Solid pseudopapillary neoplasm of the pancreas
Department of Emergency Medicine, Mater Misericordiae University Hospital, Dublin, Ireland.Case Reports 10/2012; 2012(oct24_2). DOI: 10.1136/bcr.01.2012.5589
Solid pseudopapillary neoplasms (SPNs) are rare entities accounting for between 0.13 and 2.7 per cent of pancreatic tumours. This neoplasm has a predilection for females under the age of 35. The authors report this case of a SPN incidentally discovered when a 59-year-old female underwent a chest x-ray to investigate a wheeze. A subsequent CT abdomen revealed a 10 cm well-circumscribed mass adjacent to the tail of the pancreas. This mass was successfully resected. Immunohistochemical markers established the diagnosis of a SPN. The wheeze associated with the presentation of this case was unrelated to the tumour which was an incidental finding. These neoplasms are largely asymptomatic and indolent reaching a large size before detection. Diagnosis is confirmed on histology and in this case surgical resection was curative and there was no metastasis at presentation.
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ABSTRACT: Background and Aim: Clinicopathological data regarding pancreatic solid pseudopapillary tumors (SPT) in a multiethnic country are limited. The aim of the present study was to characterize pancreatic SPT in Australia.Methods: Clinicopathological features, treatment, immunohistochemical findings and outcome data of 34 patients (79% Caucasian, 12% Asian, 6% South Pacific Islander and 3% African) with pancreatic SPT were reviewed.Results: The most presenting complaint was abdominal pain. Median diameter of tumors was 60 mm (range: 20–220); predominantly located in the pancreatic tail (tail : body : head = 23:3:8). All tumors were resected and patients underwent surgery, including a liver resection for metastasis, all patients were alive after a median follow up of 70 months (IQR: 48–178). Two patients underwent repeated surgery for local recurrences with liver metastases after 8 and 18 months, which were successfully managed by surgical resection. Completeness of excision, perineural spread, vascular space invasion, mitotic rate and cellular atypia did not predict recurrence. In all cases, there was aberrant nuclear staining of beta-catenin and a loss of membranous expression of E-cadherin with aberrant nuclear localization of the cytoplasmic domain. Most pancreatic SPT were also strongly positive for CD10 (96%), progesterone receptor (79%), cytokeratin (28%), synapthophysin (26%) and chromogranin (15%).Conclusions: Pancreatic SPT occur in all races and are uniformly indolent. Given complete resection of a pancreatic SPT is usually curative and recurrences can be treated with re-operation, correct diagnosis is important.Journal of Gastroenterology and Hepatology 01/2011; 26(2):267 - 274. DOI:10.1111/j.1440-1746.2010.06466.x · 3.50 Impact Factor
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ABSTRACT: We describe the clinical, imaging and cytopathological features of solid pseudopapillary tumor of the pancreas (SPTP) diagnosed by endoscopic ultrasound-guided (EUS-guided) fine-needle aspiration (FNA). A 17-year-old woman was admitted to our hospital with complaints of an unexplained episodic abdominal pain for 2 mo and a short history of hypertension in the endocrinology clinic. Clinical laboratory examinations revealed polycystic ovary syndrome, splenomegaly and low serum amylase and carcinoembryonic antigen (CEA) levels. Computed tomography (CT) analysis revealed a mass of the pancreatic tail with solid and cystic consistency. EUS confirmed the mass, both in body and tail of the pancreas, with distinct borders, which caused dilation of the peripheral part of the pancreatic duct (major diameter 3.7 mm). The patient underwent EUS-FNA. EUS-FNA cytology specimens consisted of single cells and aggregates of uniform malignant cells, forming microadenoid structures, branching, papillary clusters with delicate fibrovascular cores and nuclear overlapping. Naked capillaries were also seen. The nuclei of malignant cells were round or oval, eccentric with fine granular chromatin, small nucleoli and nuclear grooves in some of them. The malignant cells were periodic acid Schiff (PAS)-Alcian blue (+) and immunocytochemically they were vimentin (+), CA 19.9 (+), synaptophysin (+), chromogranin (-), neuro-specific enolase (-), a1-antitrypsin and a1-antichymotrypsin focal positive. Cytologic findings were strongly suggestive of SPTP. Biopsy confirmed the above cytologic diagnosis. EUS-guided FNA diagnosis of SPTP is accurate. EUS findings, cytomorphologic features and immunostains of cell block help distinguish SPTP from pancreatic endocrine tumors, acinar cell carcinoma and papillary mucinous carcinoma.World Journal of Gastroenterology 11/2007; 13(38):5158-63. · 2.37 Impact Factor
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ABSTRACT: Solid pseudopapillary tumors of the pancreas are exceptional. The aims of our study were to reevaluate the mode of presentation of these tumors and to analyze the role of pathological examination in diagnostic assessment and prognostic evaluation. We report the clinical, radiological and pathological findings in a retrospective series of 13 patients in whom a diagnosis of solid pseudopapillary tumor of the pancreas was made between 1983 and 1997. There were 12 females (median age: 22.5 years) and one male, aged 73. The tumor was discovered incidentally (3 cases) or because of nonspecific digestive symptoms (10 cases). Biological data were uninformative. The tumor was pancreatic in 12 cases and duodenal in 1. In all cases, imaging techniques showed an heterogeneous lesion with no or poor vascularization. A cystic component was identified in 4 cases. Surgical resection was performed in all cases. Pathological examination showed an encapsulated tumor in 8 cases, a non-encapsulated but well-limited lesion in 3 cases and an infiltrative tumor in 2 cases. At the time of diagnosis, multiple liver metastases were present in 1 case. Mean duration of follow-up was 24 months (range: 3-168). At last follow-up, all patients, including the patient with synchronous metastatic disease, were alive, without local recurrence. Our study confirms that most cases of solid pseudopapillary tumors of the pancreas present with a suggestive clinical picture, including their occurrence in young women and their good prognosis after surgical resection. However, our results also underline the occurrence of cases presenting with unusual features, including old age, male sex, extra-pancreatic localization and malignant evolution. Histopathological examination is essential for the establishment of the diagnosis but morphological data are of little prognostic value.Gastroentérologie Clinique et Biologique 03/1999; 23(2):207-14. · 1.14 Impact Factor
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