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.

Prospects and applications of enucleation in solid pseudopapillary neoplasms of the pancreas

Solid pseudopapillary neoplasms(SPNs)of the pancreas are rare,low-grade,malignant neoplasms that are mostly seen in young women in the second and third decades of life and are quite uncommon in children.Standard resection for benign and borderline neoplasms of the pancreas is associated with a substantial risk of postoperative morbidity and long-term functional impairment,whereas enucleation leads to less morbidity and preserves healthy parenchyma as well as exocrine and endocrine function.Enucleation of SPNs has been increasingly reported to be feasible and safe for preserving the normal physiological function of the pancreas,especially in teenagers and children.This review summarizes findings published in recent years on the enucleation of SPNs as well as potential future developments and directions.Enucleation has undoubtedly come to stay as an alternative surgical procedure for SPNs.However,many questions remain unresolved,and future directions toward the best surgical indication,the prevention and intervention of complications,especially pancreatic fistula,intraoperative resection margin safety assessment,and long-term oncology prognosis remain to be evaluated and should be explored in future clinical trials.

Impact of parenchyma-preserving surgical methods on treating patients with solid pseudopapillary neoplasms:A retrospective study with a large sample size

BACKGROUND Solid pseudopapillary neoplasm of the pancreas(SPN)is a rare neoplasm that mainly affects young women.AIM To evaluate the impact of parenchyma-preserving surgical methods(PPMs,including enucleation and central pancreatectomy)in the treatment of SPN patients.METHODS From 2013 to 2019,patients who underwent pancreatectomy for SPNs were retrospectively reviewed.The baseline characteristics,intraoperative index,pathological outcomes,short-term complications and long-term follow-up data were compared between the PPM group and the conventional method(CM)group.RESULTS In total,166 patients were included in this study.Of them,33 patients(19.9%)underwent PPM.Most of the tumors(104/166,62.7%)were found accidentally.Comparing the parameters between groups,the hospital stay d(12.35 vs 13.5 d,P=0.49),total expense(44213 vs 54084 yuan,P=0.21),operation duration(135 vs 120 min,P=0.71),and intraoperative bleeding volume(200 vs 100 mL,P=0.49)did not differ between groups.Regarding pathological outcomes,tumor size(45 vs 32 mm,P=0.07),Ki67 index(P=0.53),peripheral tissue invasion(11.3%vs 9.1%,P=0.43)and positive margin status(7.5%vs 6%,P=0.28)also did not differ between groups.Moreover,PPM did not increase the risk of severe postoperative pancreatic fistula(3.8%vs 3.0%,P=0.85)or tumor recurrence(3.0%vs 6.0%,P=0.39).However,the number of patients who had exocrine insufficiency during follow-up was significantly lower in the PPM group(21.8%vs 3%,P=0.024).CM was identified as an independent risk factor for pancreatic exocrine insufficiency(odds ratio=8.195,95%confident interval:1.067-62.93).CONCLUSION PPM for SPN appears to be feasible and safe for preserving the exocrine function of the pancreas.

Treatment of aggressive pancreatic solid pseudo-papillary neoplasms with apatinib plus S-1 chemotherapy:A case report and literature review

Solid pseudopapillary neoplasm(SPN) is a rare indolent pancreatic neoplasm that occurs mostly in females. Although the malignancy potential is quite limited for SPN, these tumors can sometimes be aggressive and lead to inferior prognosis for male patients. In this case report, we present a special case of a male patient with SPN who experienced an aggressive tumor expansion after two surgical resections. For further treatment, we decided to administer chemotherapy with apatinib and S-1, and subsequent CT/MRI tumor monitoring indicated satisfactory control of tumor expansion. The effectiveness of apatinib plus the S-1 regimen should be tested for more patients with SPN in the future.

