Clinical characteristics analysis of 253 cases of pancreatic solid pseudopapillary neoplasm / 中华消化杂志

Objective:To summarize the clinical characteristics of pancreatic solid pseudopapillary neoplasm (pSPN).Methods:From March 2007 to August 2022, at the First Affiliated Hospital of Zhejiang University School of Medicine, the clinical data and follow-up results of 253 patients with pSPN and underwent surgical treatment were retrospectively analyzed. The diagnostic accuracy of preoperative ultrasound, magnetic resonance imaging (MRI), computed tomography (CT) and puncture biopsy of aspiration were compared and analyzed. Kaplan-Meier method was performed to calculate disease-free survival rate. The gender differences in the clinical and pathological features of pSPN were compared. According to whether recurrence and metastasis occurred after the primary operation, the patients with pSPN were divided into recurrence and metastasis group ( n=3) and disease-free survival group ( n=250). Univariate analysis was used to analyze whether gender, age, married status, with clinical symptoms, history of alcohol drinking or smoking, puncture biopsy of aspiration before operation, location and maximum diameter of tumor, surgical method (open surgery, minimally invasive surgery), type of surgery (parenchymal preservation surgery, conventional surgery), and pathological features (cellular atypia, invasion of adjacent organs, lymphovascular invasion, peripancreatic fat invasion, perineural invasion, capsular invasion and pancreatic parenchyma invasion) were predictive factors of recurrence and metastasis of pSPN. Two independent sample t test, chi-square test and Fisher′s exact test were used for statistical analysis. Results:Among 253 patients with pSPN, 49 (19.4%) were males and 204 (80.6%) were females. The diagnostic accuracy of MRI and CT were both higher than that of ultrasound (73.4%(124/169), 64.0%(146/228) vs. 33.3%(78/234)), and the diagnostic accuracy of MRI was higher than that of CT, and the differences were statistically significant ( χ2=62.93, 43.58 and 3.89, P<0.001, <0.001 and =0.049). The diagnostic accuracy of puncture biopsy of aspiration combined with immunochemistry was higher than that without combined immunochemistry (100.0%(23/23) vs. 8/13), and the difference was statistically significant (Fisher′s exact test, P=0.003). Eight cases missed during follow-up, and all the other 245 patients survived with the 5- year and 10-year disease-free survival rates of 99.2% and 97.2%, respectively. Male pSPN patients were older than female patients at diagnosis ((40.0±13.2) years old vs. (32.6±11.9) years old) and the maximum diameter of tumor was smaller than that of female patients ((3.88±2.05) cm vs. (4.87±3.05) cm), and the differences were statistically significant ( t=3.83 and -2.15, P<0.001 and =0.032). The results of univariate analysis showed that lymphovascular invasion was significantly correlated with pSPN recurrence and metastasis (Fisher′s exact test, P=0.012), and the other factors were not correlated with pSPN recurrence and metastasis (all P>0.05). Conclusions:CT, MRI and puncture biopsy of aspiration are comparatively reliable diagnostic method for pSPN before operation. There are significant differences in pSPN tumor growth and age of onset between genders. The postoperative recurrent rate of pSPN is low and the prognosis is good. Even if recurrence and metastasis occur, the patients can still survive for a long time after surgical treatment.

Extrapancreatic 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 retropancreatic and left suprarenal foci, both suspicious for malignancy. Resection of both tumors yielded a moderately to poorly differentiated retropancreatic 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, pancytokeratin. 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.

Progress and controversies in diagnosis and treatment of solid pseudopapillary neoplasm of the pancreas / 国际外科学杂志

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare low-grade malignant tumor. In recent years, with the improvement of national health check-up awareness and the advancement of imaging diagnostic technology, the detection rate of SPN has continued to rise. With the release of guidelines for many pancreatic cystic tumors at home and abroad, the diagnosis and treatment of SPN has gradually become standardized. However, its diagnostic and treatment strategies are still difficult and controversial. Therefore, the author summarizes the current status of diagnosis and treatment of SPN by referring to the literature and combining clinical experience, in order to deepen the understanding of rare pancreatic tumor by clinicians and further promote the standardized diagnosis and treatment of SPN in China.

