Myxoglobulosis of the appendix is characterized by the presence of mucinous, often calcified, pear-like globules in the lumen of the appendix. The first case of mucocele with myxoglobulosis was described by Latham in 1897 in an autopsy case. The incidence of myxoglobulosis is between 0.35% and 8% of all mucoceles [2
]. In a study of 50,000 appendectomy specimens, Collins [5
] found eight cases of myxoglobulosis, or approximately one in 6,200 removed appendices. In contrast, Hollstrom [4
] reported an incidence of only one in 47,500 appendectomies.
Most cases of myxoglobulosis have been incidental findings at autopsy or laparotomy while a few have presented clinically as a “surgical abdomen,” most commonly acute appendicitis. Perforation of the appendix has not been an infrequent complication. However, the most usual complication is generally considered either peritonitis or pseudomyxoma peritonei [4
]. The factors leading to the transformation of mucin into globular bodies of myxoglobulosis are unknown. Various hypotheses have focused on the formation of a core, which then acts as a nidus for the concentric deposition of mucin, as the initiating event in the pathogenesis of the globules. Some authors propose the hypothesis of bacterial and necrotic epithelial debris origin and small mucinous masses putatively formed in dilated glandular crypts [2
]. Lubin and Berle [7
] in a report of two cases proposed that the core represented an organizing mass of mucin and granulation tissue originating in the appendiceal wall that broke off and underwent necrosis. In our case, examination of all the globules failed to reveal any viable cellular elements. The same general mechanisms requisite in the production of appendiceal mucocele are involved in the pathogenesis of myxoglobulosis. These mechanisms include (a) partial or complete obstruction of the lumen of the appendix and (b) continued mucin production by a normal or altered—for example, neoplastic—epithelium.
Therefore mucoceles of the appendix related to retention cysts, focal or diffuse mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma may present with myxoglobulosis. The majority of the cases of myxoglobulosis belongs to the category of mucosal hyperplasias and the retention cyst type of mucocele [6
]. Viswanath et al. [8
] reported a case of myxoglobulosis secondary to an occlusive membrane while Brustmann [9
] described another one associated with a proximal carcinoid. Lo and Kan [10
] described a case of appendiceal mucinous cystadenoma presented as “porcelain” appendix with myxoglobulosis, and Guionmet et al. [11
] reported a case of mucinous cystadenoma of the appendix with myxoglobulosis associated to cecal adenocarcinoma.
After thorough microscopic examination of the appendix, our case was diagnosed as myxoglobulosis due to mucosal hyperplasia, associated with ruptured diverticulum and acellular extra-appendiceal mucin deposits.