Solid pseudopapillary tumor associated with agenesis of the dorsal pancreas: a case report

ABSTRACT

Solid pseudopapillary tumors (SPTs) of the pancreas are rare tumors with low potential for malignancy, uncertain lineage, and favorable prognosis in most cases. The SPT has an excellent prognosis, and the standard treatment is surgical resection. The agenesis of the dorsal pancreas (ADP) is an extremely rare type of congenital pancreatic malformation and is characterized by partial or total loss of the body and tail of the gland. Its association with SPT has been reported only in two studies. We report a case of SPT associated with total ADP. A 36-year-old woman was diagnosed with a complex mass on pancreatic head topography, measuring 7.8×5.5cm, associated with complete agenesis of the body and tail of the pancreas. She underwent gastropancreatoduodenectomy with a successful postoperative outcome. The anatomopathological examination suggested an SPT and it was confirmed by immunohistochemistry.

RESUMO

Os tumores sólidos pseudopapilares (TSPs) do pâncreas são tumores raros com baixo potencial de malignidade, linhagem incerta e prognóstico favorável na maioria dos casos. O SPT tem excelente prognóstico e o tratamento padrão é a ressecção cirúrgica. A agenesia dorsal do pâncreas (ADP) é um tipo extremamente raro de malformação congênita do pâncreas e é caracterizada pela perda parcial ou total do corpo e cauda da glândula. Sua associação com TSP foi relatada apenas em dois estudos. Relatamos um caso de TSP associado à ADP total. Uma mulher de 36 anos foi diagnosticada com uma massa complexa na topografia da cabeça do pâncreas, medindo 7,8×5,5cm, associada à agenesia completa do corpo e cauda do pâncreas. Ela foi submetida à gastropancreatoduodenectomia com sucesso pós-operatório. O exame anatomopatológico sugeriu TSP e foi confirmado por imunohistoquímica.

Keywords:

Pancreatic neoplasms - Pancreatic cancer - Pancreatectomy - Pancreas - Agenesis.

Descritores:

Neoplasias pancreáticas - Câncer pancreático - Pancreatectomia - Pâncreas - Agenesia

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