Multiphasic enhanced CT-based radiomics signature for preoperatively predicting the invasive behavior of pancreatic solid pseudopapillary neoplasm / 中华放射学杂志

Objective:To explore the value of multiphasic CT-based radiomics signature in predicting the invasive behavior of pancreatic solid pseudopapillary neoplasm (pSPN).Methods:The multiphasic CT images of patients with pSPN confirmed by postoperative pathology in the First Affiliated Hospital of Zhengzhou University from January 2012 to January 2021 were analyzed retrospectively. There were 23 cases of invasiveness and 59 cases of non-invasiveness. The region of interest(ROI) was artificially delineated layer by layer in the plain scan, arterial-phase and venous-phase images, respectively. The 1 316 image features were extracted from each ROI. The data set was divided into training and validation sets with a ratio of 7∶3 by stratified random sampling, and synthetic minority oversampling technique (SMOTE) algorithm was used for oversampling in the training set to generate invasive and non-invasive balanced data for building the training model. The constructed model was validated in the validation set. The receiver operating characteristic(ROC) analysis was used to evaluate model performance and the Delong′s test was applied to compare the area under the ROC curve (AUC) of different predict models. The improvement for classification efficiency of each independent model or their combinations were also assessed by net reclassification improvement (NRI) and integrated discrimination improvement (IDI) indices.Results:After feature extraction, 2, 6 and 3 features were retained to construct plain-scanned model, arterial-phase and venous-phase models, respectively. Seven independent-phase and combined-phase models were established. Except the plain-scanned model, the AUC values of other models were greater than 0.800. The arterial-phase model had the best efficiency for classification among all independent-phase models. The AUC values of arterial-phase model in the SMOTE training and validation sets were 0.913 and 0.873, respectively. By combining the radiomics signature of the arterial-phase and venous-phase models, the AUC values of training and validation sets increased to 0.934 and 0.913 respectively. There were no significant differences of the AUC values between the scan-arterial venous-phase model and arterial venous-phase model in both training and validation sets (both P>0.05). The NRI and IDI indexes showed that the combined form of plain-scan model and arterial-venous-phase model could not significantly improve the classification efficiency in the validation set (both NRI and IDI<0). Conclusions:The arterial-phase CT-based radiomics model has a good predictive performance in the invasive behavior of pSPN, and the combination with a venous-phase radiomics model can further improve the model performance.

Combined Laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation: report of one case and literature review / 中华器官移植杂志

Objective:To Eveluate the safty and clinical efficacy of combined laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation in the treatment of solid pseudopapillary neoplasm.Methods:A 22 years old solid pseudopapillary neoplasm female patient who underwent distal pancreatectomy and an autologous islet transplantation at Tianjin First Central Hospital, clinical date for 6 months follow up was collected and analyzed.Results:The patient was well recovered after surgery, and during the post-operative follow up, the fasting blood glucose was 5.72 mmol/L, HbA1c was 6.1%, remained insulin independent, the liver function was kept well.Conclusions:Combined Laparoscopic spleen-preserving distal pancreatectomy and autologous islet transplantation can effectively prevent diabetes after distal pancreatectomy.

Neoplasia Pseudopapilar sólida del Páncreas. A Propósito de un Caso / Solid Pseudopapillary Neoplasia of the Pancreas. Case Report

