Malignant neoplasms of the appendix

Appendiceal neoplasms, first described in 1882, are still rare, with pre-operative diagnosis invariably difficult. We present our 10-year experience of these lesions with a review of current epidemiology, pathology and treatment modalities. A retrospective histopathological review of all appendicectomy specimens was completed between April 1994 and December 2003 to identify patients diagnosed with malignant neoplasms. Patient demographics, operative details, histopathology and clinical outcomes were obtained from case notes. A literature search of the PubMed database was then performed using the medical search headings; appendix, tumour, neoplasm and malignancy. Twenty-two patients (eight men) were identified during the study period, with no age difference between gender (mean age in women 58, range 14-83 vs mean age in men 55, range 16-78). Eleven patients were found to have carcinoid-type tumours, eight patients with adenocarcinomas and three patients with lymphomas. Other appendiceal pathologies were identified after appendicectomies, hemicolectomy and oophorectomy. Mean follow-up was 41 months (range 1-125 months). Fourteen patients were alive at the end of follow-up. Patients with classical carcinoid tumours (CCT) had better outcomes than patients with the goblet cell carcinoid, adenocarcinoma and lymphoma. From our own experience and a subsequent review of the literature, we recommend right hemicolectomy as the treatment of choice for all malignant appendiceal neoplasms, except for small CCT less than 2 cm in diameter at the tip of the appendix, with a low proliferative index, without angiolymphatic or mesoappendiceal extension. Further adjuvant therapy should be considered after oncological assessment.