Clinical experiences of solid pseudopapillary tumors of the pancreas in China

To discuss the clinical experiences of solid pseudopapillary tumors (SPTs) of the pancreas by summarizing clinical information of patients with this disease in China. Chinese literature concerning SPTs of the pancreas published between January 1996 and October 2006 were retrospectively reviewed and analyzed. A total of 390 cases had been reported, among which 47 were men, with a female to male ratio of 7.30:1. Mean age of the patients was 25.3 years old, more than 50 per cent were between 10 and 24 years. The mean diameter of the tumor was 8.4 cm (range, 2 cm-25 cm). There was no significant difference in patient age and tumor size between male and female. Major clinical presentations included abdominal pain or discomfort, and palpable abdominal masses, however, nearly one third of all patients were asymptomatic. The rate of pre-operative misdiagnosis was rather high. Those who tested positive to metastases or invasions, 14.4% of the patients were diagnosed as malignant SPTs. Sex, age, symptoms, tumor size and tumor markers were not significant clinical factors to predict SPTs with malignant potential. Surgical procedures mainly included pancreatoduodenectomy, distal pancreatectomy and local resection. Three patients developed local recurrence, and one patient developed hepatic metastasis, all within four years after tumor resection. Five patients with malignant SPTs died due to tumor progression within 25 months after surgery. Surgical resection is the most effective means for curing this rare tumor. Despite metastasis, a good satisfactory effect could be achieved by surgical debulking. At least 4-yearly follow-up is mandatory for all patients undergoing surgical resection.