The pseudopapillary tumor of the pancreas (or Frantz's tumor) Tumeur cystopapillaire du pancréas (tumeur de Frantz) chez l'adolescent et l'adulte jeune

摘要

The pseudopapillary tumor of the pancreas (or Frantz's tumor) is a rare exocrine pancreatic tumor with a low degree of malignancy. It occurs more frequently among women between 20 and 40 years of age and in the Asian population. This tumor is rarer in the pediatric population. The symptoms are subtle, the most striking being pain and an abdominal mass. Pathologically, the tumor is usually well circumscribed with regions of necrosis, hemorrhage and cystic degeneration. A thick, fibrous capsule is often present. The low grade of malignancy of this tumor with a fibrous capsule led to perform a surgical resection. The localization and local invasion determine the surgical technique. Despite its potential for local infiltration and metastatic disease (up to 15% confined often to the liver), the prognosis is favorable after a surgical resection with correct margins. Long follow-up is necessary to detect a possible recurrence, even late.