Solid pseudopapillary tumor of the pancreas (SPPT). Still an unsolved enigma

Solid pseudo-papillary tumor (SPPT) is a rare cystic tumor of the pancreas (1-3% of exocrine tumors of the pancreas) which shows an “enigmatic” behavior on the clinical and molecular pattern. A retrospective analysis of the citological studies and resected specimens of pancreatic cystic tumors from...

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Authors: Álvarez-Cienfuegos, J. (Javier)|||/items/88871d37-0dae-4312-a6d8-0596bc69158c, Lozano-Escario, M.D. (María Dolores)|||/items/af4b6129-2049-4083-8d70-40d37f172598, Rotellar-Sastre, F. (Fernando)|||/items/833cb788-f4b9-404d-a18b-ae1c84369b51, Marti-Cruchaga, P. (Pablo)|||/items/97577c37-4c78-4a17-8b03-55eec0b40ff9, Pedano-Rodríguez, N. (Nicolás)|||/items/a1939853-b0df-4f87-8a31-528d81779ffe, Blasco-Blanco, M. (Manuel)|||/items/af19cc07-e126-47fa-b01b-e8cca89f8554, Bellver, M. (M.)|||/items/3cd4566b-929a-4292-8ec3-35b84f530827, Sola, J.J. (Jesús Javier)|||/items/5e9c3448-a06b-4c18-ac7a-1f314af02493, Pardo, F. (Fernando)|||/items/f4488eb3-3bf9-4dff-9d3f-402f36a1721e
Format: article
Publication Date:2010
Country:España
Institution:Universidad de Navarra
Repository:Dadun. Depósito Académico Digital de la Universidad de Navarra
Language:English
OAI Identifier:oai:dadun.unav.edu:10171/22414
Online Access:https://hdl.handle.net/10171/22414
Access Level:Open access
Keyword:Solid-pseudopapillary tumor
Pancreatic cystic neoplasm
Prognosis
Beta-catenina
Description
Summary:Solid pseudo-papillary tumor (SPPT) is a rare cystic tumor of the pancreas (1-3% of exocrine tumors of the pancreas) which shows an “enigmatic” behavior on the clinical and molecular pattern. A retrospective analysis of the citological studies and resected specimens of pancreatic cystic tumors from May 1996 to February 2010 was carried out. Three cases of SPPT were found, which are the objective of this study. The diagnosis was established upon occasional finding in the abdominal CT, in spite of sizing between 3 and 6 cm of diameter. In the three cases the preoperative diagnosis was confirmed by citology and specific immunohistochemical staining. Cases 2 and 3 showed strong immunoreactivity for Beta-Catenina and E-Cadherina staining. Radical resection (R0) was carried out in the three cases. A young male –21 years of age (case 1)- who had duodenal infiltration and two lymph nodes metastases died of hepatic and peritoneal recurrence 20 months following surgery. The other two cases are free of disease. The current review of the literature reports roughly 800 cases since the first report in 1959, and shows the enigmatic character of this tumor regarding the cellular origin, molecular pathways, prognostic factors and clinical behavior.

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