Introducción: La neoplasia pseudopapilarsólida del páncreas es una patología poco frecuente, que se presenta en mujeres jóvenes y es de comportamiento incierto. La prevalencia es del 1-2% y el tratamiento es quirúrgico. En el presente caso se describe a una paciente de sexo femenino de 10 años de edad que muestra esta patología y su correspondiente manejo. Caso clínico:Paciente de sexo femenino, de 10 años de edad, con antecedentes de una nefrectomía hace 6 años por hidronefrosis, refiere dolor abdominal localizado en egigastrio tipo cólico y pérdida de peso. En los estudio de imagen a nivel peritoneal se observa lesión de aspecto ocupativo de tipo sólido neoplásico con área central de probable necrosis y que desplaza en sentido posterior al páncreas así como al estómago y se asocia con adenomegalias peritoneales y retroperitoneales. Evolución:Lapaciente fue sometido a una espleno-pancreatectomía distal.El estudio anatomo-patológico concluyó en neuplasia pseudopapilar sólida del páncreas.Continúa en valoración por consulta externa. Conclusión:Neoplasia Pseudopapilar sólida del Páncreasdebe ser sospechada en pacientes con una masa sólida o quística pancreática con un estudio de imágenes compatibles. Palabras clave:Neoplasia pseudopapilar sólida del páncreas, prevalencia, inmunohistoquímica, receptores progestágenos,Informe de caso

Introduction:Solid pseudopapillary neoplasia of the pancreas is a rare pathology that occurs in young women and has an uncertain behavior. The prevalence is 1-2% and the treatment is surgical. In the present case, a 10-year-old female patient with this pathology and its corresponding management is described. Clinical case: A 10-year-old femalepatient with a history of nephrectomy 6 years ago due to hydronephrosis, complains of colicky localized abdominal pain in the egigastrium and weight loss. In peritoneal imaging studies, a neoplastic solid type occupational lesion with a central area of probable necrosis is observed, which displaces the pancreas as well as the stomach posteriorly and is associated with peritoneal and retroperitoneal adenomegaly. Evolution: The patient underwent a distal spleno-pancreatectomy. The anatomopathological studyconcluded in solid pseudopapillary neuplasia of the pancreas. He continues to be evaluated by external consultation. Conclusion: Solid Pseudopapillary Neoplasia of the Pancreas should be suspected in patients with a solid or cystic pancreatic mass with a compatible imaging study. Key words:Solid pseudopapillary neoplasm of the pancreas, prevalence, immunohistochemistry, progestin receptors, Case Report.

Spleen Preservation in Laparoscopic Distal Pancreatectomy for Solid Pseudopapillary Neoplasm is Oncologically Safe

PURPOSE: Laparoscopic distal pancreatectomy (LDP) has been widely performed for solid pseudopapillary neoplasm (SPN) involving the body or tail of the pancreas. However, it has not been established whether spleen preservation in LDP is oncologically safe for the treatment of SPN with malignant potential. In this study, we compared the short- and long-term outcomes between patients with SPN who underwent laparoscopic spleen-preserving distal pancreatectomy (LSPDP) vs laparoscopic distal pancreatectomy with splenectomy (LDPS). METHODS: We retrospectively reviewed the medical records of 46 patients with SPN who underwent LDP between January 2005 and November 2016. Patients were divided into 2 groups according to spleen preservation: the LSPDP group (n=32) and the LDPS group (n=14). Clinicopathologic characteristics and perioperative outcomes were compared between groups. RESULTS: There were no significant differences in pathologic variables, including tumor size, tumor location, node status, angiolymphatic invasion, or perineural invasion between groups. Median operating time was significantly longer in the LSPDP group vs the LDPS group (243 vs 172 minutes; p=0.006). Estimated intraoperative blood loss was also significantly greater in the LSPDP group (310 vs 167 ml; p=0.063). There were no significant differences in incidence of postoperative complications (≥ Clavien-Dindo class IIIa) or pancreatic fistula between groups. After a median follow-up of 35 months (range, 3S153 months), there was no recurrence or disease-specific mortality in either group. CONCLUSION: The results show that LSPDP is an oncologically safe procedure for SPN involving the body or tail of the pancreas.

