Solid Pseudopapillary Tumor Research Articles

Rare Pancreatic/Peripancreatic Cystic Lesions Can Be Accurately Characterized by EUS with Through-the-Needle Biopsy-A Unique Pictorial Essay with Clinical and Histopathological Correlations.

Due to their aspecific macroscopic appearance, uncommon pancreatic cystic lesions (PCLs) are often misdiagnosed as mucinous lesions and improperly resected. We aimed to evaluate the endoscopic ultrasound (EUS)-guided through-the-needle biopsy (TTNB) capacity of the preoperative diagnosis of uncommon PCLs. Overall, 136 patients with PCLs who underwent EUS-TTNB between 2016 and 2022 were retrospectively identified. Common histotypes (e.g., IPMN, serous cystadenoma, and mucinous cystadenoma) were excluded and 26 (19.1%) patients (15 female, mean age 52.9 ± 10.4) were analyzed. The EUS findings, adverse events (AEs), and TTNB outcomes in uncommon PCLs were evaluated. The cysts histotype was accurately diagnosed by TTNB in 24/26 (92.3%) cases (seven cystic neuroendocrine tumors, four squamoid cysts, three acinar cells cystadenomas, two lymphoepithelial cysts, two mucinous non-neoplastic cysts, two bronchogenic cysts, two cystic lymphangiomas, one solid-pseudopapillary neoplasm, and one schwannoma). In the remaining two cases, lymphangioma was eventually diagnosed after resection. Surgery was performed in 15/26 (57.7%) patients. The mean follow-up of non-surgical patients was 32.5 months. One severe acute case of pancreatitis (3.8%) that required surgery occurred after EUS-TTNB. Uncommon pancreatic/peripancreatic lesions represent the 19.1% of PCLs in our series, with mainly benign histotypes. TTNB demonstrated a high diagnostic performance with a low rate of AEs in this setting, representing a reliable tool with which to avoid useless surgery.

Solid Pseudopapillary Neoplasm of the Pancreas: A Clinicopathological Study of 12 Cases With Emphasis on Diagnostic Pitfalls.

Solid pseudopapillary neoplasm of the pancreas (SPNP) is a rare primary neoplasm with distinct clinicopathological features. The tumor most commonly occurs inyounger (premenopausal) women and is typified by low malignant potential and an excellent overall prognosis. A retrospective search over 20 years at two referral tertiary care institutions (King Faisal Hospital and Research Center andKing Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia)revealed 12 female patients diagnosed with SPNPs. The reslts of ancillary studies performed at the time of diagnosis were also reviewed and placed in the context of current recommendations. The clinical and pathological findings were reviewed. All patients were females, aged 18 to 30 years. Eight patients presented with abdominal pain, of which two experienced significant weight loss, and four presented with abdominal mass/discomfort. The tumor size ranged from 1.5 and 15 cm. Two cases were initially diagnosed as neuroendocrine tumors (NETs). One of the cases presented as a multifocal disease. All patients were treated surgically with a follow-up period between one and 11 years. Only one patient presented with peritoneal metastasis after seven years of follow-up, but generally, all are doing well. We have analyzed 12 SPNP cases in our population over 20 years (2001-2021) in this study. In brief, SPNP is a low-grade malignant potential tumor. Even though SPNP is a recognized entity, diagnostic challenges can ariseparticularly in the setting of limited sampling.Pathologists must be aware of the classic morphological features of SPNP and the characteristic profile of immunohistochemistryand be able to differentiate SPNP from other mimickers, especially well-differentiated NETs of the pancreas, andultimately to avoid misdiagnosis and unnecessary oncologic treatment. Adequate surgical resection with negative margins is associated with an excellent outcome.

