Malignant Solid Pseudopapillary Tumors Research Articles

Synchronous Solid Pseudopapillary Tumor and Nonfunctioning Neuroendocrine Tumor in the Pancreas

Solid pseudopapillary tumor (SPT) is known to be cured in more than 95% of patients after surgery. However, it is reported that 7% of this tumor can recur after resection, and continued surveillance is needed after surgery for SPT. In this case report, a pancreatic mass which had been detected during a routine health checkup was followed up and surgical resection (distal pancreatectomy) was performed due to an increase in size. Postoperative pathology revealed synchronous SPT and neuroendocrine tumor (NET). At twelve months after surgery, a recurrence of the mass in the head of the remnant pancreas was noted and further surgical resection (total pancreatectomy) was performed. Postoperative pathology confirmed the diagnosis of malignant SPT. To date, there are only a few reports of the synchronous SPT and NET, and there are no reports in Korea. Therefore, we report a case of the synchronous SPT and nonfunctional NET diagnosed after surgery.

Solid pseudopapillary tumour of pancreas

Solid Pseudopapillary tumor (SPT) is a tumor with low malignant potential and comprises around 1-2% of all tumors occurring in Pancreas. Pseudocyst of Pancreas, Microcystic adenoma, neuroendocrine tumor and mucinous cystic neoplasm are considered as challenging differentials of SPT. High suspicion followed by accurate diagnosis is key to management because SPT is known for its good prognosis and successive cure rate on surgical resection. 21-year-old female patient presented with complaints of abdominal pain and dyspnoea. Imaging studies revealed a retroperitoneal tumor. Surgical resection carried out. Gross examination of the specimen showed circumscribed lobular brown mass weighing around 468 gm with a measurement of 15x12x10 cm. On sectioning, the tumor had a variegated appearance containing multiple cysts varying from 1 - 3 cm in size. Microscopic examination revealed an encapsulated cellular neoplasm with individual tumor cells arranged in solid, pseudopapillae and cystic patterns. Tumor cells were round to oval with no distinct nucleoli. Mitotic figures were very few in number. Hyaline globules were noted. Mucinous changes were seen focally. Occasional areas exhibited clear cell change of tumor cells. Cystic areas were filled with heamorrhage. Vascular areas were unremarkable. Normal Pancreatic parenchyma was noted at the tumor edge. Morphology of SPTs are varied ranging from solid to cystic appearance. No definitive morphology is identified to differentiate benign from malignant SPT. SPT are known to mimic a wide range of pancreatic and retroperitoneal lesions that may pose a challenge to diagnostic pathologists as seen in this case.

Surgical resection for malignant solid-pseudopapillary tumor: A case report

Surgical resection for malignant solid-pseudopapillary tumor: A case report

Solid pseudopapillary tumors of the pancreas: Specific pathological features predict the likelihood of postoperative recurrence.

Since their introduction in the WHO classification, the incidence of solid pseudopapillary tumors (SPTs) of the pancreas has progressively increased, mainly because of the widespread use of cross-sectional imaging. Few recent studies have analyzed the biological behavior of SPTs, but reliable data on long-term follow-up are needed. Retrospective analysis of two Institutions with high caseload, The Department of General Surgery-Pancreas Institute, University of Verona Hospital Trust and the Department of General Surgery, Massachusetts General Hospital, Harvard Medical School, was carried out. Data from 131 consecutive resections for SPT performed during the last three decades were collected and analyzed. The majority of patients were female (86.3%) with a median age of 33 (7-68) years. The prevalent location was the pancreatic tail (33.5%). Applying the WHO criteria, 16 (12.2%) SPTs were considered malignant due to the presence of at least pancreatic parenchyma (9.9%), perineural (4.6%), and/or angiovascular invasion (2.3%). After a median of 62 months after surgery, only two patients had a recurrence (1.5%). Both of them fulfilled the WHO criteria for malignant SPT (vs. 10.7% of those who did not recur, P = 0.01), had an infiltrative growth pattern (vs. 10.8%, P = 0.01), pancreatic parenchyma invasion (vs. 9.7%, P = 0.01) and capsular invasion (vs. 4.9%, P = 0.004). Overall, SPTs are associated with excellent survival results after surgical resection. Disease recurrence is extremely rare, and might occur if the primary tumor presents with either pancreatic parenchyma or capsule invasion. J. Surg. Oncol. 2016;114:597-601. © 2016 Wiley Periodicals, Inc.

Hyperaccumulation of (18)F-FDG in order to differentiate solid pseudopapillary tumors from adenocarcinomas and from neuroendocrine pancreatic tumors and review of the literature.

