Appendiceal mucocele: Case reports and ... literature CASE REPORT

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World J Gastroenterol 2008 April 14; 14(14): 2280-2283
World Journal of Gastroenterology ISSN 1007-9327
© 2008 WJG. All rights reserved.
CASE REPORT
Appendiceal mucocele: Case reports and review of current
literature
Kemal Karakaya, Figen Barut, Ali Ugur Emre, Hamdi Bulent Ucan, Guldeniz Karadeniz Cakmak, Oktay Irkorucu,
Oge Tascilar, Yucel Ustundag, Mustafa Comert
Kemal Karakaya, Ali Ugur Emre, Hamdi Bulent Ucan,
Guldeniz Karadeniz Cakmak, Oktay Irkorucu, Oge Tascilar,
Mustafa Comert, Department of General Surgery, Zonguldak
Karaelmas University, Zonguldak 67600, Turkey
Figen Barut, Department of Pathology, Zonguldak Karaelmas
University, Zonguldak 67600, Turkey
Yucel Ustundag, Department of Gastroenterology, Zonguldak
Karaelmas University, Zonguldak 67600, Turkey
Author contributions: Karakaya K designed the research;
Karakaya K, Ucan HB, Cakmak GK, Tascilar O, Barut F
performed operations and pathologic examination; Karakaya K
and Ustündag Y wrote the paper; Karakaya K, Emre AU, Irkorucu
O acquisited data and literature; Comert M and Ustündag Y
revised the paper critically for important intellectual content.
Correspondence to: Kemal Karakaya, Department of General
Surgery, Zonguldak Karaelmas Universite Hastanesi, Zonguldak
67600, Turkey. [email protected]
Telephone: +90-372-2610169 Fax: +90-372-2610155
Received: December 20, 2007 Revised: February 17, 2008
Abstract
The mucocele of the appendix is an uncommon disorder which is often asymptomatic but sometimes causes
acute appendicitis-like symptoms. Sometimes, patients
with mucocele can present with confusing symptoms.
Preoperative suspicion and diagnosis of appendiceal mucocele are important. Ultrasonography and computed tomography are useful tools for the diagnosis of appendiceal mucocele. It may be also recognised by colonoscopy
as a smooth submucosal lesion of the cecum. Optimal
management of the mucocele could be achieved through
accurate preoperative diagnosis. Preoperative diagnosis
is a major component for minimizing intra-operative and
post-operative complications. We herein report five cases
and discuss the diagnostic methods and surgical treatment.
© 2008 WJG . All rights reserved.
Key words: Appendix; Mucocele; Diagnosis; Surgical
treatment
Peer reviewer: Hugh J Freeman, Professor, Department of
Medicine, University of British Columbia, UBC Hospital, 2211
Wesbrook Mall, Vancouver, BC V6T 1W5, Canada
Karakaya K, Barut F, Emre AU, Ucan HB, Cakmak GK, Irkorucu O,
Tascilar O, Ustundag Y, Comert M. Appendiceal mucocele: Case
reports and review of current literature. World J Gastroenterol
www.wjgnet.com
2008; 14(14): 2280-2283 Available from: URL: http://www.wjgnet.com/1007-9327/14/2280.asp DOI: http://dx.doi.org/10.3748/
wjg.14.2280
INTRODUCTION
Appendiceal mucocele (AM) is a rare entity that can
present with a variety of clinical symptoms or occur as
an incidental surgical finding. The incidence is 0.2%-0.4%
of all appendectomied specimens[1-3]. AM is a progressive
dilatation of the appendix from the intraluminal accumulation of the mucoid substance[3,4]. It may be a benign or
malignant process. There are four histological types, which
lead to individualized surgical treatment and course in each
case[3].