Analysis of 4 children with pancreatic solid pseudopapillary neoplasm treated by total laparoscopic pancreaticoduodenectomy / 中华胰腺病杂志

Objective To investigate the application and surgical experience of total laparoscopic pancreaticoduodenectomy ( TLPD ) in treating children with solid pseudopapillary neoplasm ( SPN ) of pancreas. Methods Clinical data 4 children with SPN who underwent TLPD in Jilin University First Hospital from April 2017 to June 2018 were retrospectively analyzed. Results Among the 4 children, a case was male and 3 cases were female. Their age ranged from 9 to 14 year-old, the height ranged from 1. 2 to 1. 7 meters, and body weight ranged from 30 to75 kg. All patients complained of upper abdominal pain, one child had nausea and vomiting, and one child had abdominal mass. All patients underwent abdominal enhanced CT scan before operation, which showed a mass-like low-density shadow or mixed density shadow in the pancreatic head, with slightly uneven enhancement or no obvious enhancement. All 4 patients underwent TLPD, and the operation was successful without conversion to open surgery. The duration of operation time ranged from 250 to 365 minutes, the intraoperative blood loss ranged from 80 to 120 ml, the tumor size ranged from 4 to 8 cm, and the hospital stay ranged from 10 to 22 days. One patient developed grade B pancreatic fistula after surgery and was cured after conservative treatment. Pathological examinations of all patients confirmed the diagnosis of pancreatic SPN. All patients were followed up until February 2019, and no significant discomfort was observed and no recurrence or metastasis was found. Conclusions TLPD was safe and feasible in children in relatively large pancreatic surgery centers with extensive laparotomy and TLPD experience.

Analysis of 4 children with pancreatic solid pseudopapillary neoplasm treated by total laparoscopic pancreaticoduodenectomy / 中华胰腺病杂志

Objective@#To investigate the application and surgical experience of total laparoscopic pancreaticoduodenectomy (TLPD) in treating children with solid pseudopapillary neoplasm (SPN) of pancreas.@*Methods@#Clinical data 4 children with SPN who underwent TLPD in Jilin University First Hospital from April 2017 to June 2018 were retrospectively analyzed.@*Results@#Among the 4 children, a case was male and 3 cases were female. Their age ranged from 9 to 14 year-old, the height ranged from 1.2 to 1.7 meters, and body weight ranged from 30 to75 kg. All patients complained of upper abdominal pain, one child had nausea and vomiting, and one child had abdominal mass. All patients underwent abdominal enhanced CT scan before operation, which showed a mass-like low-density shadow or mixed density shadow in the pancreatic head, with slightly uneven enhancement or no obvious enhancement. All 4 patients underwent TLPD, and the operation was successful without conversion to open surgery. The duration of operation time ranged from 250 to 365 minutes, the intraoperative blood loss ranged from 80 to 120 ml, the tumor size ranged from 4 to 8 cm, and the hospital stay ranged from 10 to 22 days. One patient developed grade B pancreatic fistula after surgery and was cured after conservative treatment. Pathological examinations of all patients confirmed the diagnosis of pancreatic SPN. All patients were followed up until February 2019, and no significant discomfort was observed and no recurrence or metastasis was found.@*Conclusions@#TLPD was safe and feasible in children in relatively large pancreatic surgery centers with extensive laparotomy and TLPD experience.

Clinical and pathological features of solid pseudopapillary neoplasm of the pancreas: An analysis of 14 cases / 临床肝胆病杂志

ObjectiveTo investigate the clinical features, pathological features, differential diagnosis, and prognosis of solid pseudopapillary neoplasm (SPN) of the pancreas. MethodsA retrospective analysis was performed for the clinical data of 14 patients with SPN who were treated in our hospital from January 2014 to February 2018, and related articles were reviewed. ResultsThere were 11 female and 3 male patients with an age of onset of 13-77 years (mean 33.1 years). Most of them attended the hospital due to lesions found by physical examination or the presence of upper abdominal pain. Radiological examination revealed space-occupying lesion in the pancreas. The maximum diameter of the tumor ranged from 0.6 cm to 22 cm. Histological examination showed that most tumors were composed of solid areas and pseudopapillary areas, with a microcystic structure in local lesion. Immunohistochemistry showed positive Vimentin and negative CgA ,Glucagon, Gastrin, and Insulin in all patients. Some patients were positive for β-catenin (13/14), CD10 (10/14), CD56 (9/14), Syn (8/14), AA-T (11/14), PR (8/14), CyclinD1 (9/14), CA19-9 (3/14), CK-pan (9/14), CEA (1/14), and P53 (1/14). Ki-67 index was ≤10% in all 14 patients. ConclusionSPN of the pancreas should be diagnosed with reference to clinical data, imaging examination, histological features, and immunohistochemistry. The microcystic structure has a certain value in the diagnosis of SPN.

