The Investigation of 14 Appendiceal Mucocele Cases Encountered in 4850 Appendectomy Patients.

BACKGROUND: Appendiceal mucocele is a rarely seen disease which occurs when the appendix lumen is filled and obstructed by mucous. In our study, we aimed to reveal the surgical approach of our clinic, features of tumors, and clinical presentations in line with literature in cases of appendix mucocele. METHODS: Fourteen appendix mucocele patients who were admitted in our hospital between 2012 and 2019 were examined retrospectively in the electronic recording medium. Our patients were evaluated in terms of age, gender, clinical status, operation, imaging results, and pathology results. RESULTS: Of the fourteen patients, 12 applied to the emergency department and 2 to the general surgery clinic. All of our patients had abdominal pain at the time of admission. In the physical examination, 5 (35%) patients had defenses, 10 (71%) patients had rebound tenderness, and 12 (85%) patients had tenderness. In preoperative imaging studies, 11 patients were interpreted as having acute appendicitis and 3 patients were evaluated as having appendix mucocele. The pathological results were reported as 6 patients had appendiceal mucocele and 8 patients had appendiceal mucocele together with acute appendicitis. CONCLUSION: Appendiceal mucocele is a disease which generally causes similar clinical findings of acute appendicitis. Ultrasound and CT may be useful in preoperative diagnosis. Surgical treatment options of mucocele are open or laparoscopic appendectomy, cecum resection, and right hemicolectomy. Although its incidence is low, due to pseudomyxoma peritonei, it is a pathology that requires careful surgery.

Appendiceal tumours and pseudomyxoma peritonei: Literature review with PSOGI/EURACAN clinical practice guidelines for diagnosis and treatment

Pseudomyxoma Peritonei (PMP) is a rare peritoneal malignancy, most commonly originating from a perforated epithelial tumour of the appendix. Given its rarity, randomized controlled trials on treatment strategies are lacking, nor likely to be performed in the foreseeable future. However, many questions regarding the management of appendiceal tumours, especially when accompanied by PMP, remain unanswered. This consensus statement was initiated by members of the Peritoneal Surface Oncology Group International (PSOGI) Executive Committee as part of a global advisory role in the management of uncommon peritoneal malignancies. The manuscript concerns an overview and analysis of the literature on mucinous appendiceal tumours with, or without, PMP. Recommendations are provided based on three Delphi voting rounds with GRADE-based questions amongst a panel of 80 worldwide PMP experts.

Strategies for Preventing Pseudomyxoma Peritonei After Resection of a Mucinous Neoplasm of the Appendix.

BACKGROUND/AIM: Considering the suspected link between mucinous neoplasm (MN) and pseudomyxoma peritonei (PMP), one option could be to propose prophylactic hyperthermic intraperitoneal chemotherapy (HIPEC) to selected patients in order to prevent the occurrence of PMP. The aim of this study was to identify risk factors for developing PMP after curative surgery for a MN of the appendix. PATIENTS AND METHODS: All consecutive patients referred to our single tertiary care cancer center between September 1992 and March 2014 for MN of the appendix, initially without PMP, were retrospectively studied. Patients who had received prophylactic intraperitoneal treatment were excluded from the analysis. RESULTS: Twenty-five patients with a median age of 51 (range=25-74) years were identified. At the initial appendectomy, 19 (76%) patients had a perforated MN. Nine of these patients (40%) exhibited disseminated-free acellular intraperitoneal mucin without PMP. Six (24%) patients had an unperforated MN without free intraperitoneal mucin. After a median follow-up of 50 months, 12 (52%) patients had developed PMP after a median time of 61 (range=13-121) months. Complete cytoreductive surgery plus HIPEC was possible in only seven (64%) out of these 12 patients. No factor reached statistical significance in predicting the occurrence of PMP but a trend was found in the case of perforated MN (p=0.068), associated with a 65% recurrence rate, compared to 17% without perforation. CONCLUSION: An appendicular MN cannot be considered a benign occurrence because PMP is common after resection and can occur up to 10 years after the initial appendectomy. Patients should be systematically followed up to detect PMP as early as possible. Patients with a perforated MN could be at higher risk of developing PMP.

Management of a ruptured mucinous mesenteric cyst with hyperthermic intraperitoneal chemotherapy.

