Unraveling the enigma:A comprehensive review of solid pseudopapillary tumor of the pancreas

Solid pseudopapillary tumor of the pancreas(SPTP)is a rare neoplasm predom-inantly observed in young females.Pathologically,CTNNB1 mutations,β-catenin nuclear accumulation,and subsequent Wnt-signaling pathway activation are the leading molecular features.Accurate preoperative diagnosis often relies on imaging techniques and endoscopic biopsies.Surgical resection remains the mainstay treatment.Risk models,such as the Fudan Prognostic Index,show promise as predictive tools for assessing the prognosis of SPTP.Establishing three types of metachronous liver metastasis can be beneficial in tailoring individu-alized treatment and follow-up strategies.Despite advancements,challenges persist in understanding its etiology,establishing standardized treatments for unresectable or metastatic diseases,and developing a widely recognized grading system.This comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology,clinical presentation,pathology,molecular characteristics,diagnostic methods,treatment options,and prognostic factors.

Solid pseudopapillary tumor of the pancreas:A systematic review of clinical,surgical and oncological characteristics of 1384 patients underwent pancreatic surgery

Background:Pancreatic solid pseudopapillary tumors(SPTs)are rare clinical entity,with low malignancy and still unclear pathogenesis.They account for less than 2%of exocrine pancreatic neoplasms.This study aimed to perform a systematic review of the main clinical,surgical and oncological characteristics of pancreatic SPTs.Data sources:MEDLINE/PubMed,Web of Science and Scopus databases were systematically searched for the main clinical,surgical and oncological characteristics of pancreatic SPTs up to April 2021,in accordance with the preferred reporting items for systematic reviews and meta-analyses(PRISMA)standards.Primary endpoints were to analyze treatments and oncological outcomes.Results:A total of 823 studies were recorded,86 studies underwent full-text reviews and 28 met inclusion criteria.Overall,1384 patients underwent pancreatic surgery.Mean age was 30 years and 1181 patients(85.3%)were female.The most common clinical presentation was non-specific abdominal pain(52.6%of cases).Mean overall survival was 98.1%.Mean recurrence rate was 2.8%.Mean follow-up was 4.2 years.Conclusions:Pancreatic SPTs are rare,and predominantly affect young women with unclear pathogenesis.Radical resection is the gold standard of treatment achieving good oncological impact and a favorable prognosis in a yearly life-long follow-up.

Solid pseudopapillary tumor of the pancreas:A review of 553 cases in Chinese literature

AIM:To sum up the clinical and pathological characte- ristics of solid pseudopapillary tumor (SPT) and the experience with it.METHODS: A total of 553 SPT patients reported in Chinese literature between January 1996 and January 2009 were retrospectively reviewed and analyzed. RESULTS: 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 ultraso-nography 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 andtail (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%.CONCLUSION: 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.

Prediction of malignancy and adverse outcome of solid pseudopapillary tumor of the pancreas

Since solid pseudopapillary tumor of the pancreas(SP-TP) was officially classified by the World Health Org-anization in 1996,SPTP has recently received special attention in the literature.Studies have shown that SPTP is a heterogeneous tumor,with a small percentage of patients harboring aggressive behaviors.However,cri-teria for malignancy grade in SPTP have not been well established.The prognosis of SPTP is generally good,with cases having a chance for long-term survival even with recurrence and/or metastasis after surgical resection.The current American Joint Committee on Cancer/Union for International Cancer Control tumor,node,metastasis staging system is not specific to SPTP.The lack of a pre-dictive staging classification that accurately describes the heterogeneity of this disease hinders meaningful research into optimal individualized therapy.Here we summarize and discuss the associated factors proposed for appraisal of the malignant potential and adverse outcome of SPTP.