Preoperative diagnosis that distinguishes AM from
acute appendicitis (AA) is essential for the best choice
of surgical approach (open vs laparoscopic) to prevent
peritoneal dissemination and perform the appropriate surgery[3,5]. Herein, we report 5 cases, discuss the diagnostic
procedures and management algorithm of these patients,
and review briefly the relevant literature. We also aimed to
define the incidence of the AM in a tertiary referral centre
in Northern Black Sea region of Turkey.
CASE REPORTS
Case 1
An 82-year-old man was admitted to the hospital because
of pain in the right lower quadrant of the abdomen for
3 d. Standard laboratory tests, serum levels of CA 19-9
and carcino-embryonic antigen (CEA) were within normal
ranges. Ultrasonography (USG) and computerized tomography (CT) demonstrated a well demarcated, elliptical 7 cm ×
5 cm cystic mass with parietal calcifications in the right lower
quadrant of the abdomen. There was an indentation in the
cecum by colonoscopy. Surgical exploration revealed the
mass to be an AM. Simple appendectomy was performed.
Pathological examination revealed a mucinous cystadenoma
with dimensions of 8 cm × 6 cm × 5.5 cm. AM restricted
to the appendix and cecum was free of the disease. The patient’s postoperative course was unremarkable, and he was
discharged home on the 4th postoperative day.
Case 2
A 65-year-old woman was referred to the emergency de-
Karakaya K et al. Appendiceal mucocele
2281
Figure 1 CT imaging of a soft
tissue mass indicated by black
arrows in the region of the cecum.
←
←
Case 3
A 66-year-old woman was referred to the endoscopy unit
for evaluation of a mass in the right lower quadrant of
the abdomen with the suspicion of malignant tumor. She
had visited another health care unit where the mass was
revealed by ultrasound examination. She had been suffering from decreased appetite, nausea, and weakness
lasting for a week. She had a history of upper GI hemorrhage managed medically 16 years ago and her surgical
history was significant for cholecystectomy 14 years ago.
Routine laboratory tests, including complete blood count
and serum chemistry were unremarkable. Serum levels
of CA19-9 and CEA were also within the normal ranges.
USG of the abdomen showed a heterogenic mass (95 mm
× 40 mm × 32 mm in dimensions) involving ileocecal part
of the intestines. CT imaging revealed a soft tissue mass
measuring 8 cm × 4 cm with peripheral enhancement in
the region of the cecum (Figure 1). The patient underwent
colonoscopy, which revealed a sub-mucosal protrusion to
the lumen of the cecum, in the region of the appendiceal
orifice. Orifice of the appendix was uncertain (Figure 2).
Remaining of the colon was unremarkable. The patient
was treated surgically. Surgical exploration revealed a mass
in the appendix whitish-grey in color (Figure 3). Resection
of cecum was followed by an ileocolic anastomosis. Frozen section examination revealed benign mucoid lesion.
Formal pathologic report was hyperplastic type mucocele
with evidence of secondary changes and chronic inflammatory mucosa of the intestine. The mucocele was 9.5 cm
× 4 cm × 2 cm in dimensions with a wall thickness of 2
←
←
←
partment with a diagnosis of AA. She complained of an
abdominal pain which started 3 d before referral to our
hospital. She had a history of coronary by-pass surgery 3
years ago. Physical examination showed diffuse peritoneal
irritation. Plain radiography showed gas-fluid levels in the
right lower quadrant. She also had mild leukocytosis. USG
and CT scans of the abdomen showed presence of free
fluid in the intra-abdominal spaces.
An emergency operation revealed ileal and cecal mesenteric ischemia. Partial ileum and cecum resection was
performed. Histopathological diagnosis was ischemia of
the intestine and simple mucocele of the appendix with a
diameter of 5 mm, incidentally. Postoperative recovery was
uneventful and she was discharged home on the 7th postoperative day.
←
Figure 2 Colonoscopic view of the sub-mucosal mass.
Figure 3 Intra-operative view of the AM. Arrows indicate the mucine filled appendix.
mm. There was no evidence of malignancy. Postoperative
course was unremarkable and she was discharged home on
the 5th postoperative day.
