Solid pseudopapillary neoplasm – management of an extremely rare case of pancreatic tumor in a young patient

Abstract

Introduction: Franz Tumor or the solid pseudopapillary neoplasm is a very rare form of pancreatic cancer, that can be held responsible for aproximately 0.2-2% of the exocrine pancreatic tumors.

Case report: We present the case of a 20-year-old woman, with no significant medical history, who is admitted to our department with abdominal discomfort in the left hypochondrium and epigastrium lasting  for the past 6 months. The imaging examinations performed show a large encapsulated mass, with mixed structure, in contact with the pancreatic tail, left kidney and left adrenal gland, though without being able to certainly identify its origin.

Management and results: Considering the above mentioned, surgical indication is established and a left subcostal laparotomy is performed; the intraoperative aspect is that of  a relatively well deliniated  mass, adherent at the level of the pancreatic tail,  therefore we proceed with an en-bloc excision of the encapsulated tumor and the distal portion of the pancreas. The histopathological examination reveals a tumor with solid and pseudopapillary architecture, combined with areas of hemorrhagic and cystic degeneration, well delimited by a pseudo-capsule; therefore we reach a final diagnosis of: Pseudopapillary-solid pancreatic neoplasm, pT3 stage.

Conclusions: Frantz Tumor is an extremely uncommon type of neoplasm, with asymptomatic evolution in most of the cases, which explains for late and mostly incidental diagnosis of these patients. With an appropriate case management, in spite of its often increased dimensions and malignant character, radical surgery is generally considered curative, with favorable long-term prognosis.