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      Solid-pseudopapillary tumor of pancreas in a young woman: a case report and literature review Translated title: Tumor sólido pseudopapilar de páncreas: Presentación de un caso

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          Abstract

          Solid-pseudopapillary tumor of pancreas is a rare neoplasm which occurs pre-dominantely in young females. This tumor generally is asymptomatic or minimally symptomatic and some imaging tests are useful for its diagnosis. We report a 17 years-old woman with dyspeptic symptoms submitted to an abdominal ultrasound (US), that revealed a hypoechogenic mass between the liver, right kidney and pancreas. Computer tomography (CT) scan showed a hypodense and heterogeneous mass on pancreas head. The patient underwent elective resection of the mass and the histopathology was consistent with a solid-pseudopapillary tumor of pancreas. Immunohistochemical positivity for NSE (neuron-specific enolase), progesterone receptor, alfa-1-antitrypsin, vimentin, AE1/AE3, and negativity for synaptophysin and chromogranin A confirmed the diagnosis. After four years of clinical follow-up, the patient remains in a good general condition without signs of tumor recurrence.

          Translated abstract

          El tumor sólido pseudopapilar de páncreas es una neoplasia poco frecuente, que ocurre predominantemente en mujeres jóvenes. Este tumor generalmente es asintomático o mínimamente sintomático y las imágenes son útiles para su diagnóstico. Presentamos una mujer de 17 años de edad con dispepsia que fue sometida a una ecografía abdominal que reveló una masa tumoral hipoecogénica entre el hígado, el riñón derecho y el páncreas. La tomografía computarizada mostró una masa hipodensa y heterogénea en la cabeza del páncreas. La paciente fue sometida a resección electiva de la masa y la histopatología fue consistente con el tumor sólido pseudopapilar de páncreas. La inmunohistoquímica fue positiva para NSE (enolasa neuronal específica), receptor de progesterona, alfa-1-antitripsina, vimentina, AE1/ AE3, y negativa para sinaptofisina y cromogranina A, lo que confirmó el diagnóstico. Después de cuatro años seguimiento clínico, la paciente permanece en buen estado general sin signos de recurrencia del tumor.

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          Most cited references49

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          Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic ductal adenocarcinomas and almost always harbor beta-catenin mutations.

          Solid-pseudopapillary tumors (SPTs) are unusual pancreatic neoplasms of low malignant potential that most frequently affect young women. Genetic events contributing to the development of SPTs are unknown. Whereas the more common ductal adenocarcinomas of the pancreas essentially never harbor beta-catenin or APC gene mutations, we have recently identified alterations of the APC/beta-catenin pathway in other nonductal pancreatic neoplasms including pancreatoblastomas and acinar cell carcinomas. We analyzed a series of 20 SPTs for somatic alterations of the APC/beta-catenin pathway using immunohistochemistry for beta-catenin protein accumulation, direct DNA sequencing of beta-catenin exon 3, and direct DNA sequencing of the mutation cluster region in exon 15 of the APC gene in those SPTs that did not harbor beta-catenin mutations. Immunohistochemical labeling for cyclin D1 was performed to evaluate the overexpression of this cell-cycle protein as one of the putative downstream effectors of beta-catenin dysregulation. In addition, we analyzed the SPTs for genetic alterations commonly found in pancreatic ductal adenocarcinomas, including mutations in the K-ras oncogene and p53 and DPC4 tumor suppressor genes, using direct DNA sequencing of K-ras and immunostaining for p53 and Dpc4. Almost all SPTs harbored alterations in the APC/beta-catenin pathway. Nuclear accumulation of beta-catenin protein was present in 95% (19 of 20), and activating beta-catenin oncogene mutations were identified in 90% (18 of 20) of the SPTs. Seventy-four percent (14 of 19) showed overexpression of cyclin D1, ranging from 10 to 70% of tumor nuclei. In contrast, no K-ras mutations were present in any of the 20 SPTs, and Dpc4 expression was intact in all 16 SPTs for which immunohistochemical labeling was successful. Overexpression of p53 was limited to only 3 of 19 (15.8%) SPTs. These results emphasize the two distinct, divergent genetic pathways of neoplastic progression in pancreatic ductal and nonductal neoplasms.
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            Solid pseudopapillary tumor of the pancreas: a review of 553 cases in Chinese literature.

            To sum up the clinical and pathological characteristics of solid pseudopapillary tumor (SPT) and the experience with it. A total of 553 SPT patients reported in Chinese literature between January 1996 and January 2009 were retrospectively reviewed and analyzed. The mean age of the 553 SPT patients included in this review was 27.2 years, and the male to female ratio was 1:8.37. Their symptoms were non-specific, and nearly one third of the patients were asymptomatic. Computed tomography and ultrasonography were performed to show the nature and location of SPT. Most of the tumors were distributed in the pancreatic head (39.8%), tail (24.1%), body and tail (19.5%). Forty-five patients (9.2%) were diagnosed as malignant SPT with metastasis or invasion. None of the clinical factors was closely related to the malignant potential of SPT. Surgery was the main therapeutic modality for SPT. Local resection, distal pancreatectomy and pancreatoduodenectomy were the most common surgical procedures. Local recurrence and hepatic metastasis were found in 11 and 2 patients, respectively, after radical resection. Four patients died of tumor progression within 4 years after palliative resection of SPT. The prognosis of SPT patients was good with a 5-year survival rate of 96.9%. SPT of the pancreas is a rare indolent neoplasm that typically occurs in young females. It is a low-grade malignancy and can be cured with extended resection. The prognosis of such patients is good although the tumor may recur and metastasize.
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              Solid pseudopapillary tumor of the pancreas: typical and atypical manifestations.

              The purpose of this pictorial essay is to illustrate the various appearances of solid pseudopapillary tumor of the pancreas. Solid pseudopapillary tumor of the pancreas is a rare neoplasm usually found in young women. Typical solid pseudopapillary tumor is characterized by a well-encapsulated mass with varying amounts of intratumoral hemorrhage. However, the tumor can have an atypical appearance, such as metastasis, ductal obstruction, parenchymal and extracapsular invasion, simulation of islet cell tumor, intratumoral calcification, and occurrence in a male patient. The typical and atypical manifestations of solid pseudopapillary tumor can be visualized with cross-sectional imaging.
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                Author and article information

                Journal
                rmc
                Revista médica de Chile
                Rev. méd. Chile
                Sociedad Médica de Santiago (Santiago, , Chile )
                0034-9887
                September 2012
                : 140
                : 9
                : 1179-1184
                Affiliations
                [02] Aracaju SE orgnameTiradentes University orgdiv1Laboratory of Morphology, Science and Technology Brazil
                [01] Aracaju SE orgnameFederal University of Sergipe orgdiv1University Hospital orgdiv2Department of Medicine Brazil
                [03] Aracaju SE orgnameFederal University of Sergipe orgdiv1Medical School Brazil
                Article
                S0034-98872012000900012 S0034-9887(12)14000912
                10.4067/S0034-98872012000900012
                23354641
                6dcff888-409e-4543-b968-7244dabcefb0

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 14 October 2011
                : 19 January 2012
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 30, Pages: 6
                Product

                SciELO Chile

                Categories
                CASE REPORTS

                Pancreas,progesterone,Pancreatic neoplasms,Receptors
                Pancreas, progesterone, Pancreatic neoplasms, Receptors

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