Pancreatic Tumors In Children Research Articles

Prognostic factors of pancreatic tumors in children and adolescents: a population study based on the surveillance, epidemiology, and end results database

PurposePancreatic tumors in children are uncommon, and data is scarce. The purpose of this study is to examine the prognostic factors of pediatric pancreatic tumors in a population-based cohort.MethodsThe Surveillance, Epidemiology, and End Results (SEER) database was used to identify all pediatric patients with pancreatic tumors diagnosed between 1975 and 2018. The overall survival (OS) rates were determined using a Kaplan-Meier analysis. The log-rank test was used for univariate survival analysis. Cox proportional-hazards regression was used to determine the variables related to OS.ResultsWe identified 195 children with pancreatic tumors, with a median age at diagnosis of 16 years. Tumors were classified as neuroendocrine tumors (33.8%), solid pseudopapillary tumors (SPTs) (32.3%), pancreatoblastoma (11.3%), and others (22.6%). Of the patients, 30.3% had distant metastases, and 69.7% had surgery. Pancreatoblastomas were more common in younger children, whereas solid pseudopapillary tumors were more common in female patients. Overall 1-year, 3-year, and 5-year survival rates for all patients were 90.3%, 79.2%, and 77.7%, respectively. The Cox proportional hazard regression revealed that SEER stage and surgery were significant independent predictors of overall survival.ConclusionsPancreatic tumors are rare in children, and overall survival is grim except for SPTs. SEER stage and surgery were determined to be the most relevant determinants of OS in our study.

Neoplasia pseudopapilar sólida del páncreas (tumor de Frantz) en una joven de 13 años

Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm with a low malignant potential and represents 1- 3% of all pancreatic tumors. They usually occur in women and are the most common solid pancreatic tumor in children. A 13-year-old girl visited the emergency department due to abdominal pain in the right hypochondrium radiating to the back, nausea and vomiting lasting 5 days. A SPT was diagnosed by ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). The patient underwent cephalic pancreaticoduodenectomy and was discharged. The pathological study reported a SPT of the pancreas. The surgical approach of these tumors through open surgery or laparoscopy offers excellent long-term survival.

Single-cell RNA sequencing of solid pseudopapillary neoplasms of the pancreas in children.

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare pancreatic tumor in children. Its origin remains elusive, along with its pathogenesis. Heterogeneity within SPN has not been previously described. In addition, low malignant but recurrent cases have occasionally been reported. To comprehensively unravel these profiles, single-cell RNA sequencing was performed using surgical specimens. We identified the cell types and suggested the origin of pancreatic endocrine progenitors. The Wnt/β-catenin pathway may be involved in tumorigenesis, while the epithelial-to-mesenchymal transition may be responsible for SPN recurrence. Furthermore, NOV, DCN were nominated as primary and S100A10, MGP as recurrent SPN marker genes, respectively. Our results provide insight into the pathogenesis of SPN.

Solid pancreatic masses in children: A review of current evidence and clinical challenges.

Pancreatic tumors in children are infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often make an accurate preoperative diagnosis difficult. Tumors are categorized as epithelial or non-epithelial, with epithelial tumors further classified as tumors of the exocrine or endocrine pancreas. Although both are tumors of the exocrine pancreas, solid pseudopapillary neoplasm is the most prevalent solid pancreatic tumor in children, while pancreatoblastoma is the most common malignant tumor. Insulinoma is the most common pediatric pancreatic tumor of the endocrine pancreas. Malignant tumors require a complete, often radical, surgical resection. However, pancreatic parenchyma-sparing surgical procedures are utilized for benign tumors and low-grade malignancy to preserve gland function. This review will discuss the epidemiology, pathophysiology, clinical and diagnostic characteristics, and management options associated with both common and rare solid pancreatic masses in children. We will also discuss current challenges encountered in their evaluation and treatment.

Imaging of pediatric pancreas tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.

Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adolescents - Tailored Diagnostic and Operative Concepts in Four Patients

Pancreatic tumors in children and adolescents are rare entities that can stay asymptomatic for long periods. They often become apparent as incidental findings or due to clinical symptoms, like abdominal pain. Solid pseudopapillary neoplasms of the pancreas (SPN) are rare representatives of this group, being low-grade malignant processes and requiring radical surgical treatment. We present four cases of SPN with different diagnostic and therapeutic approaches. A retrospective analysis of four cases of SPN treated between 2015 and 2020 was performed. Four female patients (11-17 years) were found to have SPN during diagnostic procedures. Three of them were located in the pancreatic head. Histological confirmation was obtained with endosonographic-, CT-guided and open biopsy, respectively. R0 resection was achieved by a pylorus preserving, partial duodenopancreatectomy according to Traverso-Longmire. In one patient the tumor was located in the pancreatic tail with tumor adherence to the splenic vessels. A CT guided biopsy confirmed an SPN. A distal pancreatectomy and splenectomy was performed. Follow-up (6 months - 6 years) revealed no evidence of tumor recurrence, metastasis, or pancreoprive diabetes. For the treatment plan preoperative histological confirmation of SPN is necessary. Based on the tailored diagnostic and operative concepts, treatment at a center with a specialized pediatric surgery, pediatric oncology, pediatric gastroenterology, pediatric radiology and pathology is mandatory.

Pancreatic Tumors in Children

Pancreatic Tumors in Children

Metastatic pancreatic ductal adenocarcinoma in a teenage girl: A rare disease

Pancreatic ductal adenocarcinoma (PDAC) is highly uncommon in patients < 20 years of age, at less than 0.1% of population.1 Pancreatic tumors in children and adolescents can develop from endocrine or exocrine cells. The tumor types include solid pseudopapillary tumor, ductal adenocarcinoma, pancreatoblastoma, acinar cell carcinoma, and pancreatic endocrine neoplasm (malignant and benign).2 Other types of tumors may be attached to it or secondarily engage the gland or emerge from other kinds of non-pancreatic cells inside the pancreas. The prevalent type of classic PDAC in adults is highly uncommon in children. We report here on a fifteen-year old girl with metastatic pancreatic ductal adenocarcinoma (mPDAC), who presented with abdominal discomfort and jaundice.

Pancreatoblastoma. Case report and review of literature

Although pancreatoblastoma (PB) is a rare tumor, it is the most common malignant pancreatic tumor in children. Clinic presentation is insidious, so early diagnostic suspicion allows timely therapy. We reported 3 cases of PB treated at our center. The first two cases achieved complete disease response after full tumor resection. The first one is in complete remission at 7 months after chemotherapy. The second patient is in second complete remission at 206 months after diagnosis and 128 months after metastatic relapse. The third case died from disease progression 61 months after the initial metastatic unresectable tumor. Histology, clinical features and treatment options are discussed along with presentation of the cases.

Treatment patterns and outcomes for pancreatic tumors in children: an analysis of the National Cancer Database.

Pancreatic tumors are rare in children and limited data are available regarding incidence, treatment, and outcomes. We aim to describe patient and tumor characteristics and to report on survival of these diseases. Children with pancreatic tumors were queried from the National Cancer Database (2004-2014). The association between treatment and hazard of death was assessed using Kaplan-Meier method and Cox regression model. We identified 109 children with pancreatic tumors; 52% were male and median age at diagnosis was 14years. Tumors were distributed as follows: pseudopapillary neoplasm (30%), endocrine tumors (27%), pancreatoblastoma (16%), pancreatic adenocarcinoma (16%), sarcoma (6%) and neuroblastoma (5%). Seventy-nine patients underwent surgery, of which 76% achieved R0 resection. Most patients (85%) had lymph nodes examined, of which 22% had positive nodes. Five-year overall survival by tumor histology was 95% (pseudopapillary neoplasm), 75% (neuroblastoma), 70% (pancreatoblastoma), 51% (endocrine tumors), 43% (sarcoma), and 34% (adenocarcinoma). On multivariable analysis, surgical resection was the strongest predictor of survival (HR 0.26, 95% CI 0.10-0.68, p < 0.01). Overall survival of children with pancreatic tumors is grim, with varying survival rates among different tumors. Surgical resection is associated with improved long-term survival.