Laparoscopic vs open distal pancreatectomy for solid pseudopapillary tumor of the pancreas

AIM:To compare short-and long-term outcomes of laparoscopic vs open distal pancreatectomy for solid pseudopapillary tumor(SPT)of the pancreas.METHODS:This retrospective study included 28 patients who underwent distal pancreatectomy for SPT of the pancreas between 1998 and 2012.The patients were divided into two groups based on the surgical approach:the laparoscopic surgery group and the open surgery group.The patients’demographic data,operative results,pathological reports,hospital courses,morbidity and mortality,and follow-up data were compared between the two groups.RESULTS:Fifteen patients with SPT of the pancreas underwent laparoscopic distal pancreatectomy(LDP),and 13 underwent open distal pancreatectomy(ODP).Baseline characteristics were similar between the two groups except for a female predominance in the LDP group(100.0%vs 69.2%,P=0.035).Mortality,morbidity(33.3%vs 38.5%,P=1.000),pancreatic fistula rates(26.7%vs 30.8%,P=0.728),and reoperation rates(0.0%vs 7.7%,P=0.464)were similar in the two groups.There were no significant differences in the operating time(171 min vs 178 min,P=0.755)between the two groups.The intraoperative blood loss(149 mL vs 580 mL,P=0.002),transfusion requirement(6.7%vs 46.2%,P=0.029),first flatus time(1.9d vs 3.5 d,P=0.000),diet start time(2.3 d vs 4.9 d,P=0.000),and postoperative hospital stay(8.1 d vs 12.8d,P=0.029)were significantly less in the LDP group than in the ODP group.All patients had negative surgical margins at final pathology.There were no significant differences in number of lymph nodes harvested(4.6 vs6.4,P=0.549)between the two groups.The median follow-up was 33(3-100)mo for the LDP group and 45(17-127)mo for the ODP group.All patients were alive with one recurrence.CONCLUSION:LDP for SPT has short-term benefits compared with ODP.Long-term outcomes of LDP are similar to those of ODP.

Imaging features of solid pseudopapillary tumor of the pancreas on multi-detector row computed tomography

AIM： To retrospectively analyze the imaging features of solid-pseudopapillary tumors （SPTs） of the pancreas on multi-detector row computed tomography （MDCT） and define the imaging findings suggestive of malignant potential. METHODS： A total of 24 consecutive cases with surgically and pathologically confirmed SPTs of the pancreas underwent preoperative abdominal MDCT studies in our hospital. All axial CT images, CT angiographic images, and coronally and sagittally reformed images were obtained. The images were retrospectively reviewed at interactive picture archiving and communication system workstations. RESULTS： Of the 24 cases of SPTs, 11 cases （45.8%） occurred in the pancreatic head and seven （29.1%） in the tail. Eighteen were pathologically diagnosed as benign and six as malignant. MDCT diagnosis of SPTs was well correlated with the surgical and pathological results （Kappa = 0.6, P 〈 0.05）. The size of SPTs ranged from 3 to 15 cm （mean, 5.8 cm）. When the size of the tumor was greater than 6 cm （including 6 cm）, the possibilities of vascular （8 vs 1） and capsular invasion （9 vs 0） increased significantly （P 〈 0.05）.Two pathologically benign cases with vascular invasion and disrupted capsule on MDCT presented with local recurrence and hepatic metastases during follow-up about 1 year after the resection of the primary tumors. CONCLUSION： Vascular and capsular invasion with superimposed spread into the adjacent pancreatic parenchyrna and nearby structures in SPTs of the pancreas can be accurately revealed by MDCT preoperatively. These imaging findings are predictive of the malignant potential associated with the aggressive behavior of the tumor, even in the pathologically benign cases.

Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid pseudopapillary tumor of the pancreas: A case report and literature review

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.

Solid pseudopapillary tumor of the pancreas in male patients: Report of 16 cases

AIM: To investigate the clinical characteristics, surgical strategies and prognosis of solid pseudopapillary tumors (SPTs) of the pancreas in male patients. METHODS: From July 2003 to March 2013, 116 patients were diagnosed with SPT of the pancreas in our institution. Of these patients, 16 were male. The patients were divided into two groups based on gender: female (group 1) and male (group 2). The groups were compared with regard to demographic characteristics, clinical presentations, surgical strategies, complications and follow-up outcomes. RESULTS: Male patients were older than female patients (43.1 +/- 12.3 years vs 33.1 +/- 11.5 years, P = 0.04). Tumor size, location, and symptoms were comparable between the two groups. All patients, with the exception of one, underwent complete surgical resection. The patients were regularly followed up. The mean follow-up period was 58 mo. Two female patients (1.7%) developed tumor recurrence or metastases and required a second resection, and two female patients (1.7%) died during the follow-up period. CONCLUSION: Male patients with SPT of the pancreas are older than female patients. There are no significant differences between male and female patients regarding surgical strategies and prognosis. (C) 2014 Baishideng Publishing Group Inc. All rights reserved.

