Frantz's tumor: Is mutilating surgery always justified in young patients?

Abstract Background Solid pseudopapillary tumor (Frantz's tumor) of the pancreas is a rare lesion. It is of low-grade malignancy but can cause extensive local invasion. The aim of this study was to assess the outcome of Frantz's tumors after incomplete resection. Methods We contacted all authors who published case reports describing incomplete resection of Frantz's tumor between 1985 and 2008 to request follow-up information. Results Follow-up information was obtained for 11 out 18 patients who underwent incomplete resection. Estimated median survival rate was 5.7 years (69.5 months). Conclusion Since Frantz's tumor typically develops mainly in children and young women, a 5.7 year survival rate is unacceptable. Thus complete resection of locally invasive solid-pseudopapillary tumor of the pancreas is always justified, even at the price of difficult, mutilating surgery.