Pancreatoduodenectomy for solid pseudopapillary tumor of the pancreas: a multi-institution study / Duodenopancreatectomia para o tumor pseudopapilar sólido do pâncreas: estudo multi-institucional

ABSTRACT Background: Solid pseudopapillary tumor of the pancreas is a rare low-grade malignant neoplasm. Most patients present with nonspecific symptoms until the tumor becomes large. Complete surgical resection by pancreatoduodenectomy is the treatment of choice for tumors located in the head of the pancreas Aim: To analyzed the clinicopathologic features, management, and outcomes of patients who had solid pseudopapillary tumor of the head pancreas and underwent surgical resection. Methods: Were analyzed 16 patients who underwent pancreatoduodenectomy for this condition. Results: Mean age was 25.7 years old, and 15 patients were female (93.7%). Nonspecific abdominal pain was present in 14 (87.5%). All underwent computed tomography and/or magnetic resonance imaging as part of diagnostic workup. The median diameter of the tumor was 6.28 cm, and surgical resection was performed with open or laparoscopic pancreatoduodenectomy without neoadjuvant chemotherapy. Postoperative complications occurred in six patients (37.5%) and included pancreatic fistula without mortality. The mean of hospital stay was 10.3 days. Median follow-up was 3.6 years, and no patient had local recurrence or metastatic disease. Conclusion: For these patients surgical resection with pancreatoduodenectomy is the treatment of choice showing low morbidity, no mortality, and good long-term survival.

RESUMO Racional: Tumor sólido pseudopapilar do pâncreas é neoplasia maligna rara, de baixo grau de malignidade. A maioria dos pacientes apresenta sintomas inespecíficos até que o tumor aumente de tamanho. A ressecção cirúrgica completa através a duodenopancreatectomia é o tratamento de escolha para os localizados na cabeça do pâncreas. Objetivo: Analisar as características clinicopatológicas, tratamento e resultados de pacientes com tumor sólido pseudopapilar do pâncreas localizado na cabeça do pâncreas submetidos à ressecção cirúrgica. Método: Foram analisados 16 pacientes com duodenopancreatectomia devido a esse tumor localizado na cabeça do pâncreas. Resultados: Havia 15 mulheres (93,7%) e a média de idade era de 25,7 anos. Dor abdominal não específica esteve presente em 14 pacientes (87,5%). Todos realizaram tomografia computadorizada do abdome e/ou ressonância nuclear magnética como parte da investigação. O diâmetro médio do tumor era de 6,28 cm e a ressecção cirúrgica foi realizada por duodenopancreatectomia, tanto por laparotomia quanto por videolaparoscopia, com ou sem quimioterapia neoadjuvante. As complicações pós-operatórias ocorreram em seis pacientes (37,5%) e incluíram fístula pancreática, sem mortalidade. O tempo médio de internação hospitalar foi de 10,3 dias. O tempo médio de seguimento foi de 3,6 anos e nenhum paciente apresentou recorrência local ou doença metastática. Conclusões: A ressecção cirúrgica através da duodenopancreatectomia é o tratamento de escolha para estes pacientes. Os resultados mostraram baixa morbidade, nenhuma mortalidade e boa sobrevida em longo prazo.