Solid pseudopapillary tumors (SPT) are rare, unique pancreatic tumors with benign entity and low malignant potential. Limited information is available in the literature reporting their accumulation of fluorine-18 fluoro deoxyglucose ((18)F-FDG) using positron emission tomography/computed tomography (PET/CT). The aim of this retrospective study was to define t he uptake-accumulation of (18)F-FDG PET/CT in a comparatively large cohort of SPT, and to compare their uptake with the uptake of (18)F-FDG in pancreatic ductal adenocarcinomas (PAC) and neuroendocrine tumors (PNET). Between June 2007 and January 2013, 18 pathologically proven SPT were identified from the total of patients studied by PET/CT in our Center, including 13 women and 5 men, aging from 23 to 56 years old (mean age, 38.5 years). Malignant SPT was histologically classified using the WHO criteria. Eighty-six PAC patients and 28 PNET patients were also identified and included in this study for comparison. Positron emission tomography results were considered as positive if focal accumulation of (18)F-FDG exceeded the surrounding normal pancreatic tissue. Regions of interest were drawn on the pancreatic lesions, and the maximal standardized uptake values (SUVmax values) were calculated. The mean values of SUVmax were compared with independent-samples t test or with the nonparametric Mann-Whitney U method. Correlation of SUVmax values and tumor size were analyzed in cases of SPT. Receiver operating characteristics (ROC curve) were used to study the efficiency of SUV values for the differential diagnosis between SPT versus (vs) PAC and SPT vs PNET. A value of P<0.05 was considered statistically significant. All SPT cases were (18)F-FDG-PET positive, with SUVmax values ranging from 3.5-18.3. The SUVmax values of SPT had poor correlation with tumor size, and no significant difference by gender and age. Areas under the curve ROC were 0.619 and 0.526, respectively for the differentiation of SPT from PNET and PAC tumors. Five SPT tumors were malignant, and exhibited relatively low (18)F-FDG uptake (SUVmax range, 3.0-4.5) except a tumor after recurrence (SUVmax 17.7). Images of CT were of low dose and thus were not evaluated. In conclusion, our results suggest that SPT benign or malignant are consistently hyperaccumulating (18)F-FDG above SUVmax 3. Differentiation from PAC and PNET if only based on the higher SUVmax values was not possible but if based on lower SUVmax, of ≤2.6 (in 14%) and ≤2.5 (in 21,4%) of PAC and PNET, respectively, these pancreatic tumors could be differentiated from SPT.

Natural History and Malignant Risk Factors of Solid Pseudopapillary Tumors of the Pancreas

Background and Aim: Solid pseudopapillary tumors (SPTs) of the pancreas are unusual neoplasms of uncertain prognosis. Most patients with SPTs have a good prognosis after undergoing surgical resection, but there are rare cases in which a locally infiltrative growth pattern and metastatic variety are exhibited, or recurrence of the disease after surgery occurs; these cases have been reported with very poor clinical outcomes. Our study investigated the natural history of SPTs and delineated the clinicopathologic features that may predict the malignancy potential of the disease. Methods: A total of 100 patients with suspected SPTs were enrolled in our study and 77 patients underwent surgical resection. A resulting 60 tumors were pathologically proven to be SPTs and the affected patients were followed-up regularly after surgery. Clinical and pathologic data for all 100 patients were analyzed. Results: Of the 60 total patients with histologically positive SPTs, 55 (92%) were women and 5 (8%) were men. The median patient age was 34 years (range, 13−77 years). Among the 60 patients, 9 had malignant SPTs and 51 had benign SPTs. Deep parenchymal invasion into the surrounding tissue was the most frequent pathologic feature suggesting malignancy (75%) among the 60 patients who underwent surgical resection. Patient clinicopathologic characteristics and demographic factors were compared between those who had benign SPTs and those who had malignant SPTs. There were no significant differences in the various patient features between the 2 groups, including age, sex, symptoms, tumor size, tumor location, internal tumor composition, pattern of tumor calcification, tumor necrosis, hemorrhage, and immunohistochemical tumor tissue patterns. There were 2 patients who had distant metastasis; 1 presented with distal metastasis in the liver and the other patient had recurrence of cancer with a peritoneal mass after surgery. Metastasectomy was performed on the 2 patients and there was no mortality or disease progression during the follow-up period (median, 143 months; range, 53−319 months). Conclusion: Solid pseudopapillary tumors are low-grade tumors that have a generally good prognosis. However, the clinical development and malignancy potential of SPTs are neither fully understood nor predictable, even with histologically benign tumors. Further investigations in tumor biology, along with long-term patient follow-up, may provide insight into the disease process and clinical development of SPTs.