Solid pseudopapillary neoplasm – Management of an extremely rare case of pancreatic tumor in a young patient

Abstract Introduction: Franz Tumor or the solid pseudopapillary neoplasm is a very rare form of pancreatic cancer, that can be held responsible for approximately 0.2-2% of the exocrine pancreatic tumors. Case report: We report a case of a 20-year-old woman, without any comorbidities, admitted to our department accusing mild abdominal pain at the level of the left hypochondrium and epigastrium, that started approximately half a year ago. Computed tomography and magnetic resonance imaging showed a voluminous encapsulated tumor, with mixed structure, apparently adherent to the tail of the pancreas, left kidney and adrenal gland, though being unable to certainly establish its visceral origin. Management and results: After analyzing all aspects of the case, we decide in favor of a left subcostal laparotomy approach; the intraoperative aspect is that of a relatively well delineated mass, adherent at the level of the pancreatic tail, therefore a complete excision of the tumor alongside with the pancreatic tissue that came in contact with it was performed. The pathological analysis reveals an encapsulated tumor with solid and pseudopapillary structure, with hemorrhagic and cystic degeneration regions; therefore, we reach the following final diagnosis: pT3 stage pseudopapillary-solid pancreatic neoplasm. Conclusions: In spite of its malignant behavior and impressive tumor volume, the surgical intervention was curative with favorable prognosis.

Extra pancreatic Primary Retroperitoneal Solid Pseudopapillary Neoplasm: A Case Report

Solid pseudopapillary neoplasm (SPN) is a rare pancreatic neoplasm. This case reports a 63-year-old female previously diagnosed with bilateral adrenocortical carcinoma post resection who presents with persistent epigastric pain. Suspicions for recurrence prompted diagnostics revealing retro pancreatic and left suprarenal foci, both suspicious for malignancy. Resection of both tumors yielded a moderately to poorly differentiated retro pancreatic carcinoma with differentials not limited to recurrence and pancreatic neuroendocrine tumor with a left suprarenal lymph node. Immunohistochemistry was then done and was consistent with SPN having a strong expression for b-catenin, vimentin, pan cytokeratin. It was non-specific to melan-A and negative for chromogranin A, synaptophysin, inhibin-a and CD10. At 6 months’ follow up, patient is clinically well and abdominal CT scan showed no recurrence. SPNs can masquerade as other neoplasms having similar clinical, radiologic and histopathologic features. Immunohistochemistry thus plays a crucial role for accurate diagnosis and management. Surgical resection still remains the treatment of choice and can provide a 95% overall survival rate, while limited evidence supports the use of adjuvant chemotherapy or radiation. Key words: Solid pseudopapillary neoplasm, extra pancreatic, immunohistochemistry

Radiomics Based on Contrast-Enhanced Ultrasound Images for Diagnosis of Pancreatic Serous Cystadenoma.

The purpose of the study was to develop and validate a radiomics model by using contrast-enhanced ultrasound (CEUS) data for pre-operative differential diagnosis of pancreatic cystic neoplasms (PCNs), especially pancreatic serous cystadenoma (SCA). Patients with pathologically confirmed PCNs who underwent CEUS examination at Chinese PLA hospital from May 2015 to August 2022 were retrospectively collected. Radiomic features were extracted from the regions of interest, which were obtained based on CEUS images. A support vector machine algorithm was used to construct a radiomics model. Moreover, based on the CEUS image features, the CEUS and the combined models were constructed using logistic regression. The performance and clinical utility of the optimal model were evaluated by area under the receiver operating characteristic curve (AUC), sensitivity, specificity and decision curve analysis. A total of 113 patients were randomly split into the training (n=79) and test cohorts (n=34). These patients were pathologically diagnosed with SCA, mucinous cystadenoma, intraductal papillary mucinous neoplasm and solid-pseudopapillary tumor. The radiomics model achieved an AUC of 0.875 and 0.862 in the training and test cohorts, respectively. The sensitivity and specificity of the radiomics model were 81.5% and 86.5% in the training cohort and 81.8% and 91.3% in the test cohort, respectively, which were higher than or comparable with that of the CEUS model and the combined model. The radiomics model based on CEUS images had a favorable differential diagnostic performance in distinguishing SCA from other PCNs, which may be beneficial for the exploration of personalized management strategies.

