Added 06 March 2013
Treatment Options by Histologic Subtype:
Due to the rarity of these tumours, clinical trials are lacking, and most treatments are based on consensus opinion.
Mucinous Cystadenoma
Standard appendectomy with resection of the appendiceal mesentery is adequate surgery for a cystadenoma that shows no sign of mesenteric involvement or extension to adjacent organs. Patients found unexpectedly at the time of laparotomy or laparoscopy to have a mucinous appendiceal tumour should simply have an appendectomy. More extensive surgery (right hemi, omentectomy etc) increases the technical difficulty of the subsequent definitive operation without any acute benefit to the patient. There is no role for chemotherapy or radiation therapy with these tumours.
Pseudomyxoma peritonei
Special consideration should be given for cytoreductive surgery and intraperitoneal chemotherapy in patients with PMP. Repeat surgical debulking can be used for symptom control. For patients of lower functional status consideration should be given to best supportive care and hospice referral.
Goblet cell carcinoma
Stage I tumours can be treated with appendectomy alone, while higher stages of disease should be treated with right hemicolectomy to permit adequate nodal sampling. There is no data to support the role of adjuvant systemic therapy. In the metastatic setting typical regimens used to treat colon adenocarcinoma, such as FOLFOX and FOLFIRI chemotherapy have been suggested. In selected cases with peritoneal disease, cytoreductive surgery and intraperitoneal chemotherapy could be considered in specialized surgical centres. Long term prognosis with metastatic disease is poor and consideration should be given to best supportive care with referral to hospice.
Adenocarcinoma (mucinous, colonic-type, signet ring) and Cystadenocarcinoma
Right hemicolectomy is suggested for adenocarcinoma and cystadenocarcinoma in order to determine nodal status and should follow standard procedures as for colorectal cancer. Adjuvant, 5-FU based chemotherapy is suggested for node positive disease, based on extrapolated colon cancer data. In the metastatic setting with peritoneal disease, cytoreductive surgery and intraperitoneal chemotherapy should be considered in highly selected patients at specialized surgical centres. Otherwise palliative chemotherapy, as in colon cancer, should be considered. For patients of lower functional status, best supportive care and hospice referral should be pursued.
