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Solid pseudopapillary tumor associated with agenesis of the dorsal pancreas: A case report
Apresentação do caso
A 36-year-old woman sought medical assistance due to a palpable epigastric mass, she denied any other symptom. The patient had a history of insulin-dependent diabetes, diagnosed 10 years earlier. Abdominal computed tomography (CT) showed an 7.8cm × 5.5cm diameter complex mass arising from the head of the pancreas’ site, and absence of the dorsal pancreas. A radiological diagnose suggested a Solid-pseudopapillary tumor (SPT). The patient was referred for surgical treatment. On peritoneal cavity inventory, the body and tail of the pancreas were absent, and a large tumor was noted at the head of the pancreas infiltrating into the second part of the duodenum and adhered to the superior mesenteric and portal veins. Hence, gastroduodenopancreatectomy with complete resection of the pancreas was performed and a Roux-en-Y reconstruction was carried out. She showed satisfactory improvement on the first few days and was discharged home on sixth postoperative day. The pathological examination and immunohistochemistry confirmed the SPT diagnosis.
SPT is a rare type of exocrine tumor, which usually has a low malignancy rate, is associated with few or no-symptoms and has an excellent prognosis after surgical removal. The standard treatment is surgical resection. Approximately 95% of the patients were reported to be disease free after surgical treatment. Agenesis of the dorsal pancreas (ADP) is an extremely rare condition. Pancreas development is a complex process and involves the fusion of two buds that originates from the endodermal lining of the duodenum. ADP its caused by the absence or regression of the dorsal bud during embryonic development, which would develop into the cranial portion of the head, body, and tail.
Erotokritou et al. published a review and reported a case that demonstrated the association between ADP and a neuroendocrine tumor. They found fifteen similar cases involving different tumors, of which only two were SPT. Nakamura et al. published the first case report, but they described a partial pancreatic dorsal agenesis since the Santorini duct was detected on endoscopic retrograde cholangiopancreatography. The second case was published by Ulusan et al., this time presenting a complete ADP. In our case, the dorsal pancreas was not identified on abdominal CT, which indicates a true ADP case associated with an SPT.
PANCREATIC NEOPLASMS; PANCREATIC CANCER; PANCREATECTOMY; PANCREAS AGENESIS
Trato gastrointestinal alto
DIOGO VIANA ABREU, DANIEL PAULINO SANTANA, HENRIQUE ARAUJO LIMA, NATHALIA GABRIELA SOARES MORATO, JOAO LUCAS DE CARVALHO GOMES, VINICIUS NASCIMENTO FERREIRA, ALEXANDRE CARVALHO ABUD, MARCO RINOLDI, MARIA ELISA SOARES SILVEIRA CARDOSO, MATTEUS MURTA LAGE