Hindawi Publishing Corporation
Case Reports in Medicine
Volume 2010, Article ID 745021, 3 pages
Myxoglobulosisof theAppendix:ACase Associated with
PanagiotisAroukatos,DionysiosVerras,GerassimosP. Vandoros,and MariaRepanti
Department of Pathology, “Agios Andreas” General Hospital, Tsertidou 1, 26 335 Patras, Greece
Correspondence should be addressed to Dionysios Verras, email@example.com
Received 26 March 2010; Accepted 11 August 2010
Academic Editor: Herman Terence Yee
Copyright © 2010 Panagiotis Aroukatos et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
We describe a case of the extremely rare entity of myxoglobulosis of the appendix from a 45-year-old white man who was
operated urgently with the clinical diagnosis of acute appendicitis. Sectioning of the appendix revealed the presence in the dilated
appendiceal lumen of numerous whitish opaque globules ranging in size from 0.2 to 0.7cm in diameter. A ruptured diverticulum
and several smaller ones were also found. On microscopic examination, the globules consisted of faintly eosinophilic laminations
of mucin surrounding an amorphous granular core. The mucin was identified by positivity with histochemical mucin stains.
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.
Our case is a “mucocele” of the appendix associated with
extensive mucosal hyperplasia and ruptured diverticulum
, with the rare accompanying finding of myxoglobulosis.
Mucocele alone does not constitute a rare finding in routine
appendectomy. However, the coexistence of multiple small
intraluminal globules, described in the past as “frog eggs” or
“fish eggs”, constitutes a special type of mucocele described
as “myxoglobulosis” .
A 45-year-old white man entered the hospital on October
19, 2009 and was operated urgently with the clinical
diagnosis of acute appendicitis. The pathology specimen
consisted of a dilated appendix measuring 6cm in length
and 2cm in diameter, adherent to the mesoappendix.
Sectioning of the appendix revealed the presence in the
dilated lumen of numerous whitish opaque globules ranging
in size from 0.2 to 0.7cm in diameter. The mesoappendix
near the distal portion of the appendix showed mucin
deposits and globules, which appeared to originate from a
ruptured diverticulum (Figure 1). Several smaller nonrup-
tured diverticula were also found. The entire appendix was
submitted for histological evaluation. Hematoxylin-Eosin
and histochemical stains Mucicarmine, Alcian Blue pH 2.5,
PAS, and Diastase-PAS were performed. Histologically, the
globules consisted of faint eosinophilic laminations of mucin
surrounding an amorphousgranular core.Allof theglobules
stained uniformly positive with mucicarmine, Alcian Blue
and PAS and were diastase resistant (Figure 2). Exuberant
hyperplasia of the appendiceal mucosa was also identified.
A diagnosis of Low-Grade Appendiceal Mucinous Neoplasm
(LAMN), Low Risk of Recurrence  was excluded, as
mucosal architecture was mainly preserved in all sections
and exhibited mild disarray. Focal gland serration and
increased mucin-producing cells were found in the upper
half of the mucosa, instead of slender filiform villi that
are usually observed in LAMNs. Due to concomitant acute
inflammation, focal epithelial crowding of the villi was
interpreted as reactive atypia rather than low-grade dys-
plasia. Around the ruptured diverticulum, mucin had been
extruded through the appendiceal wall and mesoappendiceal
body giant cell inflammatory reaction. In serial sections
examined microscopically, the periappendiceal mucin did
2 Case Reports in Medicine
Figure 1: Macroscopic appearance of the extruded mucin and
Figure 2: Alcian Blue positive staining of the globules, adjacent
luminal mucin and mucosa (Alcian Blue pH 2.5, x100).
not contain any epithelial elements. Thus the diagnosis of
acellular extra-appendiceal mucin was set. As no molecular
analysis was performed to exclude with certainty LAMN, a
recommendation was given that the patient be followed up
to exclude recurrence with mucinous ascites.
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, 4]. In a study of 50,000
appendectomy specimens, Collins  found eight cases of
myxoglobulosis, or approximately one in 6,200 removed
appendices. In contrast, Hollstrom  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 pre-
sented 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, 6]. The factors leading to the
transformation of mucin into globular bodies of myxoglob-
ulosis 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, 4, 6]. Lubin and Berle  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 req-
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 cys-
tadenoma, and mucinous cystadenocarcinoma may present
with myxoglobulosis. The majority of the cases of myx-
oglobulosis belongs to the category of mucosal hyperplasias
and the retention cyst type of mucocele . Viswanath et
al.  reported a case of myxoglobulosis secondary to an
occlusive membrane while Brustmann  described another
one associated with a proximal carcinoid. Lo and Kan 
described a case of appendiceal mucinous cystadenoma
presented as “porcelain” appendix with myxoglobulosis, and
Guionmet et al.  reported a case of mucinous cystade-
noma of the appendix with myxoglobulosis associated to
appendix, our case was diagnosed as myxoglobulosis due to
mucosal hyperplasia, associated with ruptured diverticulum
and acellular extra-appendiceal mucin deposits.
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Case Reports in Medicine3 Download full-text
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