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Mucocele of the Appendix - Appendectomy Or Colectomy?

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Mucocele of the Appendix - Appendectomy Or Colectomy? Original Article Mucocele of the appendix - appendectomy or colectomy? JANDUÍ GOMES DE ABREU FILHO1, ERIVALDO FERNANDES DE LIRA1 1Service of Coloproctology of Hospital de Base do Distrito Federal (HBDF), Secretariat of Health in Distrito Federal - Brasília (DF), Brazil. FILHO JGDA; LIRA EFD. Mucocele of the appendix - appendectomy or colectomy? Rev bras Coloproct, 2011;31(3): 276-284. ABSTRACT: Mucocele of the appendix is a rare disease. It can be triggered by benign or malignant diseases, which cause the obstruc- tion of the appendix and the consequent accumulation of mucus secretion. The preoperative diagnosis is difficult due to non-specific clinical manifestations of the disease. Imaging tests can suggest the diagnosis. The treatment is always surgical and depends on the integrity and size of the appendix base and on the histological type of the original lesion. The prognosis is good in cases of integrity of the appendix. The perforation of the appendix and subsequent extravasation of its contents into the abdominal cavity may lead to pseudomyxoma peritonei, which has very poor prognosis if not treated properly. Keywords: mucocele; appendix; pseudomyxoma peritonei; treatment. INTRODUCTION first one defends the right colectomy as a treatment9, and the second one recommends only appendecto- The mucocele of the appendix was first de- my10. Despite the different adopted conducts, in both scribed in 1842 by Rokitansky1. This disease is reported cases a cystadenoma was diagnosed in the considered as a rare lesion of the appendix, which appendix; the choice was for elective surgery. is found in 0.2 to 0.3% of the appendectomies2. It The objective of this review is to analyze liter- is characterized by the dilation of the organ lumen ature as to mucocele, especially regarding diagnosis with mucus accumulation, being more frequent and treatment, besides discussing follow-up and prog- among individuals aged 50 years or more3,4. Gender nosis of the individuals who have this disease. prevalence is controversial. Appendix mucocele may come as a conse- LITERATURE REVIEW quence of obstructive or inflammatory processes, cys- tadenomas or cystadenocarcinomas7. Besides these The mucocele of the appendix is a descriptive causes, other tumor lesions in the appendix or cecum and unspecific term to define the cystic dilation of the may present as mucocele8. Its main complication is appendix caused by the accumulation of mucus secre- pseudomyxoma peritonei. tion. This process is slow and gradual, with no signs Treatment is always surgery and determined by of infection inside the organ. It results from the lumen the organ’s integrity, the dimensions of the base and obstruction in the appendix, which is secondary to the histological type of the lesion. inflammatory or neoplastic proliferation of the appen- Revista Brasileira de Coloproctologia has re- dix mucosa, or of lesions in the cecum, adjacent to the cently published two articles about this disease. The appendiceal ostium. Study carried out at the Service of Coloproctology of HBDF, Brasília (DF), Brazil. Financing source: none. Conflict of interest: nothing to declare. Submitted on: 13/09/2010 Approved on: 31/01/2011 276 Journal of Coloproctology Mucocele of the appendix - appendectomy or colectomy? Vol. 31 July/September, 2011 Janduí Gomes de Abreu Filho et al. Nº 3 While some articles confirm its prevalence Mucocele of the appendix can also result from al- among women3,4, others demonstrate a higher inci- terations in the cecum, such as fecal impaction, polyps dence among men5,6. or malignant neoplasms, which, in theory, can obstruct Mucocele in the appendix may be classified ac- the appendiceal ostium. Another rare cause found in lit- cording to the histological characteristics of lumen ob- erature is endometriosis, which may be established on struction7. the wall of the appendix, causing lumen obstruction8. Simple mucocele (inflammatory, obstructive or The classification based on histopathological retention cyst) is characterized by degenerative epi- standards is really important, once the progress of the thelial changes and results in the obstruction and the disease and its prognosis are related to the subtypes. distension of the appendix. There is no evidence of Simple and benign mucoceles have an insidious hyperplasia or mucosal atypia. evolution and are rarely perforated; on the other hand In hyperplastic mucocele, the appendix dilation oc- malignant mucocele evolution is faster, like in acute ap- curs due to the hyperplastic growth of the appendix or pendicitis, usually presenting as an organ perforation4. cecal mucosa, just like hyperplastic polyps in the colon. The diameter of the non-neoplastic mucocele Simple and hyperplastic mucoceles correspond to (simple and hyperplastic) is smaller than the neoplas- 5 to 25% of the cases, and mucus is usually acellular. tic mucocele (cystadenoma and cystadenocarcinoma); The mucinous cystadenoma is an appendix neoplasm however, there is no