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 approximately 0.2-2% of the exocrine pancreatic tumors. Case report: We report a case of a 20-year-old woman, without any comorbidities, admitted to our department accusing mild abdominal pain at the level of the left hypochondrium and epigastrium, that started approximately half a year ago. Computed tomography and magnetic resonance imaging showed a voluminous encapsulated tumor, with mixed structure, apparently adherent to the tail of the pancreas, left kidney and adrenal gland, though being unable to certainly establish its visceral origin. Management and results: After analyzing all aspects of the case, we decide in favor of a left subcostal laparotomy approach; the intraoperative aspect is that of a relatively well delineated mass, adherent at the level of the pancreatic tail, therefore a complete excision of the tumor alongside with the pancreatic tissue that came in contact with it was performed. The pathological analysis reveals an encapsulated tumor with solid and pseudopapillary structure, with hemorrhagic and cystic degeneration regions; therefore, we reach the following final diagnosis: pT3 stage pseudopapillary-solid pancreatic neoplasm. Conclusions: In spite of its malignant behavior and impressive tumor volume, the surgical intervention was curative with favorable prognosis.