Preoperative CT histogram analysis to predict the expression of Ki-67 in solid pseudopapillary tumours of the pancreas

To explore the value of preoperative computed tomography (CT) histogram features in predicting the expression status of Ki-67 in patients with solid pseudopapillary pancreatic tumours (SPTP). This retrospective study analysed venous phase CT images of 39 patients with SPTP confirmed at surgery and histopathology and measured using the Ki-67 proliferation index from November 2015 to February 2022. According to the Ki-67 proliferation index, they were divided into high expression (Ki-67≥4%) and low expression (Ki-67<4%) groups. The histogram features of quantitative parameters were extracted using MaZda software, and the quantitative parameters of CT histograms were compared between groups. The receiver operating characteristic (ROC) curves of the patients were plotted according to the parameters, with statistically significant differences. The area under the curve (AUC), sensitivity, and specificity were calculated, and the effectiveness of the histogram parameters in predicting Ki-67 expression was analysed and evaluated. In total, 27 SPTP patients were enrolled, including 11 with high expression of Ki-67 and 16 with low expression. Comparative analysis of the Ki-67 high- and low-expression groups revealed a statistically significant in necrosis and variance (p<0.05). ROC curve analysis showed that the AUC of necrosis and variance predicting Ki-67 expression status were 0.753 and 0.841, the sensitivities were 81.8% and 81.3%, and the specificities were 68.7% and 81.8%, respectively. Preoperative CT histogram features help predict Ki-67 expression status in patients with SPTP.

Analysis of the clinical efficacy of laparoscopic middle pancreatectomy in the treatment of benign or low-grade malignant tumors of the pancreas.

The aim of this study was to investigate the clinical efficacy of laparoscopic middle pancreatectomy in the treatment of benign and junctional tumors of the pancreas. Retrospective analysis of basic data, tumor diameter, statistical analysis, and evaluation of efficacy-related indicators such as operative time, intraoperative bleeding, pathological findings, postoperative hospital stay, postoperative pancreatic fistula incidence, and pancreatic endocrine function was carried out on 17 patients diagnosed with benign or low-grade malignant tumors of the pancreas and laparoscopic middle pancreatic resection from January 2018 to January 2023 at the First Affiliated Hospital of Hunan Normal University. A total of 17 patients were screened. There were eight males and nine females; mean age was 42.8 ± 17.4 years (range: 15-69 years); BMI was 22.6 ± 2.5 kg/m2 (range: 18.4-27.5 kg/m2), and the tumor size was 3.4 ± 1.2cm (range: 1.5-5.5 cm). Preoperative glycan antigen CA19-9 was negative and CA125 was negative. Surgical time was 393.2 ± 57.9min; intraoperative bleeding was 211.7 ± 113.9ml; tumor diameter size was 3.4 ± 1.2cm; postoperative admission time was 19.4 ± 7.6 days; postoperative pancreatic fistula (POPF) grading was 17 cases, including nine cases of A-grade fistula, three cases of B-grade fistula, and none of C-grade fistula; postoperative pathology results were five cases of plasmacytoma, three cases of mucinous cystadenoma, four cases of SPN (solid pseudopapillary neoplasm), one case of Intraductal Papillary Mucinous Neoplasm (IPMN), three cases of pancreatic Neuroendocrine Neoplasm (pNEN), one case of inflammatory myofibroblastic osteoblastoma. All cases did not develop pancreatic origin diabetes or exacerbation of previous diabetes, and no cases presented symptoms of exocrine insufficiency such as dyspepsia and diarrhea. Laparoscopic middle pancreatectomy is safe and feasible in the treatment of benign or low-grade malignant tumors in the body of the pancreatic neck and is not accompanied by increased risk of intraoperative and postoperative complications and endocrine dysfunction of the pancreas.