Analysis of diagnosis and treatment of solid pseudopapillary neoplasm of pancreas in 12 children / 中华实用儿科临床杂志

Objective To analyze the experience in diagnosis and surgical treatment for solid pseudopapillary neoplasm(SPN) of pancreas in children.Methods A retrospective study was performed in 12 pediatric patients with SPN who had been admitted to Affiliated Cancer Hospital of Zhengzhou University during January 2004 to December 2016,and their general data,demographic data,types of operations,postoperative complications and follow-up were analyzed.Results Among the 12 patients,3 cases were male and 8 cases were female,with average age 14.3 years old (11-17 years old).The main clinical manifestations included abdominal pain(4/12 cases,33.3％),abdominal mass (2/12 cases,16.7％) and trauma(2/12 cases,16.7％).In those 12 patients,33.3％ (4/12 cases) SPN was located at the head of the pancreas,and 66.7％ (8/12 cases) at the body and tail of it.The tumors were usually large,the largest diameter ranged from 4.0 to 15.3 cm(average largest diameter,8.2 cm).The color uhrasonography indicated heterogeneous echogenic mass and clear boundary.CT scanning indicated that the tumor was a low-density cystic mass with a clear boundary,with enhanced tumor real component and irregular reinforcement.No calcification was found in the patients.Dynamic enhanced magnetic resonance imaging scan revealed gradual strengthening solid components in tumor.All the patients received surgical resection,with distal pancreatectomy in 4 patients,pancreaticoduodenectomy in 4 patients,spleen-preserving distal pancreatectomy in 2 patients,Enucleation in 1 patient,and distal pancreatectomy and self-splenic slices transplantation in 1 patient.Lymphadenectomy was performed in 4 patients,and all the 21 removed lymph nodes were all negative.Pathological diagnosis confirmed the SPN in all the patients,among them 3 cases were malignant SPN,and one of them with tumor rupture and hemorrhage.The mean follow-up duration was 57.7 months(19-156 months) and no recurrence was found.Conclusion SPN is a rare neoplasm in children who go to see doctors because of clinical symptoms.Surgical resection,especially organs-preserving resection,may improve the long-term results.

Indocyanine Green-Fluorescent Pancreatic Perfusion-Guided Resection of Distal Pancreas in Solid Pseudopapillary Neoplasm: Usefulness and Feasibility During Pancreaticobiliary Surgery

ICG is widely applied in real-time imaging during abdominal surgery, plastic surgery, as well as oncologic staging and treatment. A twenty-eight year-old female patient was found to have a 4.5 cm solid pseudopapillary neoplasm in the tail of the pancreas. Under ICG-fluorescent pancreatic perfusion-guidance, we easily defined the margin of the pancreatic tumor and secured the resection margin when performing laparoscopic distal pancreatosplenectomy in the patient. No clinically relevant complications, including postoperative pancreatic fistula, were noted. Intravenous ICG can be very easily and quickly detected in the pancreas under near infrared light. This enhanced vision gives strong contrast to the organ compared to a necrotic tumor with poor blood perfusion, such as solid pseudopapillary neoplasm. Based on our current experience, ICG pancreatic perfusion-guided determination of appropriate resection margin is useful and feasible during pancreaticoduodenectomy.

Solid pseudopapillary neoplasm of pancreas arising from majus omentum: A case report and literature review / 吉林大学学报(医学版)

Objective: To explore the pathological diagnosis features and differential diagnosis of one case of patient with solid pseudopapUlary neoplasm (SPN) of pancreas occurred in the majus omentum, and to improve the understanding of extra-pancreatic SPN. Methods : One female patient was admitted to hospital because of abdominal mass found 20 d ago. The patient was diagnosed as multiple hysteromyoma by gynecological color ultrasound, and the results of abdominal ultrasound had no obvious abnormity. The patient underwent hysterotomy, and a well-defined mass in the majus omentum was found during operation, so the patient received hysterectomy and omental tumor resection. Results : The patient was confirmed as multiple hysteromyoma by the postoperative pathological examination. The patient was clearly diagnosed as SPN arising from the majus omentum on the basis of pathological characteristics and immunohistochemical staining results. There were no ectopic pancreas tissue arround the tumor and no SNP metastasis in examination of ultrasound and CT. No recurrence and metastasis were found in the follow-up of 3 years. Conclusion: SPN arising from the majus omentum is extremely rare. The tumor has clear boundary, typical morphology and immunohistochemical appearances.

