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.

Imaging, pathology, and diagnosis of solitary fibrous tumor of the pancreas: A case report and review of literature

BACKGROUND A solitary fibrous tumor(SFT)is often located in the pleura,while SFT of the pancreas is extremely rare.Here,we report a case of SFT of the pancreas and discuss imaging,histopathology,and immunohistochemistry for accurate diagnosis and treatment.CASE SUMMARY A 54-year-old man presented to our hospital with pancreatic occupancy for over a month.There were no previous complaints of discomfort.His blood pressure was normal.Blood glucose,tumor markers,and enhanced computed tomography(CT)suggested a malignant tumor.Because the CT appearance of pancreatic cancer varies,we could not confirm the diagnosis;therefore,we performed endoscopic ultrasound-guided fine-needle biopsy(EUS-FNB).Pathology and immunohistochemistry were consistent with SFT of the pancreas.The posto-perative pathology and immunohistochemistry were consistent with the puncture results.The patient presented for a follow-up examination one month after discharge with no adverse effects.CONCLUSION Other diseases must be excluded in patients with a pancreatic mass that cannot be diagnosed.CT and pathological histology have diagnostic value for pancreatic tumors.Endoscopic puncture biopsy under ultrasound can help diagnose pancreatic masses that cannot be diagnosed preoperatively.Surgery is an effective treatment for SFT of the pancreas;however,long-term follow-up is strongly recommended because of the possibility of malignant transformation of the tumor.

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.

Sclerosing epithelioid fibrosarcoma of the pancreas:A case report

BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including its clinical manifestations,preoperative imaging features,gross specimen and pathological findings.CASE SUMMARY A 33-year-old male patient was admitted to Peking Union Medical College Hospital in December 2023 due to a one-year history of intermittent upper abdominal pain and the discovery of a pancreatic mass.The patient underwent an enhanced computed tomography scan of the abdomen,which revealed a welldefined,round mass with clear borders and calcifications in the pancreatic head.The mass exhibited progressive,uneven mild enhancement,measuring approximately 6.6 cm×6.3 cm.The patient underwent laparoscopic pylorus-preserving pancreaticoduodenectomy.Postoperative pathological examination revealed that the lesion was consistent with a SEF.At the 3-month postoperative follow-up,the patient did not report any short-term complications,and there were no signs of tumor recurrence.CONCLUSION SEFs are rare malignant fibrous soft tissue tumors.SEFs rarely develop in the pancreas,and its preoperative diagnosis depends on imaging findings,with confirmation depending on pathological examination and immunohistochemistry.Currently,only four cases of pancreatic SEF have been reported in studies written in English.This case is the first reported case of a pancreatic SEF by a clinical physician.

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.

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.

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.Themean 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.

Non-functional neuroendocrine tumors of the pancreas: Advances in diagnosis and management

Nonfunctional neuroendocrine tumors of the pancreas(NF-PNETs) are a heterogeneous group of neoplasms. Although rare, the incidence of NF-PNETs is increasing significantly. The classification of PNETs has evolved over the past decades and is now based on a proliferation grading system. While most NF-PNETs are slow growing, tumors with more aggressive biology may becomeincurable once they progress to unresectable metastatic disease. Tumors of higher grade can be suspected preoperatively based on the presence of calcifications, hypoenhancement on arterial phase computed tomography, positron emission technology avidity and lack of octreotide scan uptake. Surgery is the only curative treatment and is recommended for most patients for whom complete resection is possible. Liver-directed therapies(thermal ablation, transarterial embolization) can be useful in controlling unresectable hepatic metastatic disease. In the presence of unresectable progressive disease, somatostatin analogues, everolimus and sunitinib can prolong progression-free survival. This article provides a comprehensive review of NF-PNETs with special emphasis on recent advances in diagnosis and management.

Multidisciplinary management of nonfunctional neuroendocrine tumor of the pancreas

Pancreatic neuroendocrine tumors(PNETs) are a rare and diverse group of tumors; nonfunctional(NF) PNETs account for the majority of cases. Most patients with NF-PNETs have metastatic disease at the time of presentation. A variety of treatment modalities exist, including medical, liver directed, and surgical treatments. Aggressive surgical management is associated with prolonged survival, however available data are limited by selection bias and the frequent combination of PNETs with carcinoid tumors. Although few patients with metastatic disease will be cured, application of currently available therapies in a multidisciplinary setting can lead to excellent outcomes with prolonged patient survival.