Case 4
A 58-year-old man was referred to the general surgery
department for surgical treatment of toxic multi-nodular
goitre. He had multiple previous admittances to internal
medicine department and emergency department because
of pain in the right lower quadrant of the abdomen and
anemia. Physical examination was negative for abdominal
mass or perianal or rectal lesions. The patient underwent
endoscopy to investigate the etiology of anemia. Colonoscopy revealed a submucosal cecal mass. Abdominal CT
revealed a polipoid a soft tissue mass measuring 4 cm ×
3 cm with peripheral enhancement in the region of the
cecum. The patient underwent total thyroidectomy and appendectomy. Histopathological diagnosis was benign cystadenoma without cecal involvement. Postoperative course
was unremarkable and he was discharged home on the 6th
postoperative day.
Case 5
A 72-year-old man underwent an open inguinal herniorrhaphy due to recurrent right inguinal hernia. He had
complained of bulging and right groin pain which were
exacerbated with activity. An appendiceal mass was defined
during laparoscopic herniorrhaphy. Open access to the
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2282
ISSN 1007-9327
CN 14-1219/R
World J Gastroenterol
abdominal cavity was chosen. Simple appendectomy was
performed with a clinical suspicion of appendiceal mucocele. Frozen section examination revealed appendiceal
mucocele. Pathological examination reported a mucinous
cystadenoma measuring 12.5 cm and 5.5 cm without cecal
involvement. Postoperative course was unremarkable and
he was discharged home on the 7th postoperative day in
good conditions.
DISCUSSION
Mucocele of the appendix is an uncommon tumor, with
an incidence of 0.29%-0.4% of all appendectomied specimens[1-3,6]. There has been no exact reported incidence of
AM in Turkey. Histopathological examinations revealed 5
patients with AM among 240 patients who underwent appendectomies from January 2001 to October 2007 at our institution. The incidence of AM is revealed as 2.01%, which
is higher than that reported in literature[7,8]. This may be
related to the fact that our centre is a tertiary referral centre.
Although a small portion of AM is asymptomatic,
clinical manifestations include right lower abdominal pain,
palpable abdominal mass, or gastrointestinal bleeding in
majority of the AM[1,3,7,9-12]. Among the five cases reported
above, three had abdominal pain secondary to mucocele
while one had symptoms related to groin hernia and the
last patient had abdominal pain due to intestinal infarction.
AM was an incidental finding for those two patients.
USG, CT and colonoscopic examinations can facilitate
preoperative diagnosis of AM[1,13-15]. Ultrasound is the firstline diagnostic modality for patients with acute abdominal
pain or mass. Different sonographic findings of AM and
AA have been described [3,16,17]. Appendix diameter 15
mm or more in USG examination has been determined
as the threshold for AM diagnosis with a sensitivity of
83% and a specificity of 92%[3]. Outer diameter threshold
for AA diagnosis has been established as 6 mm[18]. USG
examination revealed a cystic mass in the right lower
quadrant in two of our patients. These findings revealed
suspicion of AM.
CT is also an effective diagnostic tool for AM. CT can
determine the relation between lesion and the neighbouring organs, and help confirm the diagnosis[15,16,19,20]. CT
reveals a cystic mass with enhancing wall nodularly in the
expected area of the appendix, especially in older patients,
in whom AM should be considered[9,15,16,21]. AM could be
visualized by CT in three of our patients. CT examination
was normal in respect of AM for the third patient. AM
was an incidental finding for this lady. Radiological investigation was not performed for the patient who had herniorrhaphy for recurrent groin hernia.
USG and CT findings are non-specific and the differential diagnosis should be established with benign appendiceal neoplasms and other pathologies such as carcinoid,
lymphoma, mesenteric cysts, ovarian masses, and malign
neoplasms of the appendix[7,22]. We did not perform fine
needle aspiration as dictated in the literature in order to
avoid dissemination of the mucus leading to pseudomyxoma peritonei[16].