Mesenteric cysts are rare intra-abdominal cysts that are generally regarded as benign, and the incidence of malignancy is often cited to be 3%. The typical recommendation for treatment is complete excision to minimize recurrence. Excision can be performed laparoscopically, but this can lead to intra-abdominal dissemination of the cyst contents. There has been one case report describing the development of pseudomyxoma peritonei following rupture of a mesenteric cyst. We describe the treatment and outcome of a patient who underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy for the treatment of an incompletely resected mucinous cystadenocarcinoma originating from the colonic mesentery.

Classification of and cytoreductive surgery for low-grade appendiceal mucinous neoplasms.

BACKGROUND: Low-grade appendiceal mucinous neoplasm (LAMN) is a precursor lesion for pseudomyxoma peritonei (PMP), which, if treated suboptimally, may later disseminate throughout the abdominal cavity. The role of cytoreductive surgery for these relatively early lesions is unclear. METHODS: Clinicopathological details and treatment outcomes of patients with a LAMN and disease limited to the appendix or immediate periappendiceal tissues, referred to a national treatment centre between 2002 and 2009, were evaluated prospectively. RESULTS: Of 379 patients with a diagnosis of PMP, 43 (median age 49 years) had LAMNs localized to the appendix and periappendiceal tissue. Thirty-two patients initially presented with symptoms of acute appendicitis or right iliac fossa pain. Two distinct lesions were identified: type I (disease confined to the appendiceal lumen) and type II (mucin and/or neoplastic epithelium in the appendiceal submucosa, wall and/or periappendiceal tissue, with or without perforation). Type I lesions were managed by a watch-and-wait surveillance policy with serial measurement of tumour markers and computed tomography in 14 of 16 patients. Seventeen of 27 patients with type II lesions underwent risk-reducing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy with low morbidity. After a median follow-up of 40 months, there was no disease progression in either treatment pathway. CONCLUSION: This study identified two LAMN subtypes. Type II lesions have pathological features of increased risk for dissemination and should be considered for risk-reducing cytoreductive surgery.

[Appendiceal tumors found at appendectomy]. / Diagnostic de tumeur appendiculaire lors d'une appendicectomie.

There are three main histologic types of appendiceal tumor: adenoma, adenocarcinoma, and neuroendocrine tumor. Neuroendocrine tumors (carcinoids) are by far the most common and account for two-third of all appendiceal tumors. Adenomas and adenocarcinomas are both rare; they share two particularities: (a) a mucinous component is both frequent and predominant, (b) they have a tendency to intraperitoneal dissemination. Rupture of any mucinous tumor-whether spontaneous or occurring during surgery-may result in pseudomyxoma peritonei; treatment of this condition requires complete resection of all lesions followed by hyperthermic intraperitoneal chemotherapy. For unruptured appendiceal tumor, the appendix should be removed by a carcinologic right hemicolectomy if the tumor appears aggressive.

Appendiceal mucocele of mucinous cystadenocarcinoma with a cutaneous fistula.

We report a novel case of cystadenocarcinoma forming an appendiceal mucocele with development of a skin fistula. The patient was a 75-year-old Japanese woman who originally presented with a skin ulcer on the right flank (inferior to the ribs and superior to the iliac bone) with mucus discharge. The serum concentration of carcinoembryonal antigen was elevated (57.4 ng/ml). Ultrasonography and computed tomography demonstrated a cystic mass with septations in the right iliac fossa. Fistulography from the skin ulceration showed a communication via the fistula to the caecum. A right hemicolectomy and enbloc resection of the skin fistula was performed. The histological findings revealed a well-differentiated mucinous cystadenocarcinoma of the appendix. The patient has been alive for 7 years following surgery without any sign of recurrence. This report is of interest as it demonstrates that tumour rupture to the extraperitoneal space could result in a good outcome by preventing the development of pseudomyxoma peritonei.

Appendiceal mucinous cystadenoma presenting as "porcelain" appendix with myxoglobulosis--a rare cause of a right lower quadrant mass.

Mucinous cystadenoma is a rare tumour of the vermiform appendix and is associated with cystic dilatation of the appendix, to which the more general term of mucocoele has been applied. Mucocoele of the appendix is only a descriptive term for abnormal mucus accumulation causing distension of the appendiceal lumen, irrespective of the underlying cause. Pre-operative diagnosis of appendix mucocoele, though infrequently made, is important as some of these lesions may be malignant, and also is essential in order to avoid the risk of rupture at surgery with subsequent development of pseudomyxoma peritonei. The appearances of mucocoele of the appendix and its uncommon variant of myxoglobulosis on plain radiograph, ultrasound and barium study are presented, together with review of the literature.