Pancreatic panniculitis and solid pseudopapillary tumor of the pancreas: A case report

Solid pseudopapillary tumor of the pancreas(SPTP), also known as solid and papillary epithelial neoplasm of the pancreas, is a rare pancreatic exocrine tumor that is difficult to diagnose before surgery. Pancreatic panniculitis is a rare type that occurs in less than 3% of all patients with pancreatic diseases. We here report a 19-year-old woman who presented with persistent left upper quadrant pain without obvious cause for 1 d. The patient also developed subcutaneous nodules involving lower abdomen bilaterally and lower limbs, and subcutaneous nodules were pathologically diagnosed as pancreatic panniculitis. Plain abdominal computed tomography revealed a soft-tissue mass in the body and tail of the pancreas, which was closely associated with the gastric wall. Contrast-enhanced ultrasound showed inhomogeneous echogenicity in the anterior pancreatic body, which had blurred parenchymal demarcation of the body and tail of the pancreas. Contrast-enhanced abdominal computed tomography revealed a mixed density mass with solid and cystic components in the body and tail of the pancreas, and the solid component was markedly enhanced. The lesion was pathologically diagnosed as SPTP after laparoscopic resection. Clinicians should be aware of the clinical manifestation, diagnosis, and treatment of pancreatic panniculitis and SPTP.

A case of solid pseudopapillary tumor of the pancreas

We present ultrasound, computed tomography and magnetic resonance imaging findings in a case with pancreatic solid pseudopapillary tumor and their correlations with histopathology. Ultrasound revealed a hypoechogenic mass, and computed tomography revealed a hypodense mass at the pancreatic head minimally enhanced after intravenous contrast agent administration. Magnetic resonance imaging showed a hypointense mass on unenhanced Tl-weighted images including a hyperintense focus representing the hemorrhage. The lesion was hyperintense on T2- weighted images. On the postcontrast images the lesion showed peripheral thin contrast enhancement in arterial phase and enhanced slightly diffusely in venous and equilibrium phases. The patient underwent elective resection of the mass and pancreatoduodenectomy with jejunostomy tube placement. A final diagnosis of solid pseudopapillary tumor was made histoPathologically. Solid pseudopapillary tumor is a rare pancreatic tumor. It is important to make the diagnosis preoperatively because with an adequate surgical resection the prognosis is good. A multimodalitary approach, especially magnetic resonance imaging can suggest the diagnosis without the need for biopsy.

Combined treatment for solid pseudopapillary tumor of the pancreas with liver metastasis

Solid pseudopapillary tumor (SPT) of the pancreas is a rare pancreatic disease. Generally, it is considered a benign or low-grade malignant tumor. SPT of the pancreas with liver metastasis or invasion to adjacent organs is usually uncommon.

Retroperitoneal congenital epidermoid cyst misdiagnosed as a solid pseudopapillary tumor of the pancreas:A case report

BACKGROUND Retroperitoneal cysts are rare and usually asymptomatic abdominal lesions.Epidermoid cysts are frequent benign cutaneous tumors,but retroperitoneal localization of these cysts does not occur very often.CASE SUMMARY We report a case report of a 25-year-old woman with a giant mass in the abdominal cavity.Because imaging examination indicated that the mass probably originated from the pancreas,the mass was considered a solid pseudopapillary tumor of the pancreas(SPTP).However,surgery revealed a retroperitoneal epidermoid cyst located behind the pancreas neck and the root of the superior mesenteric artery(SMA).We performed complete resection of the tumor.Postoperative pathology showed an epidermoid cyst.The patient fared well after two months of follow-up.CONCLUSION Surgery is the gold standard for the diagnosis and treatment of retroperitoneal epidermoid cysts.Retroperitoneal epidermoid cysts around the pancreas are easily misdiagnosed as cystic SPTPs.Surgeons should pay particular attention to preoperative diagnosis to reduce severe surgical complications and improve the quality of life of patients.