Unraveling the landscape of pediatric pancreatic tumors:Insights from Japan

Pediatric pancreatic tumors,though rare,pose significant diagnostic and manage-ment challenges.The recent,22-year nationwide survey on pediatric pancreatic tumors in Japan by Makita et al offers valuable insights into this uncommon enti-ty,revealing striking geographical variations and questioning current treatment paradigms.This editorial commentary analyzes the study's key findings,inclu-ding the predominance of solid pseudopapillary neoplasms and their younger age of onset,which contrast sharply with Western data.It explores the implications for clinical practice and research,emphasizing the need for population-specific approaches to diagnosis and treatment.The revealed limited institutional expe-rience and surgical management patterns prompt a reevaluation of optimal care delivery for these complex cases,suggesting benefits of centralizing healthcare services.Furthermore,the commentary advocates for international collaborative studies to elucidate the genetic,environmental,and lifestyle factors influencing the development and progression of pediatric pancreatic tumors across diverse populations.It also outlines future directions,calling for advancements in precision medicine and innovative care delivery models to improve global patient outcomes.Unraveling Makita et al's findings within the broader landscape of pediatric oncology can stimulate further research and clinical advancements in managing pancreatic and other rare tumors in children.

Extrapancreatic solid pseudopapillary neoplasm followed by multiple metastases:Case report

Solid pseudopapillary neoplasm(SPN), also known as Gruber-Frantz tumor, is a rare form of neoplasm that almost exclusively occurs in the pancreas and in young females. While the potential of malignancy is low for SPN, these tumors can mimic other diseases and require a meticulous investigation and a standard treatment by total surgical resection. We present an unusual case of SPN arising in the mesentery of a 40-year-old man with subsequent multiple metastases. Histopathological examination showed similar properties of the mesenteric neoplasm to those of SPN in pancreas. Although the mass was surgically removed, the patient died of recurrent disease 4 years after the initial presentation. We speculate that SPN originates from pancreatic progenitor cells. Further histopathological analyses are required for the prediction of SPN recurrence after resection.

Solid pseudopapillary neoplasm of the pancreas

Solid pseudopapillary neoplasms are rare.This article reviews the clinical and pathologic features of solid pseudopapillary neoplasm of the pancreas,including the epidemiology,cytology,molecular pathology,differential diagnosis,treatment,and prognosis.Solid pseudopapillary neoplasms are low-grade malignant tumours of the pancreas characterized by poorly cohesive epithelial cells with solid and pseudopapillary patterns.Solid pseudopapillary neoplasms occur predominantly in young women.Although solid pseudopapillary neoplasms can occur throughout the pancreas,they arise slightly more frequently in the tail of the pancreas.The aetiology is unknown.Extremely rare cases have been reported in the setting of familial adenomatous polyposis.There are no symptoms unique to solid pseudopapillary neoplasms,however,the most common symptom is abdominal pain or discomfort.The features of solid pseudopapillary neoplasms on computed tomography imaging are indicative of the pathologic changes within the tumour.Typically,well-demarcated masses with variably solid and cystic appearances.Microscopically,these tumours are composed of epithelial cells forming solid and pseudopapillary structures,frequently undergoing haemorrhagic cystic degeneration.Typically,these tumours express nuclear and/or cytoplasmicβ-catenin.Almost all solid pseudopapillary neoplasms harbour mutations in exon 3 of CTNNB1,the gene encodingβ-catenin.The overall prognosis is excellent,and most patients are cured by complete surgical resection.

A new combined criterion to better predict malignant lesions in patients with pancreatic cystic neoplasms

