Clinicopathological analysis of appendiceal mucinous tumors: A single-center experience

Abstract

Clinicopathological analysis of appendiceal mucinous tumors: A single-center experience INTRODUCTIONAppendiceal mucocele is a pathological process occurring due to mucosal inflammatory or neoplastic diseases that result in the formation of an enlarged appendix and thinned appendicular wall caused by abnormal accumulation of mucus in the appendicular lumen, depending on any obstructive etiologic agent. It is at least two times more common in women than in men, and is only observed in 0.2%-0.7% of all appendectomy specimens (1-3). Although it is one of the most common primary epithelial tumors in the appendix, it is a rare clinical condition compared with other pathologies. This disease has unpredictable biological behavior in consideration of its outcomes. There is a large body of literature showing that mucocele is an important pathological condition as spontaneous or iatrogenic ruptures lead to pseudomyxoma peritoneum (PP) syndrome, of which a complete treatment algorithm has not been established so far. Four different types of formations widely accepted in the literature are retention cysts, mucosal hyperplasia, cystadenoma, and cystadenocarcinoma. Although clinical presentation of mucocele is nonspecific, it is incidentally detected during appendectomies, which are mostly performed for acute appendicitis. Cystadenoma and cystadenocarcinoma have more clinical significance because spontaneous or iatrogenic rupture of these can lead to PP syndrome. Opting for open laparotomy in the presence of a mucocele detected during laparoscopic procedures is extremely important to ensure that all spaces in the peritoneal cavity are explored and that the appendix wall remains intact and does not lead to PP syndrome, which is considered to be malignant and develops as a result of rupture (4). Understanding the clinical and pathological profile of patients with appendiceal mucocele can help the clinicians in better diagnosis and management of this rare condition. Therefore, in this retrospective study, besides the clinical, radiological, and pathological profile of the patients with mucocele, we also aimed to focus on the surgical management of appendiceal mucinous tumors according to different types. Objective: Appendiceal mucinous tumors can be encountered in four different types. The clinical approach varies according to these types and the severity of the present disease. We aimed to share clinical, radiological, and pathological features and surgical options of the patients diagnosed with mucinous tumors at our center. MATERIAL AND METHODS Material and Methods:Between August 2009 and March 2016, 757 patients underwent appendectomy for presumed diagnosis of acute appendicitis at the Department of Surgery, Kahramanmaraş Sütçü İmam University School of Medicine, Kahramanmaraş, Turkey. Among them, demographic, clinical, and histopathological characteristics of patients who diagnosed with appendiceal mucinous tumors were retrospectively analyzed. This study was approved by our faculty's human ethics committee.Results: Incidenc...

Histopathologic study of Mucinous lesions of the appendix

“…The most common clinical presentation is nonspecific abdominal pain as seen in 60% of cases similar to study by Emre et al 22 Abdominal pain is associated with mass in two cases. Appendicitis as clinical presentation is seen in 40% of all the cases in contrast to other studies by Omari et al 21 where only 8% to 14% of the cases presented as acute appendicitis.…”

Neoplasias benignas no carcinoides del apéndice cecal: presentación de 36 casos durante los últimos 17 años en un hospital de tercer nivel

“…Uno de los términos inicialmente utilizados para describir las tumoraciones mucinosas apendiculares fue la de «mucocele». El término «mucocele apendicular» fue definido por primera vez en 1842 por Karel Rokitansky como la dilatación del apéndice cecal con acumulación de material mucinoso en su interior 3 . Esto incluye una amplia variedad de tumores, que comprenden desde quistes simples de retención hasta adenocarcinomas invasivos, pero actualmente ha quedado obsoleto 6 .…”

“…Según el Peritoneal Surface Oncology Group International (PSOGI) y el último consenso del American Joint Committee on Cancer (AJCC), la terminología que debería utilizarse sería la de neoplasias no invasivas (adenoma, pólipo serrado con o sin displasia, neoplasia mucinosa apendicular de bajo y alto grado) y neoplasias invasivas (adenocarcinoma mucinoso, carcinoma de células en anillo de sello y adenocarcinoma no mucinoso) 3 .…”

“…El diagnóstico de sospecha preoperatorio es un factor importante, dado que orienta a determinar el tipo y la extensión de la cirugía que debe realizarse, pero en general esto es difícil debido a que en un gran número de pacientes el diagnóstico se realiza intraoperatoriamente o más tarde durante el estudio anatomopatológico 3 , como ocurrió en la mayoría de los casos de nuestra serie.…”

“…Son una afección rara, que corresponde al 0,2-0,7% del total de las muestras de apendicectomías y representa en torno a un 8-10% de todas las tumoraciones apendiculares. Suelen ser más frecuentes en las mujeres que en los hombres 3 .…”