difference related to size among with dysplastic epithelium similar to colon adenomatous benign and malignant neoplastic mucoceles. Besides, polyps, and corresponds to 63 to 84% of the cases. no neoplastic mucocele has a diameter smaller than The mucinous cystadenocarcinoma presents two centimeters4. high grade cellular dysplasia and stromal invasion, The clinical presentation of mucocele in the ap- besides muscularis mucosae, and represents 11 to pendix is usually unspecific, with difficult preopera- 20% of the cases. tive diagnosis. The most common complaint is pain in In both types described, the mucus material con- the right lower quadrant of the abdomen, which may tains epithelial adenoma cells with low or high grade last for months, being noticed in up to 50% of the cas- of dysplasia. The rupture of the appendix may lead to es, associated or not with a palpable tumor. About 25 the dissemination of the epithelium that produces mu- to 50% of the subjects can be asymptomatic4,5. cins in the abdominal cavity, causing mucinous ascites The carcinoembryonic antigen (CEA) level at or pseudomyxoma peritonei. preoperative may suggest malignity in the appendix Stocchi et al.4 analyzed 135 patients with mucocele of or in the colon9,10. the appendix. Out of these, 129 were submitted to surgery Imaging tests, such as ultrasound, computed to- and histological analyses, and 37 presented pseudomyxo- mography and enema, besides colonoscopy, may sug- ma peritonei. From the 37 cases of pseudomyxoma peri- gest the presence of mucocele of the appendix, which donei, 95% had malignant mucocele, and 83% of the per- helps to define the treatment. forated mucoceles were malignant. The ultrasound shows an encapsulated cystic lesion Ronnet et al.11 studied 109 cases of pseudomyxo- in the lower quadrant of the abdomen with a liquid con- ma peritonei and classified it in two types: disseminated tent of variable echogenicity, according to the density of peritoneal adenomucinosis, which is a result of mucino- the mucus12. Some tests present images of multiple echo- us adenoma, with survival rate of 84%; and peritoneal genic layers in the dilated appendix, which may be con- mucinous carcinomatosis, which is secondary to muci- sidered as pathognomonic of appendix mucocele13. nous adenocarcinoma, with survival rate of 6.7%. At computer tomography, the typical feature of Thus, both the benign and the malignant mu- mucocele of the appendix is a cystic mass with a thin cocele may cause pseudomyxoma peritonei, howev- wall and of low density, which communicates directly er, this complication is more frequent and has worse with the cecum (Figure 1). The presence of punctate prognosis for malignant cases, probably because in or curvilinear calcification in this wall confirms the this situation the appendix ruptures more easily, and mucocele diagnosis and differs from the appendicular the celular seeding is more aggressive4,5,11. abscess, which does not have this characteristic6. 277 Journal of Coloproctology Mucocele of the appendix - appendectomy or colectomy? Vol. 31 July/September, 2011 Janduí Gomes de Abreu Filho et al. Nº 3 At enema, the presence of mucocele can be char- used due to the risk of cell dissemination and evolu- acterized by a cecal fi lling defect, besides the lateral tion to pseudomyxoma peritonei18,19. displacement of the cecum and terminal ileum14. The treatment of appendix mucocele is surgeryl Colonoscopy may show a soft erythematous and determined by some factors, such as the integrity of mass, with a central crater due to the protrusion of ap- the wall of the organ, the dimensions of its base and his- pendiceal ostium, which can increase or decrease ac- topathological examination of the cause of mucocele. cording to the respiratory movement15. This condition For mucocele (Figure 3), appendectomy with is known as “volcano sign”16 (Figure 2). This exami- lymphadenectomy, including all the fat from the me- nation may still show the presence of synchronic neo- soappendix in the resection, is indicated for cases of plastic lesions in the colon, which occur in up to 20% simple or benign mucocele, when the appendicular of the cases17. base is not compromised by the dilation and is smaller The cytology of the mucus inside the mucocele than 2.0 cm (Figure 4). obtained by puncture with a thin needle may distin- The partial cecal resection with linear stapler cut- guish benign and malignant forms, but it should not be ter, including the site of implantation of the appendix, Mucocele of the appendix Free base (up to 2.0 cm) Compromised base (>2.0 cm) Lesion in the cecum Appendectomy + (no biopsy) Lymphanedectomy Typhlectomy Frozen section Benign Histopathological Malignant Right colectomy Figure 1. Computed tomography: cystic image adjacent to the cecum. Follow-up Figure 3. Treatment protocol of mucocele. Figure 2. Colonoscopy: Protrusion of the appendiceal ostium, volcano sign (fi le: Dr. José Juvenal de Araújo). Figure 4. Mucocele of the appendix with a narrow base. 278 Journal of Coloproctology Mucocele of the appendix - appendectomy or colectomy? Vol. 31 July/September, 2011 Janduí Gomes de Abreu Filho et al.
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