Mucocele of the appendix is a term used to describe a dilated, mucin-filled appendix. It is most commonly the result of epithelial proliferation, but can be caused. Appendiceal mucoceles occur when there is an abnormal accumulation of mucin causing abnormal distention of the vermiform appendix due to various. Appendiceal Mucocele: A Diagnostic Dilemma in Differentiating Malignant From Benign Lesions With CT. Hao Wang1, Yong-Qi Chen2, Ran Wei1, Qing-Bing.
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Surgical Treatment of Appendiceal Mucocele. | Oncology | JAMA Surgery | JAMA Network
To receive news and publication updates for Case Reports in Oncological Medicine, enter your email address in the box below. A corrigendum for this article has been published. To view the corrigendum, please click here. This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Appendiceal mucocele is a rare cause of acute abdomen.
Mucinous appendiceal neoplasms represent 0. The aim of this study is to report a case of a mucinous appendiceal neoplasm presented as acute appendicitis, discussing the clinical and surgical mucoceke in the emergency setting. A year-old female patient was admitted to the emergency department with a clinical examination indicative of acute abdomen. The patient underwent abdominal computed tomography scan which revealed a cystic lesion in the right iliac fossa measuring 8.
The patient was taken to the operating room and a right hemicolectomy was performed. The apppendiceal course was unremarkable. The histopathological examination revealed a low-grade mucinous appendiceal neoplasm with negative regional lymph nodes. Ultrasound and CT are useful in diagnosing appendiceal mucocele and synchronous cancers in the emergency setting.
The initial operation should include appendectomy and resection of the appendicular mesenteric fat along with any fluid collection for cytologic examination. During urgent appendectomy it is important to consider every mucocele as malignant in order to avoid iatrogenic perforation causing pseudomyxoma peritonei.
Although laparotomy is recommended, the laparoscopic approach is not contraindicated. Appendiceal mucoceles AM or mucinous neoplasms are rare lesions characterized by a distended and mucus-filled appendix.
Historically, Rokitansky in was the first who described the appendiceal mucocele as a dilatation of the appendiceal lumen by an abnormal accumulation of mucus [ 6 ]. The appendix epithelium contains many goblet cells and thus the accumulation of mucus is a typical finding. Because of this mucus-producing epithelium, the most common epithelial tumors of the appendix are mucinous and begin as mucoceles [ 7 ].
Appendiceal mucoceles are historically classified into four histologic subgroups: The clinical presentation is rather unspecific. Most of these AM are asymptomatic but can become symptomatic because of inflammation, presenting as an acute appendicitis or by causing nonspecific abdominal pain.
In young patients assuming an acute appendicitis, the preoperative diagnosis is rare. In older patients a preoperative diagnosis by computed tomography CT scan is more probable, which can easily detect the AM or even pseudomyxoma peritonei [ 8 ]. The preoperative diagnosis of AM helps to avoid accidental iatrogenic perforation during surgery.
This is very important because it can lead to pseudomyxoma peritonei, characterized by peritoneal dissemination with high morbidity and mortality rate [ 9 ]. The aim of this study is to report a case of giant appendiceal mucocele presented as acute appendicitis, discussing the clinical and surgical approach in the emergency setting. A year-old female patient was admitted to the emergency department complaining of severe pain on her right lower quadrate RLQ of the abdomen with duration of 12 hours.
Her clinical examination was indicative of acute abdomen with a palpable mass in the right iliac fossa. Her past medical history included dyslipidemia, hypertension, chronic constipation, lower extremity varices, and a total left hip replacement. Standard laboratory examination showed mild leukocytosis with increase of neutrophil count. There was a cystic lesion in the right iliac fossa measuring 8. The lesion had calcified walls.
There were also multiple air bubbles with air-fluid levels. Minimal fat stranding was evident around the lesion. There were no enlarged regional lymph nodes or free intraperitoneal air.
The patient was taken to the operating room after being given intravenous antibiotics cefuroxime 1. Taking into consideration the size of the lesion, the possibility of a malignant neoplasm and the prospective of an emergency right hemicolectomy in an elder patient, the open approach was decided.
A midline incision was made and the peritoneal cavity was entered. There were no other significant findings. Frozen section was not available at the time of the operation. The age of the patient and the large appendiceal mass put a high suspicion of malignancy. In this context we decided to proceed with a right hemicolectomy.
The gastrointestinal tract continuity was established by laterolateral stapled ileotransverse anastomosis. The histological examination revealed a low-grade mucinous appendiceal neoplasm with negative regional lymph nodes and without presence of mucinous peritoneal carcinomatosis. In addition two small tubular adenomas of the ascending colon with low-grade epithelial hyperplasia were revealed. No further surgical therapy was required. Oncologic consultation was recommended to the patient.
Medical oncology did not recommend adjuvant chemotherapy. One year postoperatively the patient is still alive. We presented the diagnostic and therapeutic approach in the emergency setting of a case of a giant appendiceal mucinous neoplasm presented as acute appendicitis in an elderly patient.
Computed tomography scan is an important tool for the preoperative diagnosis in the emergency setting. However, the diagnosis of an appendiceal mucinous neoplasm is intraoperative and on histopathological examination. The term appendiceal mucocele refers to a dilated appendix with increased intraluminal accumulation of mucus.
Chronic obstruction of the appendix either by mucus or as a result appndiceal mucosal hyperplasia and benign or malignant neoplasms cause the appendiceal mucocele [ 112 ]. They are characterized by increase of the appendiceal diameter and epithelial villous adenomatous changes with epithelial atypia [ 1 ].
