Added 06 March 2013
Appendiceal cystadenomas and cystadenocarcinomas are often found incidentally at the time of appendectomy or on CT imaging done for other reasons. Patients with goblet cell carcinomas most often present with acute or chronic abdominal pain. Patients with any of these appendiceal tumours can present with symptoms of acute appendicitis or chronic right lower quadrant pain. Patients with pseudomyxoma peritonei may be found incidentally at laparotomy, or present with increasing abdominal girth, inguinal hernia in men, or palpable ovarian mass in women.
In terms of a diagnostic work up, a CT scan of the abdomen and pelvis should be done to assess the extent of peritoneal and nodal involvement. Because of the high incidence of associated colonic neoplasms, colonoscopy is recommended at diagnosis in all patients. Imaging of the chest should be done if intra-abdominal metastases are found. Image guided percutaneous biopsy of the thickened peritoneum or the omentum can be used for diagnostic purposes. PET scans are not recommended for staging purposes. Pre-operative tumour markers carcinoembryonic antigen (CEA), CA 19-9 and CA-125 may be useful for future monitoring.
Given the rarity of these cancers, referral to BCCA is recommended.
Patients with simple node-negative cystadenomas do not require adjuvant therapy and can be followed by their surgeon.