Pancreatic Cancer and Its Mimics.

Pancreatic ductal adenocarcinoma (PDAC) is the most common primary pancreatic malignancy, ranking fourth in cancer-related mortality in the United States. Typically, PDAC appears on images as a hypovascular mass with upstream pancreatic duct dilatation and abrupt duct cutoff, distal pancreatic atrophy, and vascular encasement, with metastatic involvement including lymphadenopathy. However, atypical manifestations that may limit detection of the underlying PDAC may also occur. Atypical PDAC features include findings related to associated conditions such as acute or chronic pancreatitis, a mass that is isointense to the parenchyma, multiplicity, diffuse tumor infiltration, associated calcifications, and cystic components. Several neoplastic and inflammatory conditions can mimic PDAC, such as paraduodenal "groove" pancreatitis, autoimmune pancreatitis, focal acute and chronic pancreatitis, neuroendocrine tumors, solid pseudopapillary neoplasms, metastases, and lymphoma. Differentiation of these conditions from PDAC can be challenging due to overlapping CT and MRI features; however, certain findings can help in differentiation. Diffusion-weighted MRI can be helpful but also can be nonspecific. Accurate diagnosis is pivotal for guiding therapeutic planning and potential outcomes in PDAC and avoiding biopsy or surgical treatment of some of these mimics. Biopsy may still be required for diagnosis in some cases. The authors describe the typical and atypical imaging findings of PDAC and features that may help to differentiate PDAC from its mimics. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center. See the invited commentary by Zins in this issue.

Cystic pancreatic lesions-indications, timing and reasons for surveillance

Cystic pancreatic lesions are detected incidentally at an increasing rate. Often, the patients present asymptomatically. Hence, the resulting clinical consequences remain challenging and unsettling for both physicians and patients. Status of current recommendations in handling cystic pancreatic lesions. Selective literature search of PubMed while taking current guidelines into account. Correct diagnostic classification of the cystic lesion is crucial since further action depends on the type of cystic lesion. Resection is generally recommended for mucinous cystic neoplasms (MCN), solid pseudopapillary neoplasms (SPN), and intraductal papillary mucinous neoplasms (IPMN) with relevant risk criteria such as prominent main-duct dilation. Surveillance is recommended for IPMN without risk criteria, as long as comorbidities and life expectancy of the patient will allow preventive resection over the years. SCNs are benign and only symptomatic SCNs require resection. Inflammatory pancreatic cysts should only be treated under certain circumstances.

ASO Author Reflections: "Malignant" Solid Pseudopapillary Neoplasm of the Pancreas?

ASO Author Reflections: "Malignant" Solid Pseudopapillary Neoplasm of the Pancreas?