Objective:Cystic lesions of the pancreas have been increasingly recognized.Some lesions exhibit benign behavior,while others have unequivocal malignant potential.Thus,accurate identification of malignancy in patients diagnosed with pancreatic cystic neoplasms(PCNs)remains a major challenge.The aim of this study was to define a combined criterion to better predict malignant lesions in patients with PCNs.Methods:We retrospectively analyzed 165 patients who underwent resection of PCNs from October 2011 to May 2017.The relationship among malignancy and serum carbohydrate antigen 19-9(CA19-9),preoperative neutrophil-to-lymphocyte ratio(NLR),and the presence of enhanced solid component on imaging was analyzed.Results:NLR before surgery in patients with malignant PCNs(2.81±2.14)was significantly higher than that in patients diagnosed with pancreatic neuroendocrine tumor(1.90±0.69,P=0.013)or healthy volunteers(1.40±0.48;P<0.001).Serum CA19-9≥39U/m L,NLR>1.976 and presence of enhanced solid component were independent predictors of PCN malignancy.A combined criterion meeting any two or more of the three elements including CA19-9≥39 U/m L,NLR>1.976,and presence of enhanced solid component on computed tomography imaging is an indicator with a high positive predictive value of 80.5%and a high negative predictive value of 87.9%,and thus,represents a highly accurate test(86.1%).Conclusions:The new combined criterion is an effective predictor of tumor malignancy in patients with PCNs.

SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma mimicking solid pseudopapillary neoplasm: A case report and review of the literature

BACKGROUND SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a very aggressive tumor that is rarely reported in the literature.The tumor has a predominant rhabdoid cell component and different patterns of growth have been reported.CASE SUMMARY A 59-year-old woman presented with diffuse abdominal pain,increasing in severity and accompanied by weight loss,nausea,and vomiting.Imaging showed a pancreatic head mass.Fine needle aspiration demonstrated atypical epithelioid cells with a pseudopapillary growth pattern suggestive of solid pseudopapillary neoplasm.The excised neoplasm showed monotonous epithelioid and focally spindle cells with pseudopapillary structures,rhabdoid features,and loss of SMARCB1 protein expression with wild-type KRAS,consistent with a SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma.The patient’s condition deteriorated rapidly following surgery and she expired 3 mo post operation.CONCLUSION In this article,we report the first case of SMARCB1/INI1-deficient undifferentiated pancreatic rhabdoid carcinoma mimicking solid pseudopapillary neoplasm.

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.

Solid pseudopapillary neoplasm-diagnostic approach and postsurgical follow up:Three case reports and review of literature

BACKGROUND Solid pseudopapillary neoplasm(SPN)is a rare tumor that was first described by Frantz in 1959.Although this tumor is benign,some may have malignant potential that can be predicted based on demographics,imaging characteristics,and pathologic evaluation.This case series presents 3 SPN cases with discussion on gender differences,preoperative predictors of malignancy,and a suggested algorithm for diagnostic approach as well as post-surgical follow up.CASE SUMMARY Three adult patients in a tertiary hospital found to have SPN,one elderly male and two young females.Each of the cases presented with abdominal pain and were discovered incidentally.Two cases underwent endoscopic ultrasound with immunohistochemical staining(which were consistent with SPN before undergoing surgery),and one case underwent surgery directly after imaging.The average tumor size was 5 cm.Diagnosis was confirmed by histology.Two patients had post-surgical complications requiring intervention.CONCLUSION Demographic and imaging characteristics can be sufficient to establish diagnosis for SPN,while malignant cases require pre-operative evaluation with endoscopic ultrasound fine needle aspiration/fine needle biopsy.

Solid pseudopapillary neoplasm of the pancreas in a young male with main pancreatic duct dilatation: A case report

BACKGROUND Solid pseudopapillary neoplasms(SPNs)are rare tumors of the pancreas.Typically,they occur in young females,often have characteristic imaging features,such as cystic components and calcification,and have few effects on the pancreatic duct.CASE SUMMARY A 31-year-old man was admitted to our hospital with the chief complaint of epigastric pain.There was only mild tenderness in his upper abdomen,and blood tests showed only a slight increase in alkaline phosphatase.Contrast-enhanced computed tomography showed a 40-mm-diameter,hypovascular mass in the head of the pancreas,and the main pancreatic duct upstream of the mass was severely dilated.Magnetic resonance imaging showed low intensity on T1-weighted images,with high intensity on T2-weighted image in some parts.Pancreatic ductal adenocarcinoma was the primary differential diagnosis.Portal vein infiltration could not be ruled out,so this case was a candidate for neoadjuvant chemotherapy.Subsequently,endoscopic ultrasound-guided fine needle aspiration was performed,and pathological evaluation and immunostaining suggested a diagnosis of SPN.Thus,pancreatoduodenectomy was performed.One year after the operation,the patient is alive with no recurrence.CONCLUSION Main pancreatic duct dilatation is usually a finding of suspected pancreatic cancer.However,pancreatic duct dilatation can occur in SPN depending on the location and growth speed.Therefore,SPN should be considered in the differential diagnosis of tumors with pancreatic duct dilatation,and pathological evaluation by endoscopic ultrasound-guided fine needle aspiration should be actively performed.