They demonstrate severe luminal distension and glandular stromal invasion with or without peritoneal implants of epithelial cells [ 112 ]. Mucocele of the appendix can also result from fecal impaction or polyps of the cecum which can obstruct the appendiceal ostium.
Rare causes found in the literature are endometriosis and metastatic melanoma [ 1314 ]. A consensus for classification and pathologic reporting appenciceal mucinous appendiceal neoplasia was recently appfndiceal [ 15 ]. Consensus was also achieved on the pathologic classification of pseudomyxoma peritonei PMP which was defined as the intraperitoneal accumulation of mucus due to mucinous neoplasia.
Pseudomyxoma peritonei was classified into three categories: Low-grade and high-grade PMP are synonymous to disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis, respectively. Recent reports showed a male predominance 5: However, AM are considered to occur more frequently in women [ 17 ]. In a retrospective study of patients by Omari et al.
Mucocele of the Appendix: Case Report and Review of Literature
Mucoceles prevail in the 5th and 6th decades of life, though they may be diagnosed at any age [ 3 ]. Other tumors of the gastrointestinal tract, ovary, breast, and kidney can be associated with the presence of AM in up to one-third of the patients [ 1018 ].
Other symptoms included abdominal pain, abdominal mass, weight loss, nausea or vomiting obstipation, and change in bowel habits. In the emergency setting AM can also be presented as intestinal strangulation, appendiceal intussusception, or mucoccele abdominal pain [ 51920 ].
Mucocele of the appendix
Although both the benign and malignant variants of AM may cause pseudomyxoma peritonei, this is more frequent and with worse prognosis for malignant cases [ 31021 ]. In a retrospective review study of Esquivel and Sugarbaker the most common initial symptom of patients with pseudomyxoma peritonei was appendicitis; nevertheless in none of these cases did the appendicitis occur as a first event of the dissemination [ 22 ].
Ultrasound and CT imaging studies are valuable for the detection of AM and can be easily performed in the emergency setting [ 161823 ]. Simple mucoceles have a mean diameter of 4. Computed tomography can be used in order to confirm the diagnosis and also allows for a better and precise study of the relation between the lesion and the neighbor organs [ 27 ]. In cases of acute appendicitis there appendicceal be an overlap with acute appendicitis without mucocele, though features suggestive of a coexisting mucocele include well-circumscribed cystic dilatation with low attenuation, mural calcification, and a luminal diameter greater than 1.
Fine needle aspiration should be avoided to preserve the integrity apprndiceal the cyst [ 27 ]. The optimal surgical approach for treating an appendiceal mucocele remains controversial.
The mucocsle for this approach is the resection of occult lymph nodal metastases within the ileocolic lymphatic system [ 7 ]. At the time of appendectomy in the appendideal setting, gross examination and the assessment of the size of the mucocele cannot reveal the malignancy of the lesion [ 31 ].
In these cases it is important to consider every mucocele of the appendix as malignant [ 7 ]. The laparoscopic approach has been described for the management of the appendiceal mucocele and is still recommended by some authors in selected patients [ 3233 ].
Single-port laparoscopic surgery for appendiceal mucoceles has also been reported to be safe and feasible [ 34 ]. The open approach permits a safe and gentle surgical manipulation of mucocel lesion. Furthermore port site recurrence after laparoscopic approach has been reported [ 35 ].
The initial operation should include appendectomy with en-block resection of the appendicular mesenteric fat and any fluid or mucus must be recovered for cytologic examination [ 736 ]. Inside the appendicular mesentery and along the appendiceal artery approximately four to eight mucoele are lying [ 7appendicesl ].
These lymph nodes should be submitted for frozen section and if negative, right hemicolectomy mucoceld not indicated. Furthermore a positive margin on the base of the appendix can be managed by cecectomy alone in order to obtain a negative margin and thus save the ascending colon and the ileocecal valve function [ 736 ]. However, in case there is high suspicion for malignancy, the resection should be complemented with right hemicolectomy [ 36 mkcocele. If a ruptured appendiceal mucocele is appendiceaal intraoperatively, then the primary resection should be accompanied by removal of all gross implants [ 735 ].
A complete abdominal exploration during the initial operation is indicated due to the occurrence of synchronous tumors and possible peritoneal seedlings. This wppendiceal is highly indicated when the surgery is performed with urgency and specific and accurate preoperative examinations have not been made [ 38 ]. After an initial urgent operation if the histological diagnosis reveals positive lymph nodes, adenocarcinoma of the intestine, mucinous adenocarcinoma, carcinoid or adenocarcinoid tumors larger than 2.
Patients with perforated AM in the initial surgery but with negative lymph nodes or margins in the histological diagnosis should not be appehdiceal for a right hemicolectomy as they present lower survival rates when compared to those who only had an mucoxele at the time of the primary surgery [ 41 ]. If the histological exam shows the presence of mucinous peritoneal carcinomatosis, then the patient will need cytoreductive surgery and hyperthermic intraperitoneal chemotherapy HIPEC with the prospective of a long-term survival [ 4243 ].
Low-grade tumors have the maximum survival benefit from these locoregional treatments [ 42 ]. Systemic chemotherapy before cytoreductive surgery and HIPEC may improve the prognosis in patients with peritoneal mucinous carcinomatosis [ 45 ].