Local resection for solid pseudopapillary neoplasms of the pancreas shows improved postoperative gastrointestinal function and reduced mental stress: a multiquestionnaire survey from a large cohort.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant pancreatic tumor with a highly favorable prognosis. Most SPN patients are young and middle-aged women. The main controversial topic for SPN is local resection (LR) versus radical resection (RR). Theoretically, LR could lead to better gastrointestinal function (GIF) and less mental stress. However, no data is available to support this hypothesis. All SPN patients undergoing surgical treatment in Peking Union Medical College Hospital from 2001 to 2021 were included in the study. A cross-sectional online multiquestionnaire survey containing 110 questions was sent to them (Clinicaltrial.org, NCT05604716). This online multiquestionnaire survey focused on GIF and mental stress and consisted of eight questionnaires. Multiple linear regression analysis was conducted to identify independent factors impacting GIF and mental stress. A total of 183 cases provided valid results. Among them, 46 patients (25.1%) underwent LR, and 137 (74.9%) underwent RR. Ninety-four cases (51.4%) underwent minimally invasive surgery (MIS), while 89 (48.6%) underwent open surgery. The average GSRS score of the patients was 1.9±0.7, indicating that most suffered from mild gastrointestinal dysfunction. The scores of PHQ-9 and GAD-7 in 16 patients (8.7%) and 27 (14.8%) patients, respectively, were beyond 10.0, which indicated clinical depression and anxiety. Additionally, 19 (10.4%) patients reported poor ability to work, and 31(16.9%) patients had significant body image concerns. Compared to other clinicopathological characteristics, LR (LR vs. RR: PHQ-9 score, P =0.018; WAI average score, P =0.010; EORTC QLQ-C30, nine subdomains, P <0.05; GSRS average score, P =0.006) and MIS (MIS vs. open surgery: EORTC QLQ-C30, three subdomains, P <0.05; GSRS average score, P =0.006) were the most significant factors predicting improved GIF and reduced mental stress. This study systematically presents postoperative GIF and mental stress of SPN patients using validated multiquestionnaires for the first time. It provides solid evidence that LR and MIS can improve GIF and reduce mental stress after surgery for SPN patients, which could be helpful for the surgeons to make more personalized surgical plans for their patients.

Organ-preserving enucleation in a case of solid pseudopapillary neoplasm of pancreas

Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor affecting mostly females in the second or third decade of life. These tumors have a low malignant potential, and hence, surgical resection remains the main treatment. Large tumors may present with compressive symptoms such as abdominal pain or vomiting. Once resected completely, SPNs usually have an excellent long-term prognosis. We report a 16-year-old girl who presented with vomiting and abdominal pain localized to the right upper quadrant. Imaging showed an encapsulated tumor arising from the pancreatic head which prompted us to perform organ-preserving enucleation. Histopathology confirmed the tumor as an SPN.

The Broad Spectrum of Paediatric Pancreatic Disease: A Single-center 26-years Retrospective Review

BackgroundPaediatric pancreatic pathology and its management is rarely described. We present our experience. MethodsA retrospective case-note review of all patients with pancreatic disease from 1995 to 2021 was completed. Data are quoted as median (range). ResultsTwo hundred and twelve patients were identified with 75.9% presenting with pancreatitis. Referrals for pancreatitis increased during the study period and affected a wide age range (2 months–15.6 years).Acute pancreatitis (n = 118) (age 10.6 (0.18–16.3) years). The most common causes were idiopathic (n = 60, 50.8%) and biliary (n = 28, 23.8%). About 10% required treatment for complications or underlying biliary causes.Recurrent pancreatitis (n = 14) (11.6 (0.3–14.3) years). The most common cause was hereditary pancreatitis (n = 6, 42.9%). One patient required endoscopic drainage of pseudocyst.Chronic pancreatitis (n = 29) (16 (0.38–15.5) years). The underlying diagnosis was idiopathic (n = 14, 48.4%) or hereditary pancreatitis (n = 10, 34.5%). 13 patients required active management, including pancreaticojejunostomies (n = 5).Blunt Trauma (n = 34) was managed conservatively in 24 (70.5%). 6 patients required open surgery, but 4 were managed by either endoscopy or interventional radiology.Pancreatic tumours (n = 13) presented at 11.2 (2.3–16) years. Pathology included pancreaticoblastomas (n = 3), solid pseudopapillary tumours (n = 3), neuroendocrine tumours (n = 2), acinar cell cystadenoma (n = 1), intraductal papillary mucinous neoplasm (n = 1), pancreatic insulinoma (n = 1), pancreatic ductal adenocarcinoma (n = 1), and embryonal rhabdomyosarcoma (n = 1). Others (n = 4)Pancreatic cyst (n = 3) and annular pancreas (n = 1). ConclusionPaediatric pancreatic disease spans a wide spectrum of both benign and malignant disease and benefits from access to specialist medical, surgical, endoscopic, and interventional radiology expertise. Referrals for paediatric pancreatitis are increasing, but aetiology is different to that seen in adults. Level of EvidenceIV

Study on the application value of fluorescent laparoscopy in pancreatic tumor surgery.

