aug 31, 2015|
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