Appendiceal Mucocele and Pseudomyxoma Peritonei; The Clinical Boundaries of a Subtle Disease

Abstract

Patient: Male, 70 • Male, 84Final Diagnosis: Appendiceal mucocele and pseudomyxoma peritoneiSymptoms: —Medication: —Clinical Procedure: —Specialty: SurgeryObjective:Rare diseaseBackground:Mucocele of the appendix is an uncommon cystic lesion characterized by distension of the appendiceal lumen with mucus. Most commonly, it is the result of epithelial proliferation, but it can also be caused by inflammation or obstruction of the appendix. When an underlying mucinous cystadenocarcinoma exists, spontaneous or iatrogenic rupture of the mucocele can lead to mucinous intraperitoneal ascites, a syndrome known as pseudomyxoma peritonei.Case Report:We report 2 cases that represent the clinical extremities of this heterogeneous disease; an asymptomatic mucocele of the appendix in a 70-year-old female and a case of pseudomyxoma peritonei in an 84-year-old male. Subsequently, we review the current literature focusing to the optimal management of both conditions.Conclusions:Mucocele of the appendix is a rare disease, usually diagnosed on histopathologic examination of appendectomized specimens. Due to the existing potential for malignant transformation and pseudomyxoma peritonei caused by rupture of the mucocele, extensive preoperative evaluation and thorough intraoperative gastrointestinal and peritoneal examination is required.

Mesothelial inclusion cyst: a rare occurrence

“…However, generally the risk of cyst rupture is higher in a laparoscopic approach than in an open approach [6]. As cystadenocarcinoma is a differential for cystic neoplasms of the appendix, rupture of any undifferentiated cyst associated with the appendix may place a patient at risk of pseudomyxoma peritonei [7]. Therefore, whilst laparoscopy may reduce early morbidity, a low threshold for conversion to open to facilitate safe dissection and avoid cyst rupture should be employed.…”

Clinicopathological analysis of patients operated with appendiceal mucocele

“…[3] A simple retention cyst determined by the intraluminal accumulation of mucoid material is rarely greater than 2 cm. Mucosal hyperplasia, a mild dilatation, constitutes 5%-25% of mucoceles.…”

“…[2] It was first described by Rokitansky in 1842. [3] Mucocele of the appendix can be categorized in four histological types, including retention cyst, mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma. [4,5] The affected patients are usually above the age of 50 years.…”

Mucinous appendiceal neoplasm: A case report

“…Mucinous appendiceal neoplasms represent a rare and poorly understood heterogeneous pathology that is present in less than 0.3% of all appendectomies[ 1 , 2 ]. They have a very low incidence in the general population, with age at diagnosis between 50 and 60 years and a male-to-female ratio slightly in favor of the latter[ 1 , 3 ]. These types of neoplasms are incidentally discovered during appendectomies or in routine imaging for other conditions[ 4 ].…”

“…Complications depend on tumor size and histological type and can range from intestinal obstruction and bleeding to spontaneous perforation and peritoneal dissemination causing pseudomyxoma peritonei (PMP)[ 3 , 6 ].…”