Fluorescent laparoscopy is rarely used in pancreatic surgery. The aim of this study was to investigate the value of fluorescent laparoscopy in pancreatic tumor surgery. A total of 19 patients with pancreatic tumors who were treated in the Department of Hepatobiliary Surgery at the First Affiliated Hospital of Wannan Medical College from January 2021 to August 2022 were selected. Fluorescent laparoscopy was used intraoperatively, and the imaging characteristics of different tumors were recorded and analyzed. Among the 19 participants, postoperative pathology confirmed 12 cases of pancreatic cancer (8 cases of moderately differentiated adenocarcinoma, 3 cases of moderately-poorly differentiated adenocarcinoma, and 1 case of acinar cell carcinoma), 4 cases of pancreatic cystic tumors (1 case of microcystic serous cystadenoma, 1 case of serous cystadenoma, 1 case of solid pseudopapillary tumor, and 1 case of solid-cystic pseudopapillary tumor), 1 case of pancreatic neuroendocrine tumor (G1 stage), and 2 cases of inflammatory lesions. There were 8 cases of pancreaticoduodenectomy, 6 cases of distal pancreatectomy, 3 cases of middle pancreatectomy, 1 case of local pancreatectomy, and 1 case of duodenum-preserving pancreatic head resection. One minute after intravenous injection of indocyanine green (ICG), 10 of the 12 patients with pancreatic cancer showed tumor peritumor imaging; 2 cases of pancreatic serous cystic tumors did not show imaging; 2 cases of solid pseudopapillary tumors had tumor body imaging; 1 case of neuroendocrine tumor had tumor body imaging, with complete fluorescence imaging after specimen dissection; there were 2 cases pathologically confirmed as inflammatory lesions, 1 case with tumor body imaging, and 1 case with capsule imaging. By reasonably controlling the administration time and dose of ICG during surgery, some pancreatic tumors can be fluorescently imaged, which is beneficial for intraoperative tumor localization and margin determination.

S151 Characteristics and Outcomes of Solid Pseudopapillary Tumors of Pancreas: A US Multicenter Retrospective Study

S151 Characteristics and Outcomes of Solid Pseudopapillary Tumors of Pancreas: A US Multicenter Retrospective Study

Pancreatic cystic neoplasms: Still high rates of preoperative misdiagnosis in the guidelines and endoscopic ultrasound era

BackgroundA wrong diagnosis of nature is common in pancreatic cystic neoplasms. The aim of the current study is to reappraise the diagnostic errors for presumed pancreatic cystic neoplasms in patients undergoing surgery. MethodsAll pancreatic resections for presumed pancreatic cystic neoplasms following international guidelines between 2011 and 2020 were analyzed. Misdiagnosis was defined as the discrepancy between preoperative diagnosis of nature and final pathology. Mismatch was defined as the discrepancy between the preoperative suspect of malignancy (or its absence) and final pathology. ResultsA total of 601 patients were included. Endoscopic ultrasound was performed in 301 (50%) patients. Overall misdiagnosis and mismatch were 19% and 34%, respectively, with no significant benefit for those patients who underwent endoscopic ultrasound. The highest rate of misdiagnosis was reached for cystic neuroendocrine tumors (61%) and the lowest for solid pseudopapillary tumors (6%). Several diagnostic errors had clinical relevance, including 7 (13%) presumed serous cystic neoplasms eventually found to be other malignant entities, 50 (24%) intraductal papillary mucinous neoplasms with high-risk stigmata revealed to be non-malignant, and 38 (33%) intraductal papillary mucinous neoplasms without high-risk stigmata revealed to be malignant at final pathology. A preoperative presumption of malignant mucinous cystic neoplasm was correct in only 20 (16%) patients. ConclusionsDespite not always being clinically relevant, diagnostic errors are still common among resected pancreatic cystic neoplasms when applying international guidelines. New diagnostic tools beyond endoscopic ultrasound are needed to refine the diagnosis of those lesions at higher risk for unnecessary surgery or accidentally observed, nevertheless being malignant.