Ten-year case review of solid-pseudopapillary neoplasm of pancreas / 西安交通大学学报(医学版)

Objective To summarize the methods of diagnosing and treating solid-pseudopapillary neoplasm of pancreas SPN so as to provide reference for its early clinical diagnosis.Methods We collected the clinical data of 62 patients with pathologically confirmed diagnosis of SPN treated in the First Affiliated Hospital of Xi'an Jiaotong University between Feb.2004 and Sep.2014.Then we retroactively analyzed and summarized the clinical characteristics,diagnosis and treatment of the disease.Results Among the 62 patients,55 were female and 7 were male;the mean age was (31.58±12.67) years old.The clinical features showed no specificity and the tumor was mostly detected upon physical check-up.The tumor was seen to be located mostly in the body and tail of the pancreas,and the average maximum diameter was (7.81±3.54)cm.We did not find obvious abnormality in routine pre-operative blood test results or liver and kidney functions.The imageological examination indicated tumor occupation in the pancreas.All the patients underwent surgical resection with no complications or death and had a good recovery after operation.Pathological diagnosis after operation was SPN without metastasis in lymph modes.Until the last time we followed up all the patients,we found relapse in two patients (3.2％).Conclusion SPN is a tumor that tends to affect young and middle-aged females.The malignancy grade of SPN is low,and patients with this disease show no specificity in clinical manifestation.The imageological examination is of vital importance in diagnosing SPN and surgical resection is an effective way to treat SPN.

Solid pseudopapillary neoplasm of the pancreas with liver metastasis initially misinterpreted as benign haemorrhagic cyst

Solid pseudopapillary neoplasm (SPN) of the pancreas is considered a low-malignant neoplasm witha good prognosis. However, 5% to 15% of patients with SPNs develop metastatic disease, mostcommonly in the liver. Metastatic hepatic malignancies that show pseudocystic features are rare.Here we describe the case of a middle-aged female with a cystic liver metastasis from SPN. To thebest of our knowledge, SPN with a single cystic liver metastasis has not been described, althoughthese tumours frequently undergo haemorrhagic-cystic degeneration. Thus, in these patients themarked cystic change could be misinterpreted as a benign lesion.

A Rare Case of Recurrent Metastatic Solid Pseudopapillary Neoplasm of the Pancreas

A 61-year-old woman visited our hospital for bilateral multiple lung nodules and a mass in her thorax. She had a long history of multiple metastatic recurrences of solid pseudopapillary neoplasm (SPN); 24 years previously, the patient had undergone pylorus-preserving pancreaticoduodenectomy for a 9.9 × 8.6 cm mass in the pancreatic head. The tumor was diagnosed as an SPN. Nine years later, metastatic nodules were found on computed tomography in the patient's liver and peritoneum and were excised. She subsequently underwent an additional eight metastatectomy procedures in diverse organs. For the presented event, the lung nodules were removed. The prevalence of malignant SPN in the general population is 5%–15%. However, multiple metastatic recurrence of malignant SPN is rare; the lung is a particularly rare site of metastasis, found in only three cases in the literature. Here, we describe this exceptional case and provide a literature review.

Solid pseudopapillary neoplasm of the ovary with metastases to the omentum and regional lymph nodes.

Extrapancreatic solid pseudopapillary neoplasms (SPNs) are rare tumors, which bear morphological, immunohistochemical, and molecular features similar to those of pancreatic counterparts. SPN occurs primarily in adolescent girls and young women. It is considered to be a malignant neoplasm with low‑grade biology. Ovarian SPNs are uncommon, have benign morphology, usually limited to the ovary and local surgical excision is curative. We report an unusual case of SPN of right ovary with extraovarian spread and metastases to lymph nodes. To the best of our knowledge, this is the second documented case of extragonadal spread of ovarian SPN.

