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 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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Sociedad Española de Patología Digestiva
2010
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oai:scielo:S1130-010820100012000092011-06-29Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigmaCienfuegos,J. A.Lozano,M. D.Rotellar,F.Martí,P.Pedano,N.Arredondo,J.Bellver,M.Sola,J. J.Pardo,F. Solid-pseudopapillary tumor Pancreatic cystic neoplasm Prognosis Beta-catenina 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.Sociedad Española de Patología DigestivaRevista Española de Enfermedades Digestivas v.102 n.12 20102010-12-01journal articletext/htmlhttp://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082010001200009en |
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Cienfuegos,J. A. Lozano,M. D. Rotellar,F. Martí,P. Pedano,N. Arredondo,J. Bellver,M. Sola,J. J. Pardo,F. |
| spellingShingle |
Cienfuegos,J. A. Lozano,M. D. Rotellar,F. Martí,P. Pedano,N. Arredondo,J. Bellver,M. Sola,J. J. Pardo,F. Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma |
| author_facet |
Cienfuegos,J. A. Lozano,M. D. Rotellar,F. Martí,P. Pedano,N. Arredondo,J. Bellver,M. Sola,J. J. Pardo,F. |
| author_sort |
Cienfuegos,J. A. |
| title |
Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma |
| title_short |
Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma |
| title_full |
Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma |
| title_fullStr |
Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma |
| title_full_unstemmed |
Solid pseudopapillary tumor of the pancreas (SPPT): Still an unsolved enigma |
| title_sort |
solid pseudopapillary tumor of the pancreas (sppt): still an unsolved enigma |
| description |
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. |
| publisher |
Sociedad Española de Patología Digestiva |
| publishDate |
2010 |
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http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082010001200009 |
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