Metastatic malignant solid pseudopapillary tumor of the pancreas

An 85-year-old Korean female presented with continuous weight loss. A physical examination revealed a soft, flat abdomen without a palpable mass or tenderness, and no enlargement of the superficial lymph nodes was detected. Her medical history was notable for diabetes. Routine laboratory tests and tumor markers including cancer antigen 19-9 (17.25 U/mL; normal range, < 34 U/mL) and carcinoembryonic antigen (CEA) (2.86 ng/mL; normal range, < 6.0 ng/mL) were within the normal ranges. Abdominal computed tomography (CT) demonstrated a 3.7-cm cystic mass with calcification in the head of the pancreas (Fig. 1A). In addition, there was a 3.4-cm lobulating solid mass with necrosis above the cystic mass that was located along the hepatic hilum. A 2.9-cm solid mass and a 1.4-cm cystic mass were observed at the right side of the liver and appeared to be metastatic tumors of the pancreas (Fig. 1). An ultrasound-guided biopsy was taken from the solid mass at the liver dome. Figure 1 Contrast enhanced abdominal computed tomography scans. (A) An axial scan shows a 3.7-cm sized cystic mass with peripheral curvilinear calcification in the head of pancreas (long arrow) and a 1.4-cm sized cystic mass with focal solid component that seems ... Tumor cells near the portal area were relatively uniform, with light pink foamy cytoplasm (Fig. 2A). The possibility of adenocarcinoma from the pancreatic duct was excluded by negative CEA and mucin-1 staining. Neuroendocrine markers were positive for synaptophysin and negative for chromogranin. β-Catenin and CD10 immunochemical staining, which are known markers of most solid pseudopapillary neoplasms, were positive (Fig. 2B). The final diagnosis was a malignant solid pseudopapillary tumor (SPT) of the pancreas with metastasis to the liver. Figure 2 (A) Light microscopy showing relatively uniform cells with light pink foamy cytoplasm and coordinated with pseudorosettes (H&E, × 100). (B) The tumor cells were stained with β-catenin which is known as the markers of most of solid ... SPTs are quite rare tumors usually observed in young females. This case was a malignant SPT in an elderly female patient that was confirmed by histological and immunohistochemical studies but was initially suspected to be a pancreatic ductal adenocarcinoma.

Management of a Malignant Case of Solid Pseudopapillary Tumor of Pancreas

Solid pseudopapillary tumor of the pancreas is a rare neoplasm with low malignant potential, which affects predominantly young females. Only approximately 10% to 15% cases of solid pseudopapillary tumors (SPTs) are malignant. We present the case of a 57-year-old woman who developed malignant SPT of the pancreas. Meanwhile, a literature review was carried out. Some clinicopathological features and strategies of management of malignant SPT are presented. In general, surgical removal of the tumor even in case of metastases or recurrence offers an excellent prognosis. Chemotherapy and radiotherapy should be taken into consideration in patients with unresectable tumor.

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.

Solid pseudopapillary tumor of the pancreas: a case series of 26 consecutive patients

Objective Solid pseudopapillary tumor (SPT) of the pancreas, which predominantly affects young women, is a relatively indolent entity with favorable prognosis. The aim of this study is to describe the clinicopathologic features and surgical management of this disease in our institution. Methods A retrospective study of clinical data from 26 consecutive patients with SPT managed in a tertiary academic center between January 2002 and December 2007 was performed. Clinicopathologic factors were compared between benign and malignant cases to determine what features of the tumor could suggest malignant potential. Results The 26 cases included 22 female and 4 male patients, and the average age was 32.3 years (range 15 to 64). Clinical symptoms were nonspecific and included upper abdominal pain or discomfort, abdominal distention, and back pain. The neoplasm was localized in the pancreatic head/neck in 14 patients and in the body/tail in 12 patients. The median diameter of these lesions was 6.25 cm (range 2 to 15). All of the tumors–including 8 pancreaticoduodenectomies, 10 distal pancreatectomies, 6 local resections, 1 total pancreatectomy, and 1 central pancreatectomy–were resected successfully. No patient received chemotherapy or radiotherapy after surgery. All of the patients except 1 were alive at a median follow-up of 32.5 months (range 3 to 69). One of the 2 patients with malignant SPT, in whom Ki-67 immunoreactivity was >25%, developed local recurrence with liver metastasis 4 months and died 6 months after surgery. There were no significant associations between clinicopathologic factors and malignancy. Conclusions SPT is a rare neoplasm with low malignant potential. Characteristic computed axial tomography and magnetic resonance imaging scans combined with age and sex profile should be sufficient for the decision to operate. Patients with malignant SPT should have careful follow-up. The high proliferative index assessed by immunohistochemical staining for Ki-67 may predict poor outcome of malignant SPT.