Surgical treatment of patients with solid-pseudopapillary neoplasm of pancreas: A single center experience in 51 cases / 第二军医大学学报

Objective To summarize the clinicopathologic features of solid-pseudopapillary neoplasm of pancreas (SPNP), and to explore its diagnosis and surgical strategy. Methods The clinical data of 51 patients with SPNP were collected from Department of Pancreas Surgery, Changhai Hospital between September 2008 and June 2012, and the clinical characteristics and surgical strategy were retrospectively analyzed. The patients were divided into solid tumor and cystic tumor group according to tumor texture, and a case-control study was designed to analyze the correlation between tumor texture and invasive pathological characteristics. Results Forty-five of the 51 patients were females and 6 were males (7. 5:1), with a mean age of (33. 5±10. 8) years old at diagnosis, ranging from 8 to 53 years. Twenty-two (43. 1%) cases with no symptom were detected by physical examination, 23 cases were detected by epigastric pain (16, 31. 4%) or discomfort (7, 13. 7%), 3 (5. 9%) patients had a palpable abdominal mass and another 3 (5. 9%) had nausea and vomiting. The average diameter of the neoplasms was (6. 3±5. 8) cm (ranged from 1 to 37. 8 cm). The neoplasms were mainly located at the body and tail of the pancreas (25, 49. 0%), followed by the neck (14, 27. 5%) and head (12, 23. 5%) of the pancreas. The patients underwent surgical resection via pancreaticoduodenectomy (n=5), pylorus-preserving pancreaticoduodenectomy (n=5), segmental pancreatectomy (n=7), distal pancreatectomy (n=21), spleen-preserving distal pancreatectomy (n=7) and local resection of pancreatic neoplasms (n=6). Only one patient had tumor marker (CA19-9) elevated slightly, and others had normal level (CA19-9, CEA, CA125). Pathologic results showed that 15 of the 51 patients had invasive pathological characteristics. The results of case-control study indicated that solid tumor was significantly correlated with pancreas parenchymal invasion (P<0. 01), cellular pleomorphism (P<0. 01) and perineural invasion (P<0. 05). Six of the 51 patients were lost to follow-up, and the follow-up time of other patients lasted for 26-74 months, during which no evidence of recurrence or metastasis was noted. Conclusion SPNP is a rare low-grade malignant tumor; surgical resection is currently the optimal and effective treatment for SPNP, and conservative operation procedure is better than the relatively aggressive ones. This study also suggests that solid tumor is associated with some malignant pathological characteristics, such as pancreas parenchymal invasion, cellular pleomorphism and perineural invasion.

La neoplasia sólida-pseudopapilar del páncreas como entidad enigmática / The solid tumor-the pancreas pseudopapillary as enigmatic entity

El tumor sólido-pseudopapilar del páncreas, conocido también como tumor de Frantz, es una rara enfermedad. Afecta principalmente a mujeres jóvenes, no blancas. Tiene componentes sólidos y quísticos. Constituye una neoplasia maligna con un pronóstico, generalmente alentador. Realizamos una revisión de la literatura con el objetivo de actualizar algunos puntos clave acerca del tema. Se incluyeron para la búsqueda las siguientes palabras claves: tumor sólido-quístico, tumor quístico papilar, pseudopapilar, sólido y quístico, tumor de páncreas, tumor pancreático, neoplasia del páncreas, neoplasia pancreática, sólido pseudopapilar y tumor de Frantz. Los acápites que desarrollamos son patogénesis, biología molecular, patología, características clínicas, imaginología y diagnóstico, tratamiento y por último pronóstico. Existen algunos aspectos no bien dilucidados en estos tumores. Por ejemplo, se necesitan más investigaciones en áreas como patogénesis, biología molecular y tratamiento no quirúrgico(AU)

Solid-pseudopapillary of the pancreas, also known as Frantz tumor, is a rare disease. It mainly affects non-Caucasian young women; it has solid and cystic components and represents a malignant neoplasm with generally encouraging prognosis. To this end, a literature review was made to update some key points on the subject. The following keywords were used: solid-cystic turmor, papillary cystic tumor, pseudopapillary, solid and cystic, pancreas tumor, pancreatic tumor, pancreas neoplasm, pancreatic neoplasm, solid-pseudopapillary and Frantz' tumor. The sections that we further developed were pathogenesis, molecular biology, pathology, clinical characteristics, imaging and diagnosis, treatment and finally prognosis. There are some poorly clarified in these tumors, so more research work is needed in pathogenesis, molecular biology and non-surgical treatment(AU)