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.

Peripancreatic paraganglioma:Lesson from a round table

We described the case of a peripancreatic paraganglioma(PGL)misdiagnosed as pancreatic lesion.Surgical exploration revealed an unremarkable pancreas and a large well-defined cystic mass originating at the mesocolon root.Radical enucleation of the mass was performed,preserving the pancreatic tail.Histologically,a diagnosis of PGL was rendered.Interestingly,two previously unreported mutations,one affecting the KDR gene in exon 7 and another on the JAK3 gene in exon 4 were detected.Both mutations are known to be pathogenetic.Imaging and cytologic findings were blindly reviewed by an expert panel of clinicians,radiologists,and pathologists to identify possible causes of the misdiagnosis.The major issue was lack of evidence of a cleavage plane from the pancreas at imaging,which prompted radiologists to establish an intraparenchymal origin.The blinded revision shifted the diagnosis towards an extrapancreatic lesion,as the pancreatic parenchyma showed no structural alterations and no dislocation of the Wirsung duct.Ex post,the identified biases were the emergency setting of the radiologic examination and the very thin mesocolon sheet,which hindered clear definition of the lesion borders.Original endoscopic ultrasonography diagnosis was confirmed,emphasizing the intrinsic limit of this technique in detecting large masses.Finally,pathologic review favored a diagnosis of PGL due to the morphological features and immonohistochemical profile.Eighteen months after tumor excision,the patient is asymptomatic with no disease relapse evident by either radiology or laboratory tests.Our report strongly highlights the difficulties in rendering an accurate preoperative diagnosis of PGL.

Organ-preserving surgery and classic surgery for pancreatic solid pseudopapillary neoplasms:a multicenter analysis from Central and Western China

Background:Pancreatic solid pseudopapillary neoplasms(SPNs)are low-grade malignant tumors of the pancreas.Organ-pre-serving surgery is being increasingly performed for these tumors,although there is little evidence to support its use.This retro-spective multicenter study aimed to determine the clinicopathologic characteristics of pancreatic SPNs in Central and Western China and to determine the efficacy of organ-preserving surgery.Methods:The clinicopathologic,treatment and follow-up data of 227 pancreatic SPN patients treated between July 2003 and December 2016 at 5 tertiary care centers were retrospectively reviewed.Results:Among the 227 patients(38 males,189 females;mean age 33.30±12.70 years),only 72(31.7%)had symptoms.The mean tumor size was 55.08±29.56 mm.Adjacent organ/lymphovascular invasion was present in 16(7.1%)patients and distant metastasis in 3(1.3%)patients;no patient had spread to distant lymph nodes.Surgery included organ-preserving surgery in 108(47.6%)patients,classic surgery in 115(50.7%)patients,and palliative surgery in 4(1.8%)patients.Tumor recurrence was seen in 8(3.6%)patients.Cox regression analysis showed positive surgical margin(P<.01)and metastasis(P=.03)to be independent predictors of recurrence.Tumor characteristics were comparable between patients receiving organ-preserving surgery and classic surgery.The risk of recurrence was also similar in both groups(P=.72).Conclusions:Organ-preserving surgery appears to be an effective procedure for the treatment of pancreatic SPNs.Resection surgery should aim for negative surgical margins.