Solid pseudopapillary neoplasms of the pancreas in childhood and adolescence—an analysis of the German Registry for Rare Pediatric Tumors (STEP)

Solid pseudopapillary neoplasms (SPNs) are the most common entity among pediatric pancreatic tumors. Still, these are rare tumors with an annual incidence of 0.1–0.2/1,000,000, and little is known about their optimal treatment. This analysis aimed to increase knowledge about the occurrence and treatment strategies of SPN in childhood. Data regarding diagnostics, treatment, and outcome of children aged 0–18 years with SPN recorded in the German Registry for Rare Pediatric Tumors (STEP) were analyzed. Thirty-eight patients were identified with a median age of 14.5 years at diagnosis (range: 8–18) and a female preponderance (81.6%). The most frequent location of the tumor was the pancreatic tail. In histopathological and immunohistochemical examination, pseudopapillary, solid, and cystic lesions as well as expression of beta-catenin, progesterone receptors, and cyclin D1 were the most common findings. All patients underwent surgical resection. Most patients underwent open resection, predominantly tail resection for tumors in the tail region and pylorus-preserving pancreaticoduodenectomy for tumors in the head region. The main postoperative sequela was exogenous pancreatic insufficiency (23.7%), especially with SPN in the pancreatic head. No recurrence occurred during follow-up, although two patients underwent resection with microscopic residue.Conclusion: SPN of the pancreas in childhood are low-grade malignancies with usually favorable treatment outcomes. However, therapy can lead to relevant long-term sequelae. To prevent recurrence, complete surgical resection is recommended, sparing as much healthy pancreatic tissue as possible. Interdisciplinary collaboration between specialists is essential to optimize treatment. Molecular genetic analysis of these tumors could improve understanding of their genesis.What is Known:• Solid pseudopapillary neoplasms (SPNs) of the pancreas are very rare tumors in childhood.• Little is known about tumorigenesis, and there are no specific guidelines for treatment and follow-up in pediatric patients.What is New:• Characteristics, treatment, and outcome were comprehensively assessed in a large cohort of pediatric patients with SPN.• We propose recommendations for diagnosis, treatment, and follow-up of children with SPN, based on our analysis and considering published experience.

Clinical outcomes of minimally invasive duodenum-preserving pancreatic head resection

BackgroundThe procedure of total duodenum-preserving pancreatic head resection (DPPHRt) has been reported frequently, but rare in minimally invasive procedure, especially robotic-assisted operation. Here we share our experience and analyze the clinical outcomes of minimally invasive DPPHRt in the treatment of benign lesions or low-grade malignant tumors of the pancreatic head in this study.Materials and methodsFrom October 2016 to January 2022, three patients received robot-assisted DPPHRt(RA-DPPHRt), and seventeen patients received laparoscopic DPPHRt(LDPPHRt). Data were retrospectively collected in terms of demographic characteristics (age, gender, body mass index, and pathological diagnosis), intraoperative variables (operative time, estimated blood loss), and post-operative variables (post-operative hospital stay, and complications).ResultsAll 20 patients received minimally invasive total duodenum-preserving pancreatic head resection successfully without conversion, including 8 males and 12 females. Pathological diagnosis suggested 1 case of serous cystadenoma (SCA), 4 cases of intraductal papillary mucinous neoplasm (IPMN) ,5 cases of mucinous cystic neoplasm (MCN), 4 cases of pancreatic neuroendocrine neoplasm (PNET), 2 cases of chronic pancreatitis (CP),4 case of solid pseudopapillary tumor (SPT). The average operation time was (285.35 ± 95.13 min), ranging from 95 to 420 min. The average estimate blood loss was (196.50 ± 174.45ml) ,ranging from 10 to 600ml.The average post-operative hospital stay was(20.90 ± 14.44days),ranging from 8 to 54 days. Postoperative complications occurred in 10 patients (50%). A total of 5 patients (20%) suffered grade B or C pancreatic fistula. Two patients (10%) suffered from biliary fistula. Two patients (10%) suffered from delayed gastric emptying. One patient (5%) suffered from abdominal bleeding. The 90-day mortality was 0. No patient was observed tumor recurrence and new-onset diabetes but one developed diarrhea.ConclusionRA-DPPHRt or LDPPHRt provided a minimally invasive approach with good organ-preservation for patients with benign and low-grade malignant pancreatic head tumor. It is only recommended to be performed in high-volume pancreatic centers by experienced pancreatic surgeons.