Cryoablation for liver metastasis from solid pseudopapillary tumor of the pancreas: A case report

BACKGROUND Solid pseudopapillary tumor(SPT) of the pancreas is a rare pancreatic tumor and 10% to 15% of cases are associated with metastasis. Cryoablation is a new method that can induce tumor necrosis, and treatment of tumors by cryoablation can cause anti-tumor immune responses.CASE SUMMARY A 16-year-old woman with SPT of the pancreas developed liver metastases 5.3 years after complete resection of the primary pancreatic tumor. She was admitted with chief complaints of abdominal pain in the upper abdomen and a weight loss of approximately 5 kg over 4 mo. Carbohydrate antigen(CA) 125,carcinoembryonic antigen, and CA 199 were normal. An abdominal computed tomography scan found multiple nodules in the right lobe of the liver that measured approximately 13.5 cm × 10.8 cm × 21.4 cm. Immunohistochemical staining results showed that CD10 and CD56 were positive, and the patient was diagnosed with SPT of the pancreas with liver metastasis. The patient underwent percutaneous cryoablation and interventional embolization. During the 5-year follow-up, the patient remained disease-free after cryoablation, with relatively normal immune function.CONCLUSION Herein, we for the first time report the treatment of liver metastasis from SPT of the pancreas using cryoablation plus interventional embolization, which could be a promising alternative therapy for pancreatic SPT liver metastasis.

Solid Pseudopapillary Tumor of the Pancreas:a Case Report

Solid pseudopapillary tumors （SPT） to be a rare low-grade malignancy of the pancreas are considered that mainly appears in young women. It accounts for less than 1% of all pancreatic neoplasms and is pathologically distinctive from other types of pancreatic cancers. Curative resection is the optimal choice for SPT and the 5-year survival rate of SPT is about 95%. Here we report a case of a young girl who presented with this rare pancreatic tumor.

SOLID PSEUDOPAPILLARY TUMOR OF THE PANCREAS:CLINICAL AND COMPUTED TOMOGRAPHIC FINDINGS WITH PATHOLOGIC CORRELATION IN 21 CASES

Objective To correlate the clinical and imaging features of the solid pseudopapillary tumor (SPPT) of the pancreas with the pathologic diagnosis. Methods The computed tomographic findings in 21 patients (18 women and 3 men) with pathologically proven solid pseudopapillary tumor of the pancreas were reviewed retrospectively. Two radiologists reviewed images for location, size and morphology, capsule and margin, density, enhancement pattern, calcification, dilatation of pancreatic duct, lymphadenopathy, vascular invasion, distant metastasis, and recurrence. Results On CT, the lesion presented as solitary, round (28.57%), oval (57.14%), or lobulated (14.28%) mass in pancreatic head (47.62%) with complete capsule (85.71%). Lesions smaller than 4cm in maximum diameter (47.62%) presented as predominantly solid mass whereas lesions greater than 4cm in diameter (52.28%) presented as heterogenous mass. On contrast administration, the viable solid portion of the tumor showed mild peripheral enhancement in arterial phase [enhancement degree<20Hounsfield unit (HU)] with progressive fill in during the portal and hepatic parenchyma phase (enhancement degree 20-40HU). Eighteen lesions (85.7%) had complete capsule. Two lesions (9.5%) had areas of high density (>60HU) on plain CT which due to hemorrhage. Dilatation of the pancreatic duct was rare (25%), and distant metastasis, regional lymphadenopathy, and tumor recurrence were absent. Nine patients (42.85%) had an abdominal CT follow-up for 24.55months (range, 2-60months) post-surgery without recurrence or distant metastasis. Conclusion Solid pseudopapillary tumor of the pancreas usually occurs in young females in the pancreatic head or tail. On CT, they manifest as moderate vascular mixed tumor, progressive enhancement of the viable solid portion, distinct tumor margin with capsule formation, and absence of regional lymphadenopathy.