Osteoclastic and pleomorphic giant cell tumors of the pancreas:A review of clinical,endoscopic,and pathologic features

Giant cell tumors of the pancreas come in three varieties-osteoclastic,pleomorphic,and mixed histology.These tumors have distinctive endoscopic,clinical,and cytological features.Giant cell tumors have a controversial histogenesis,with some authors favoring an epithelial origin and others favoring a mesenchymal origin.The true origin of these lesions remains unclear at this time.These are also very rare tumors but proper identification and differentiation from more common pancreatic adenocarcinoma is important.The risk factors of these tumors and the prognosis may be different from those associated with standard pancreatic adenocarcinoma.Recognition of these differences can significantly affect patient care.These lesions have a unique appearance when imaged with endoscopic ultrasound(EUS),and these lesions can be diagnosed via EUS guided Fine Needle Aspiration(FNA).This manuscript will review the endoscopic,clinical,and pathologic features of these tumors.

Granular cell tumor of the pancreas:A case report and review of literature

Granular cell tumors,also called Abrikossof's tumors,were originally described by Abrikossof A in 1926.The f irst case of a pancreatic granular cell tumor was described in 1975 and only 6 cases have been reported.We describe a case of granular cell tumor in the pancreas showing pancreatic duct obstruction.Because imaging studies showed f indings compatible with those of pancreatic carcinoma,the patient underwent distal pancreatectomy.Histological examination showed that the tumor consisted of a nested growth of large tumor cells with ample granular cytoplasm and small round nuclei.The tumor cells expressed S-100 protein and were stained with neuron-specific enolase and periodic acid-Schiff,but were negative for desmin,vimentin,and cytokeratin.The resected tumor was diagnosed as a granular cell tumor.To our knowledge,this is the seventh case of Granular cell tumor of the pancreas to be reported.

Solitary fibrous tumor of the pancreas:Case report and review of the literature

Solitary fibrous tumor(SFT) is a mesenchymal tumor typically located in the pleura,but can also be found as an asymptomatic mass in other areas,including the liver,peritoneum,kidney and salivary glands.However,SFT rarely locates in the pancreas.We present such a case of pancreatic SFT,along with a review of all reported cases.A 55-year-old man was treated surgically for an asymptomatic pancreatic mass after a rigorous preoperative control.Histologic examination of the resected specimen showed characteristics of an SFT.As only 15 cases of pancreatic SFT have been reported so far,an attempt to compare the cases was considered intriguing.We found that patients with pancreatic SFT were mainly women(81.25%),with a median age of 54 years at the time of diagnosis and a median tumor size of 5.83 cm.Pancreatic SFTs were revealed incidentally in 50% of cases,and all of them showed an enhancement through arterial computed tomography.All tumors were positive for CD34,ten were positive for Bcl-2,and twelve were negative for S100.The diagnosis of this pancreatic tumor is established by a combination of clinical suspicion,imaging procedures and histological findings,and is confirmed by immunohistochemical staining.Although the behavior of SFTs is rather benign,close clinical follow-up is recommended due to a potentially malignant nature.

Role of frozen section assessment for intraductal papillary and mucinous tumor of the pancreas

Intraductal papillary mucinous neoplasms(IPMN) of the pancreas include a spectrum of dysplasia ranging from minimal mucinous hyperplasia to invasive carcinoma and are extensive tumors that often spread along the ductal tree.Several studies have demonstrated that preoperative imaging is not accurate enough to adapt the extent of pancreatectomy and have suggested routinely using frozen sectioning(FS) to evaluate the completeness of resection and also to check if ductal dilatation is active or passive,in order to avoid an excessive pancreatic resection.Separate main duct and branch duct analysis is needed due to the difference in the natural history of the disease.FS accuracy averages 95%.Eroded epithelium on the main duct,severe ductal inflammation mimicking dysplasia and reactive epithelial changes secondary to obstruction can lead to inappropriate FS results.FS results change the planned extent of resection in up to 30% of cases.The optimal cut-off leading to extend pancreatectomy is not consensual and our standard option is to extend pancreatec-tomy if FS reveals:(1) at least IPMN adenoma on the main duct;or(2) at least borderline IPMN on branch ducts;or(3) invasive carcinoma.However,the decision to extend resection must be taken after a multidisciplinary discussion since it does not exclusively depend on the FS result but also on age,general condition and expected prognosis after resection.The main limitation of using FS is the existence of discontinuous("skip") lesions which account for approximately 10% of IPMN in surgical series and can lead to reoperation in up to 8% of cases.

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.

Long-term survival of a patient after resection of a gastrointestinal stromal tumor arising from the pancreas

BACKGROUND:Gastrointestinal stromal tumors (GISTs) may arise in any part of the gastrointestinal tract;extragastrointestinal locations are extremely rare.Only a few cases of extragastrointestinal stromal tumor arising from the pancreas were reported.None of the reports described a long-term follow-up of the patients.METHOD:This report describes an interesting and unusual case of GIST arising from the pancreas.RESULTS:A 74-year-old female presented with a palpable abdominal mass.CT scan showed a large mass 11×8×4 cm originating from the tail of the pancreas.Percutaneous biopsy revealed a GIST predominantly with spindle cells,but some parts also contained epitheloid cells.The patient was treated by distal pancreatic resection with splenectomy.Immunohistochemistry of the tumor showed a staining pattern characteristic of GIST.The patient has achieved a long-term survival of five years and six months without any sign of recurrence of the disease.CONCLUSION:This is the first reported case of an extragastrointestinal stromal tumor arising from the pancreas treated surgically,with a long-term survival.

