Clinicopathologic characteristics and surgical treatment of solid pseudopapillary tumor of the pancreas

Nursing Department, Zhejiang Cancer Hospital, Hangzhou, China.
Hippokratia (Impact Factor: 0.37). 08/2013; 17(1):68-72.
Source: PubMed


Background and aim:
Solid pseudopapillary tumor (SPT) of the pancreas is a very rare neoplasm of low malignant potential that mostly affects young women. The aim of the present study is to report our experience in surgical treatment of SPT and review of the literature.

Material and methods:
A retrospective review of three cases of SPT who were treated at our department during the last two years was performed. The clinicopathologic characteristics, surgical treatment, and prognosis are described in detail.

Case 1 described an asymptomatic SPT in a pregnant woman. To the best of our knowledge, only one case of SPT in pregnancy has been reported in the literature. Case 2 described an SPT in the pancreatic tail causing splenic infarction, and a distal pancreatectomy combined with splenectomy was performed. Case 3 described an SPT in the pancreatic head, for which a pancreatoduodenectomy was successfully performed. All of the three patients were followed up for 10-22 months without recurrence or metastases after the initial surgery at the time of reporting.

At present, radical resection is the treatment of choice for SPT. Enucleation can be performed for tumors with complete amicula. Distal pancreatectomy combined with or without splenectomy can be performed for pancreatic body and/or tail tumor, and pancreatoduodenectomy for pancreatic head tumor. The prognosis of SPT is good.

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    ABSTRACT: Solid-pseudopapillary tumor of the pancreas (SPT) has distinctive morphologic and biologic features but an unclear origin. It is classified among the pancreatic epithelial tumors, though many are reported to be negative for cytokeratin. Also unclear are its neuroendocrine differentiation, its capability to express alpha-1-antitrypsin (AAT) and, in view of the tumor's striking prevalence in women, its relationship with the female genital tract. To clarify these issues, the immunoprofiles of 59 SPTs were defined by applying a battery of antibodies against cytokeratin, vimentin, neuron-specific enolase (NSE), synaptophysin, chromogranin A, tyrosine hydroxylase (TH), AAT, LeuM1, Ki-M1P, smooth-muscle actin, CD34, alpha-inhibin, calretinin, placental alkaline phosphatase (PLAP), and progesterone and estrogen receptors. The most consistent markers with the strongest immunoreactivity were vimentin, AAT, NSE, and the progesterone receptor, which were each found in more than 90% of the tumors. Using immunocytochemical methods involving antigen retrieval, cytokeratin was demonstrated in almost 70% of the cases. Synaptophysin was found in 22% of the tumors, while chromogranin was absent and tyrosine hydroxylase was only present in a few tumors. None of the other markers tested were expressed by SPTs. This staining pattern fails to reveal a clear phenotypic relationship with any of the defined cell lineages of the pancreas. In view of the striking female preponderance of SPTs and the known close approximation of the genital ridges to the pancreatic anlage during embryogenesis, it is, however, hypothesized that SPTs might derive from genital ridge/ovarian anlage-related cells, which were attached to the pancreatic tissue during early embryogenesis.
    Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin 06/2000; 436(5):473-80. DOI:10.1007/s004280050475 · 2.65 Impact Factor
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    ABSTRACT: To investigate differential points of solid-pseudopapillary neoplasm (SPN) of the pancreas and pancreatic endocrine tumor (PET). Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features, immunostaining reactions and beta-catenin gene mutations were analyzed. The mean age of SPN patients was 25.6 years and these patients had no specific symptoms. The mean diameter of the tumors was 11.0 cm, 9/10 cases were cystic or a mixture of solid and cystic structures, and there was hemorrhage and necrosis on the cut surface in 8/10 (80%) cases. Characteristic pseudopapillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of beta-catenin and loss of E-cadherin in all the cases, was only seen in SPN. Molecular studies discovered that 9/10 (90%) cases harbored a point mutation of exon 3 in beta-catenin gene. On the other hand, the mean age of PET patients was 43.1 years. Eight of 14 cases presented with symptoms caused by hypoglycemia, and the other 6 cases presented with symptoms similar to those of SPN. The mean size of the tumors was 2.9 cm, most of the tumors were solid, only 3/14 (21%) were a mixture of solid and cystic structures, and macroscopic hemorrhage and necrosis were much less common (3/14, 21%). Histologically, tumor cells were arranged in trabecular, acinar or solid patterns and demonstrated no pseudopapillary structure and discohesive appearance in all 14 (100%) cases. The results of immunostaining and mutation detection were completely different with SPN that membrane and cytoplastic expression of beta-catenin without loss of E-cadherin, as well as no mutation in beta-catenin gene in all the cases. Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary, immunostaining of beta-catenin and E-cadherin is quite helpful to make the differential diagnosis.
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    ABSTRACT: Solid pseudopapillary tumors (SPTs) are unusual neoplasms of the pancreas of uncertain histogenesis that occur mostly, but not exclusively, in young women. The pathologic features and immunophenotype of SPT are unique and well characterized. Despite its low malignant potential, proximately 15% of patients with SPT develop metastatic disease, mostly involving the liver or peritoneum. Even in the presence of disseminated disease, the clinical course is usually protracted, and the overall 5-year survival is reportedly 97%. We have encountered 2 cases of SPT possessing unusual pathologic features and exhibiting an aggressive clinical course. At the time of presentation, 1 patient had liver metastasis, and the other had a lymph node metastasis and developed liver metastases within 3 months. Both died of disease at 6 and 16 months, respectively, following the initial diagnosis. Review of other cases of SPT treated at Memorial Sloan-Kettering Cancer Center (New York, NY) revealed that 5 of 34 cases (15%) with conventional histologic features developed liver metastases. In contrast to the 2 cases reported here, all 5 patients survived for a mean of 106 months (39-193 months), and only 2 died of disease 5 and 10 years, respectively, following the initial resection. The pathologic features of the two rapidly fatal cases, which might have been indicative of their aggressive behavior, included a diffuse growth pattern, extensive tumor necrosis, significant nuclear atypia, an unusually high mitotic rate (35-70/50 high power fields), and in one a component of sarcomatoid carcinoma. However, regions displaying the typical histologic features of SPT were also evident. Abnormal beta-catenin distribution and markedly increased MIB1 expression were detected by immunohistochemistry in both cases. The immunohistochemical staining patterns were otherwise similar to those of conventional SPTs. Although precise pathologic criteria suggesting a high risk for aggressive behavior are uncertain, recognition of some of the unusual pathologic features displayed in these 2 cases may be useful in the prediction of potentially more aggressive SPTs. The possibility that these tumors represent high-grade malignant transformation of a conventional low-grade SPT is proposed.
    American Journal of Surgical Pathology 05/2005; 29(4):512-9. · 5.15 Impact Factor
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