Tumor de Franz: tres casos clínicos / Franz tumor: three clinical cases

RESUMEN

El tumor sólido seudopapilar del páncreas o tumor de Franz es una neoplasia poco frecuente de bajo potencial de malignidad. Afecta con mayor frecuencia a mujeres jóvenes y su pronóstico suele ser bueno tras la resección quirúrgica. Se estima una frecuencia del 1 a 2 por ciento entre todas las neoplasias exocrinas del páncreas. Crecen lentamente sin correlación exacta entre aspecto histológico y comportamiento biológico. Se presentan los casos de 3 pacientes con tumor de Franz; de 12,14 y 11 años, que consultaron principalmente por dolor abdominal. El primer caso se presentó con una apendicitis aguda en cuya ecotomografía preoperatoria se identificó el tumor pancreático. Los casos 2 y 3 consultaron por dolor abdominal, de 2 semanas y 48 h. de evolución respectivamente, cuyo estudio permitió plantear el diagnóstico y actuar en consecuencia. Los 3 fueron resueltos mediante cirugía con conservación esplénica, que resultó curativa en todos los casos. Un paciente presentó como complicación postoperatoria un pseudoquiste pancreático que se resolvió en forma espontánea. La histología en todos los casos fue compatible con tumor sólido seudopapilar del páncreas.

ABSTRACT

Solid pseudopapillary tumors of the pancreas (SPT) of Franz tumor is rare (1-2 percent of exocrine pancreatic tumors). This typically benign tumor is found mainly in young women usually asymptomatic. They may present with a gradually enlarging abdominal mass or complain of vague abdominal pain or discomfort. It can be an ultrasonographic finding in most cases. We present 3 patients operated of Franz tumors, one in 2004 and two in 2007. Case 1 12 y/o female, operated for acute appendicitis, whose which preoperatory ultrasonography (US) showed an image of solid-cystic tumor of the pancreatic tail. Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the diagnosis. She was operated a month later, with resection of a 9 cm tumor of the pancreatic tail, without splenectomy. She presented a pancreatic pseudocyst after surgery, that disappeared spontaneously. Case 2 15 y/o boy, that presented with 2 weeks of abdominal pain and vomiting. Physical examination was normal. US and CT showed a cystic tumor of the pancreatic tail. After serial CT, MRI and US, tumoral size reduction and a bigger solid component was confirmed. He was operated 11 months after the diagnosis, with resection of a 6 cm tumor, without splenectomy. Post operative progress was without complications. Case 3 11 y/o female, admitted with suspected acute appendicitis. US revealed an image of solid-cystic tumor of the pancreatic body and tail. CT and MRI confirmed the diagnosis of a 6.5 cm solid-cystic mass of the pancreas. She was operated 3 weeks later, with resection of a 7 by 6 cm tumor, without splenectomy. Post operative progress was without complications. In the three cases the diagnosis of SPT was confirmed by histopathology an malignancy was discarded. Close inspection of the surgical specimens in the three cases did not reveal capsular, vascular or nerve sheath invasion.