Solid-pseudopapillary tumor of the pancreas:CT and MRI features of 3 cases

BACKGROUND: Solid-pseudopapillary tumor of the pancreas is a rather rare but low-grade malignant tumor with good prognosis after surgical excision. METHODS: In 3 patients with solid-pseudopapillary tumor of the pancreas which were proved pathologically, the imaging features ( CT in all and MRI in one patient) of the tumor were analyzed. RESULTS: In the 3 female patients under 35-year-old who presented no jaundice, CT revealed that huge solid-cystic masses of the pancreatic head with calcification in one and slight dilation of the pancreatic duct in another, but without dilation of the bile duct system in all. MRI demonstrated mixed signal intensity on T1WI and T2WI of the mass in one patient. CT and MRI revealed obvious enhancement of the cystic wall and solid part of the masses. CONCLUSIONS: CT and MRI findings of solid-pseudopapillary tumor of the pancreas are characteristic, and the diagnosis can be made preoperatively with the combination of clinical features.

Solitary concomitant endocrine tumor and ductal adenocarcinoma of pancreas

Pancreatic tumors with combined exocrine and endocrine features are rare.Most reported cases are classified as mixed exocrine and endocrine carcinoma of the pancreas.We report the first case of solitary concomitant endocrine tumor and ductal adenocarcinoma of the pancreas.A 58-year-old patient was admitted for uncontrolled diabetes mellitus and body weight loss.The tumor was fortuitously discovered in the pancreatic tail after a tumor survey panel.Grossly,the solitary tumor had a central fibrous band that clearly divided it into two parts.On microscopic examination,the tumor contained both endocrine and exocrine components distinctly separated by the central fibrous band.The exocrine part showed a poorly-differentiated adenocarcinoma.The endocrine part was strongly immunoreactive to chromogranin,synaptophysin and glucagon.We reviewed the literature on pancreatic tumors with combined exocrine and endocrine features.A simple classification for this group of neoplasms is suggested,including five types:amphicrine,mixed,collision,solitary concomitant and multiple concomitant.

Malignant solitary fibrous tumor of the pancreas with systemic metastasis: A case report and review of the literature

BACKGROUND Pancreatic solitary fibrous tumor(SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Here, we report the first case of malignant pancreatic SFT with typical Doege-Potter syndrome, along with the clinical and pathologic evidence of its systemic metastasis.CASE SUMMARY The patient was a 48-year-old man with a 1-year history of pancreatic and liver masses and refractory hypoglycemia. Increased uptake of the tracer fluorodeoxyglucose(FDG) was found in the liver and bones by fluorine-18 FDG positron emission tomography/computed tomography. After multidisciplinary discussion, a distal pancreatectomy procedure was performed, and histological examination showed a lesion composed of abundant heterogeneous spindle cells with localized necrosis. On immunohistochemistry evaluation, STAT6 was found to be diffusely expressed in the tumor. Based on the overall evidence, the patient was diagnosed with malignant pancreatic SFT with liver and bone metastases.CONCLUSION The diagnosis of malignant SFT requires comprehensive evidence including clinical, immunohistochemistry, and histological features. This case may be presented as a reference for diagnoses and management of malignant pancreatic SFTs with systemic metastasis.

The clinicopathological and immuohistochemical analysis of solid-pseudopapillary tumor of the pancreas:report of 9 cases

Objective：To investigate the clinical features, pathological characteristics and immunophenotype of solid-pseudopapillary tumor of the pancreas（SPTP）. Methods：Nine surgically treated cases of SPTP were retrospectively reviewed. Hematoxylin and Eosin（HE） staining and immunohistochemical staining were used to analyze all cases, and the general clinical data was collected. Results：Six patients were asymptomatic except for a palpable mass. Two patients complained of vague-epigastric pain. One patient appeared jaundice. The tumor was encapsulated and solid tissues alternately with cystic tissues. Histologically, the histological structure of solid portion was pseudopapillary with a fibrovascular core. Tumor cells were uniform and medium-sized which were arranged in sheets ets or nests or pseudopapillary patterns. Immunohistochemical studies demonstrated that SPTP proved positive in vimentin（9/9 cases）, AAT（9/9 cases）, NSE（9/9 cases）, ACT（7/9 cases）, CK20（2/9 cases）, CgA（1/9 cases）, S-100（3/gcases）, PR（4/gcases）, Syn（3/9 cases） and CD56（5/9cases）, negative in CEA and ER. Conclusion：SPTP is a tumor predominantly occurring in young women frequently without special symptoms. This tumor has various characteristical histological patterns with different immunophenotype.

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.