Colonoscopy in patients with abdominal pain is a use-
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April 14, 2008
Volume 14
Number 14
ful tool for determination of mucocele[2,23]. Generally, an
elevation of the orifice of the appendix is seen. A yellowish mucous discharge would be visible from appendiceal
orifice during colonoscopy. It is also important for the
diagnosis of synchronous or metachronous colon tumor
which would be as high as 29%[1,2,7,13-15,24,25]. Colonoscopic
examinations on three of our patients revealed indentation in the cecum due to AM. The remaining colon was
unremarkable in all of them. The 4th patient in emergency
conditions was treated surgically based on the diagnosis of
mesenteric ischemia and the 5th patient underwent surgery
with the diagnosis of groin hernia without colonoscopic
examination.
The spontaneous and surgery induced complications
of AM include intestinal obstruction, intussusceptions[20,22],
intestinal bleeding[11,13,25], fistula formation[15,26], and volvulus[27,28]. The worst complication is pseudomyxoma peritonei, characterized by peritoneal dissemination caused by
iatrogenic or spontaneous rupture of the mucocele[5]. The
tissues should be handled carefully during surgery in order
to avoid rupture of the mucocele. Thus, conventional surgery is preferred rather than laparoscopic approaches for
the treatment in our cases[2,5,8,22]. Laparoscopic approach
has an increased risk of rupture and subsequent pseudomyxoma peritonei formation[2,5,8]. Moreno et al[5] suggest
conversion to an open appendectomy in case of mucocele
when laparoscopic appendectomy is intended. Few authors
still recommend a minimally invasive approach in selected
patients for this rare entity[9,27,29]. However, in these reports, laparoscopic approach has been adopted for a small
number of patients. Thus, we need a large series to substantiate recommendations of laparoscopic approach.
A simple and thorough evaluation of these patients
with a new algorithm has been suggested by Dhage-Ivatury and Sugarbaker[30]. Simple appendectomy is the choice
of surgical treatment for patients with benign mucocele
that has negative margins of resection without perforation.
No long term follow-up is needed for these patients[2,8,27,30].
Appendectomy was performed for three of our patients
with a mucocele limited to the appendix. For patients with
perforated mucocele, with positive margins of resection,
positive cytology and positive appendiceal lymph nodes,
right colectomy/cytoreductive surgery (CRS)/heated
intraperitoneal chemotherapy (HIIC) and early postoperative intraperitoneal chemotherapy (EPIC) should be
performed. Long term follow-up is obligatory for these
patients[5,24,30,31].
Perforated mucocele with positive margins of resection, positive cytology, and negative appendiceal lymph
nodes necessitate cecectomy/CRS/HIIC and EPIC. Long
term follow-up is also obligatory for these patients[2,30,32].
Cecectomy had been performed for one of the patients
to obtain negative surgical margins, since the appendiceal
wall was contiguous with the cecum and the intraoperative
pathology indicated benign mucocele. Long term followup in this case has been carried out in the outpatient clinics. Perforated mucocele with positive cytology but negative margins of resection and negative appendiceal lymph
nodes should be treated with appendectomy/CRS/HIIC
and EPIC[26,30]. We did not apply these treatment modali-
Karakaya K et al. Appendiceal mucocele
ties as none of our patients had had positive lymph nodes
or perforated mucocele.
13
CONCLUSION
14
Although a rare disease, surgical treatment of the AM is
mandatory because of the potential for malignant transformation and prevention of pseudomyxoma peritonei
due to rupture of the mucocele itself. Therefore, preoperative diagnosis or suspicion is required for carefully planned
resection to excise the tumor. The incidence of AM in a
tertiary referral centre in Northern Black Sea region of
Turkey is revealed as 2.01%, which is higher than the incidence reported in the literature.
15
16
17
18
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