Peritoneal carcinomatosis from solid pseudopapillary neoplasm (Frantz's tumour) of the pancreas treated with HIPEC

Solid pseudopapillary neoplasm (SPN) is a rare malignant tumour accounting for 0.1% to 2.7% of all pancreatic neoplasms and affecting young women. Peritoneal carcinomatosis (PC) is even rarer, with only 11 reported cases. We describe a twelfth case occurring 13 years after the resection of an SPN which ruptured peroperatively. This 35-year-old woman had first undergone complete cytoreductive surgery (CCRS) alone and disease had relapsed within 8 months. Ultimately, further CCRS was combined with hyperthermic intraperitoneal chemotherapy (HIPEC) with oxaliplatin and irinotecan. The patient is now alive and disease free 31 months after her last operation. In the literature, the surgical treatment of PC from an SPN has yielded disappointing results, with a 58% recurrence rate at intervals ranging from 1 to 19 years. As none of these patients developed distant metastases, indicating a strictly peritoneal disease, HIPEC might be a solution for preventing such recurrences.