“…These include its rarity (Remick et al, 1987;Acea Nebril et al, 1996), the predominance of older patients (Medgyesy et al, 2000;Maitra et al, 2001), the characteristic presentation (Wick et al, 1987;Hoff and Pazdur, 1999), including occasional symptoms that are secondary to hormone production (Taniguchi et al, 1973;Chejfec and Gould, 1977;Doherty et al, 1984) and the typical advanced stage at diagnosis (Matsui et al, 1991;Bennouna et al, 2000). The high proportion of oesophageal tumours is also in agreement with previous reports (Ibrahim et al, 1984;Remick et al, 1987); 30% of our patients and half of the cases reported in the literature had oesophageal tumours. In concordance with the literature, the colon and rectum was the leading location in our study, involved in 39% of the patients (Hoff and Pazdur, 1999).…”