High frequency of alternative splicing variants of the oncogene Focal Adhesion Kinase in neuroendocrine tumors of the pancreas and breast.

The characteristic genetic abnormality of neuroendocrine neoplasms (NENs), a heterogeneous group of tumors found in various organs, remains to be identified. Here, based on the analysis of the splicing variants of an oncogene Focal Adhesion Kinase (FAK) in The Cancer Genome Atlas datasets that contain 9193 patients of 33 cancer subtypes, we found that Box 6/Box 7-containing FAK variants (FAK6/7) were observed in 7 (87.5%) of 8 pancreatic neuroendocrine carcinomas and 20 (11.76%) of 170 pancreatic ductal adenocarcinomas (PDACs). We tested FAK variants in 157 tumor samples collected from Chinese patients with pancreatic tumors, and found that FAK6/7 was positive in 34 (75.6%) of 45 pancreatic NENs, 19 (47.5%) of 40 pancreatic solid pseudopapillary neoplasms, and 2 (2.9%) of 69 PDACs. We further tested FAK splicing variants in breast neuroendocrine carcinoma (BrNECs), and found that FAK6/7 was positive in 14 (93.3%) of 15 BrNECs but 0 in 23 non-NEC breast cancers. We explored the underlying mechanisms and found that a splicing factor serine/arginine repetitive matrix protein 4 (SRRM4) was overexpressed in FAK6/7-positive pancreatic tumors and breast tumors, which promoted the formation of FAK6/7 in cells. These results suggested that FAK6/7 could be a biomarker of NENs and represent a potential therapeutic target for these orphan diseases.

Solid pseudopapillary epithelial neoplasm of pancreas presenting as an abdominal lump: a case report

Solid pseudopapillary epithelial neoplasm (SPEN) of the pancreas is a rare cystic exocrine tumour of the pancreas that predominantly affects women between 30 and 40 years of age. This case report aims to describe clinical presentation and management of SPEN. We report a case of 20 years old female who presented with palpable lump in upper abdomen and continuous dull aching pain. Preoperative contrast enhanced computed tomography (CECT) abdomen was suggestive of large solid mass of size 11×10×10 cm with liquified content in relation to tail of pancreas with a possibility of retroperitoneal tumour or gastrointestinal tumour (GIST). Spleen sparing distal pancreatectomy with segmental resection of transverse colon with colo-colic anastomosis was performed and the diagnosis of SPEN was subsequently confirmed on histopathology examination. SPEN is a slow-growing tumour with a low-grade malignant potential found incidentally in asymptomatic patients. The average tumour size is about 4 to 6 cm in diameter at presentation. Distal pancreatectomy with splenectomy is the procedure of choice as described in literature. SPEN should be kept as a differential diagnosis in a young female patient presenting with vague abdominal pain and retroperitoneal lump in relation to the pancreas. Complete surgical resection is the definite treatment with a 5-year survival rate of 95%.