Comparison between solid pseudopapillary neoplasms of the pancreas and pancreatic ductal adenocarcinoma with cystic changes using computed tomography

BACKGROUND Solid pseudopapillary neoplasms of the pancreas(SPN)share similar imaging findings with pancreatic ductal adenocarcinoma with cystic changes(PDAC with cystic changes),which may result in unnecessary surgery.AIM To investigate the value of computed tomography(CT)in differentiation of SPN from PDAC with cystic changes.METHODS This study retrospectively analyzed the clinical and imaging findings of 32 patients diagnosed with SPN and 14 patients diagnosed with PDAC exhibiting cystic changes,confirmed through pathological diagnosis.Quantitative and qualitative analysis was performed,including assessment of age,sex,tumor size,shape,margin,density,enhancement pattern,CT values of tumors,CT contrast enhancement ratios,“floating cloud sign,”calcification,main pancreatic duct dilatation,pancreatic atrophy,and peripancreatic invasion or distal metastasis.Multivariate logistic regression analysis was used to identify relevant features to differentiate between SPN and PDAC with cystic changes,and receiver operating characteristic curves were obtained to evaluate the diagnostic performance of each variable and their combination.RESULTS When compared to PDAC with cystic changes,SPN had a lower age(32 years vs 64 years,P<0.05)and a slightly larger size(5.41 cm vs 3.90 cm,P<0.05).SPN had a higher frequency of“floating cloud sign”and peripancreatic invasion or distal metastasis than PDAC with cystic changes(both P<0.05).No significant difference was found with respect to sex,tumor location,shape,margin,density,main pancreatic duct dilatation,calcification,pancreatic atrophy,enhancement pattern,CT values of tumors,or CT contrast enhancement ratios between the two groups(all P>0.05).The area under the receiver operating characteristic curve of the combination was 0.833(95%confidence interval:0.708-0.957)with 78.6%sensitivity,81.3%specificity,and 80.4%accuracy in differentiation of SPN from PDAC with cystic changes.CONCLUSION A larger tumor size,“floating cloud sign,”and peripancreatic invasion or distal metastasis are useful CT imaging features that are more common in SPN and may help discriminate SPN from PDAC with cystic changes.

Role of endoscopic ultrasound in the characterization of solid pseudopapillary neoplasm of the pancreas

BACKGROUND Solid pseudopapillary neoplasm(SPN)is an uncommon pathology of the pancreas with unpredictable malignant potential.Endoscopic ultrasound(EUS)assessment plays a vital role in lesion characterization and confirmation of the tissue diagnosis.However,there is a paucity of data regarding the imaging assessment of these lesions.AIM To determine the characteristic EUS features of SPN and define its role in preoperative assessment.METHODS This was an international,multicenter,retrospective,observational study of prospective cohorts from 7 large hepatopancreaticobiliary centers.All cases with postoperative histology of SPN were included in the study.Data collected included clinical,biochemical,histological and EUS characteristics.RESULTS One hundred and six patients with the diagnosis of SPN were included.The mean age was 26 years(range 9 to 70 years),with female predominance(89.6%).The most frequent clinical presentation was abdominal pain(80/106;75.5%).The mean diameter of the lesion was 53.7 mm(range 15 to 130 mm),with the slight predominant location in the head of the pancreas(44/106;41.5%).The majority of lesions presented with solid imaging features(59/106;55.7%)although 33.0%(35/106)had mixed solid/cystic characteristics and 11.3%(12/106)had cystic morphology.Calcification was observed in only 4(3.8%)cases.Main pancreatic duct dilation was uncommon,evident in only 2 cases(1.9%),whilst common bile duct dilation was observed in 5(11.3%)cases.One patient demonstrated a double duct sign at presentation.Elastography and Doppler evaluation demonstrated inconsistent appearances with no emergence of a predictable pattern.EUS guided biopsy was performed using three different types of needles:Fine needle aspiration(67/106;63.2%),fine needle biopsy(37/106;34.9%),and Sonar Trucut(2/106;1.9%).The diagnosis was conclusive in 103(97.2%)cases.Ninety-seven patients were treated surgically(91.5%)and the post-surgical SPN diagnosis was confirmed in all cases.During the 2-year follow-up period,no recurrence was observed.CONCLUSION SPN presented primarily as a solid lesion on endosonographic assessment.The lesion tended to be located in the head or body of the pancreas.There was no consistent characteristic pattern apparent on either elastography or Doppler assessment.Similarly SPN did not frequently cause stricture of the pancreatic duct or common bile duct.Importantly,we confirmed that EUS-guided biopsy was an efficient and safe diagnostic tool.The needle type used does not appear to have a significant impact on the diagnostic yield.Overall SPN remains a challenging diagnosis based on EUS imaging with no pathognomonic features.EUS guided biopsy remains the gold standard in establishing the diagnosis.

Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas

BACKGROUND: Solid-pseudopapillary tumor (SPT) of the pancreas is a rare exocrine pancreatic tumor. Despite the increasing recognition of the tumor in recent years, its pathogenesis and apparent therapeutic algorithm remain unclear. This study was designed to define the clinical, imaging, and pathologic features and to improve the diagnosis and treatment of this rare disease. METHOD: The clinical, imaging, and pathologic findings of 9 SPT patients managed in our hospital between 2001 and 2005 were retrospectively analyzed, and related literatures were reviewed. RESULTS: In the 9 patients aged from 14 to 68 years, 8 were female and 1 male. The mean age of these patients at diagnosis was 30 years. Initially, 8 patients complained of vague abdominal pain and one patient had pancreatic mass detected incidentally by abdominal CT. The levels of blood and urine amylase and tumor markers were all within the normal range. B-US, CT and MRI demonstrated that tumors were well encapsulated and contained some degree of internal hemorrhage or cystic degeneration. The mean transverse diameter of these tumors was 5.4 cm (range, 2-10.5 cm). The tumors were located at the head (2 patients), body (2), body and tail junction (4), and tail (1) of the pancreas. Surgical procedures included pancreaticoduodenectomy, distal pancreatectomy, distal pancreatectomy with splenectomy, and enucleation. Histological examination showed solidified cystic areas and papillary protrusions. Two malignant tumors demonstrated retroperitoneal metastases and vascular invasion. Follow-up for 2.5 years on average showed that one patient died of tumor recurrence at 10 months and the rest were alive. CONCLUSIONS: SPT exhibits unique clinical and pathologic features and is readily diagnosed by its characteristic imaging and histological appearance. Surgical resection of the primary tumor and metastases is the treatment of choice.

Clinical analysis of solid-pseudopapillary tumor of the pancreas:report of 15 cases

BACKGROUND: Solid-pseudopapillary tumor of the pancreas (SPTP) is an uncommon and enigmatic pancreatic neoplasm that occurs mainly in young women. Although more and more cases have been reported in recent years, misdiagnosis and incorrect treatment still frequently take place. This study was designed to stimulate consideration of this tumor. METHODS: We retrospectively reviewed the experience of diagnosis and treatment of 15 patients with SPTP and compared them with 516 patients with pancreatic cancer from January 1997 to March 2007. RESULTS: Most of the SPTP cases were asymptomatic except for one palpable mass. Almost all SPTPs demonstrated a solid structure with hypo- or isoattenuation, cystic structure with hypo-attenuation on precontrast CT scan, and enhancement of solid portions on post-contrast CT scan. By contrast, most cases of pancreatic carcinoma had multiple symptoms and abnormal blood results. The tumors showed hypo-attenuation on both precontrast and post-contrast CT scan, and only a few showed iso-attenuation on post-contrast CT scan. All cases of SPTP in our group were cured by surgical resection, while only 16.86% of patients with pancreatic carcinoma could undergo a radical resection. CONCLUSIONS: Clinical features and CT scans were helpful to differentiate SPTP from pancreatic carcinoma. Radical surgical resection was the most effective and safe method for the treatment of SPTP.

Pathological differential diagnosis of solid-pseudopapillary neoplasm and endocrine tumors of the pancreas

AIM:To investigate differential points of solid-pseudo-papillary neoplasm (SPN) of the pancreas and pancre-atic endocrine tumor (PET).METHODS:Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features,immunostaining reactions and β-catenin gene mutations were analyzed.RESULTS: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 pseudo-papillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of β-catenin and loss of E-cadherin in all the cases,was only seen in SPN. Molecular studies discov-ered that 9/10 (90%) cases harbored a point mutation of exon 3 in β-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 hypoglyce-mia,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 β-catenin without loss of E-cadherin,as well as no mutation in β-catenin gene in all the cases. CONCLUSION:Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary,immunos-taining of β-catenin and E-cadherin is quite helpful to make the differential diagnosis.