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Open-access Tumor sólido pseudopapilar de páncreas: abordaje quirúrgico de una neoplasia rara. Reporte de caso

Solid pseudopapillary tumor of the pancreas: surgical approach to a rare neoplasm. Case report

Problem:  Solid pseudopapillary tumor of the pancreas, also known as Frantz-Gruber tumor, is an extremely rare pancreatic neoplasm that accounts for less than 1 % of pancreatic tumors. It predominates in young women and is usually diagnosed incidentally through imaging studies.

Clinical case:  We present the case of a 24-year-old patient with symptoms of dyspepsia, nausea, and gastric outlet obstruction. Imaging studies showed a 69x59 mm solidocystic lesion in the head of the pancreas with signs of infiltration into the lesser curvature of the stomach and the adjacent peritoneum with multiple mesenteric lymphadenopathies.

Findings:  A pancreaticoduodenectomy with Roux-en-Y reconstruction was performed. Histopathological examination confirmed the diagnosis of solid pseudopapillary tumor with positivity for nuclear β-catenin and progesterone receptors. The resected specimen did not show evidence of infiltration into other organs, and the surgical margins were negative (R0). The patient had a favorable postoperative course and was discharged without major complications. She has been followed up as an outpatient without evidence of recurrence to date.

Conclusion:  Surgery remains the treatment of choice, with a 5-year survival rate of over 90 %, despite metastatic disease. This case underscores the importance of timely diagnosis and a comprehensive surgical approach to optimize the clinical outcome of these patients.

Keywords:
Solid tumor; pseudopapillary; Frantz-gruber tumor; pancreatic neoplasia; pancreaticoduodenectomy; low malignancy

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Instituto Tecnológico de Santo Domingo Santo Domingo DN, Dominican Republic Av. Los Próceres, Jardines del Norte. P.O. Box 342-9 and 249-2 - E-mail: manuel.colome@intec.edu.do
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