Clinical experiences of solid pseudopapillary tumors of the pancreas in China

To discuss the clinical experiences of solid pseudopapillary tumors (SPTs) of the pancreas by summarizing clinical information of patients with this disease in China. Chinese literature concerning SPTs of the pancreas published between January 1996 and October 2006 were retrospectively reviewed and analyzed. A total of 390 cases had been reported, among which 47 were men, with a female to male ratio of 7.30:1. Mean age of the patients was 25.3 years old, more than 50 per cent were between 10 and 24 years. The mean diameter of the tumor was 8.4 cm (range, 2 cm-25 cm). There was no significant difference in patient age and tumor size between male and female. Major clinical presentations included abdominal pain or discomfort, and palpable abdominal masses, however, nearly one third of all patients were asymptomatic. The rate of pre-operative misdiagnosis was rather high. Those who tested positive to metastases or invasions, 14.4% of the patients were diagnosed as malignant SPTs. Sex, age, symptoms, tumor size and tumor markers were not significant clinical factors to predict SPTs with malignant potential. Surgical procedures mainly included pancreatoduodenectomy, distal pancreatectomy and local resection. Three patients developed local recurrence, and one patient developed hepatic metastasis, all within four years after tumor resection. Five patients with malignant SPTs died due to tumor progression within 25 months after surgery. Surgical resection is the most effective means for curing this rare tumor. Despite metastasis, a good satisfactory effect could be achieved by surgical debulking. At least 4-yearly follow-up is mandatory for all patients undergoing surgical resection.

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Solid Pseudopapillary Tumor of the Pancreas Suggesting Malignant Potential

It has been suggested that the histopathologic features associated with malignant potential in solid pseudopapillary tumors (SPTs) of the pancreas include cellular polymorphism, mitotic activity, peripancreatic tissue invasion, neural invasion, lymphovascular invasion, and lymph node metastasis. This study aimed to delineate the features predicting SPTs with malignant potential, which can be easily assessed in the clinical setting, and long-term results of patients who underwent resection of SPT. A retrospective review and analysis were done of 33 patients who had SPTs that were surgically treated. Eleven patients (33.3%) had SPTs suggesting malignant potential among surgically treated patients with SPTs. The local invasion to the peripancreatic tissue was the most frequent malignant pathological feature (7/11 patients, 63.7%). Using univariate analysis, tumor size greater than 5 cm was a significant clinical factor to predict SPTs with malignant potential (P = 0.022). The prognosis was excellent. No statistically significant survival difference was noted between groups of malignant potential and benign pathology (P = 0.1904). Malignant SPTs are low-grade tumors with good prognosis. Adequate surgical intervention is necessary. Especially, SPTs over 5 cm in diameter need to be treated carefully because of the chance of malignant pathology. Careful long-term follow-up is necessary in those patients in case of possible tumor recurrences.

Significance of Cellular Distribution of Ezrin in Pancreatic Cystic Neoplasms and Ductal Adenocarcinoma

Pancreatic cystic neoplasms and ductal adenocarcinoma manifest diverse clinical features and prognoses, which might be related to cellular distribution of ezrin modulated through various trophic molecules. Laboratory investigation and retrospective analysis. Medical school-affiliated university hospital. Patients with solid pseudopapillary tumor (SPT) (n = 12), mucinous cystic neoplasm (MCN) (n = 18), intraductal papillary mucinous tumor (IPMT) (n = 18), and ductal adenocarcinoma (PA) (n = 73) of the pancreas were studied. Expression of epidermal growth factor (EGF) and its receptor (EGFR) and ezrin were determined using immunohistochemistry. Epidermal growth factor receptor and ezrin expression in sodium butyrate (SB)-treated PA cell line PANC-1 was determined using immunocytochemistry. Messenger RNA expression of ezrin in the PANC-1 cell line treated with SB was determined using reverse transcriptase-polymerase chain reaction. Multivariate analysis of survival of patients with PA was performed. None of 12 SPTs displayed synchronous expression of EGF and EGFR, while all 3 malignant SPTs displayed membranous ezrin expression. One of 18 MCNs displayed synchronous expression of EGF and EGFR, while 4 of 6 borderline malignant and 8 of 8 malignant MCNs displayed membranous ezrin expression. Two of 4 borderline malignant and 11 of 11 malignant IPMTs displayed synchronous expression of EGF and EGFR, and all borderline malignant and malignant IPMTs displayed membranous ezrin expression. Less differentiated PA displayed EGF, EGFR, and membranous ezrin expression more frequently compared with more differentiated PA. Epidermal growth factor receptor expression of PANC-1 cells decreased in an SB dose dependent manner, in which PANC-1 cells became more differentiated and membranous ezrin expression of PANC-1 cells decreased correspondingly. Messenger RNA expression of ezrin in PANC-1 cells also decreased in an SB dose dependent manner. Patients with PA with membranous ezrin expression had a poorer prognosis compared with those without (P = .02). Membranous translocation of ezrin might play a role during malignant transformation of SPT, MCN, IPMT, and PA, which are either dependent on (IPMT and PA) or independent of (SPT and MCN) the EGF-EGFR pathway. Membranous ezrin expression represents a prognostic factor for PA.