High-grade neuroepithelial tumor with medulloepithelioma-like areas out of the central nervous system in an infant with hemihypertrophy: a unique association

High-grade neuroepithelial tumor with areas resembling medulloepithelioma was diagnosed in an infant with coccygeal and inguinal masses. Hemihypertrophy is associated with Wilms tumor, hepatoblastoma and pancreatic tumors in children. The authors report on the first case of peripheral HNET associated with hemihypertrophy in an infant, with special discussion on histopathological differential diagnosis and management of this rare and highly malignant tumor. HNET should be included into the list of hemihypertrophy associated tumors. Complete surgical excision with free margins is essential for the successful treatment of such cases and should be tried in suitable cases at the time of diagnosis. Continued treatment should be decided individually on a case to case basis.

Complications of solid pseudopapillary pancreatic tumors

A solid pseudopapillary tumor of the pancreas is a very rare tumor of unclear histogenesis. Its frequency is 1–2% of the total number of exocrine tumors and about 5% of cystic tumors of the pancreas. Only 130 mainly publications related to separate clinical observations of this pathology are available in medical literature. We found no works describing the complicated course of a solid pseudopapillary tumor of the pancreas in children. The article describes a clinical case with a probable complicated, life threatening course of a solid pseudopapillary pancreatic tumor in children due to peptic erosion of tissues and vessels with capsular rapture.

Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center.

Pancreatoblastoma is a very rare malignant pancreatic tumor in children. Pancreatoblastoma is the most common pancreatic tumor in children less than 10 years of age, accounting for 25% of the pancreatic neoplasm. There were only a few published literatures about the standardized diagnostic and management protocol for PB in the last decade. To summarize our experience in the management of pancreatoblastoma in children and adolescents with emphasis on the presentation, diagnosis, treatment, and outcomes. A management strategy will also be discussed. This was a retrospective case-series study of all pancreatoblastoma in patients < 18years of age who were treated at Beijing children's hospital (BCH) from January 2002-January 2015. The diagnoses of PB were confirmed by histopathology analysis of the resected specimen. The variables being analyzed included patient demographics, age at diagnosis, clinical presentation, tumor size, metastasis if present, tumor markers (AFP), type of surgery, length of follow-up, and outcome. The assessment of the tumor location, size, extent of the tumor, and distant metastasis was made by ultrasound (US), computed tomography (CT), and/or magnetic resonance imaging (MRI). 21 patients with pancreatoblastoma were diagnosed at a median age of 4years, 7 girls, and 14 boys. The diagnosis of pancreatoblastoma was identified by the histology examination. The most common syndrome was abdominal mass (n = 11), followed by abdominal pain (N = 10), elevated serum AFP levels were noted in almost all cases (17/18), 17 patients with disease initially unresectable on diagnosis accepted neo-adjuvant chemotherapy consisting of CDV, OPEC, PLADO, IEV, and AVCP. All patients underwent surgery, including pancreaticoduodenectomy (Whipple's procedure), the Pylorus-preserving pancreaticoduodenectomy (traverse-Longmire procedure), Spleen-preserving distal pancreatectomy, and distal pancreatectomy with en bloc splenectomy, Roux-en-Y end-to-end pancreatojejunostomy. In all, 13 children were disease free with a median follow-up of 53months (range 11-156months). The pancreatoblastoma in children and adolescents is a malignant tumor. Complete resection combined with chemotherapy is associated with long-term survival. For the unresectable tumor at diagnosis, preoperative chemotherapy was recommended to reduce tumor volume. AFP is critical for diagnosis and monitoring the disease as a tumors marker.

Pancreatic tumours in children

Pancreatic tumours in children

Surgical treatment of pancreatic tumors in children.

Background: Pancreatic tumors in children are uncommon. The knowledge of their presentation accrues principally through institutional case series. The very limited experience, together with the broad histological heterogeneity, has made it difficult to identify possible prognostic factors and develop treatments protocol. The aim of the present paper was perfection treatment of pancreatic tumors in children. Methods: Retrospective review of medical records and pathology reports of children who underwent surgery at National Cancer Institute from 1991 through 2016. Results: between 1990 and 2016, 18 children with pancreatic tumors were diagnosed at our institute. 2 patients( 11,7%) were treated by chemotherapy, 1 patient (5,6%) was treated by radiological therapy, in 1 case (5,6%) patient underwent symptomatic operation, in 1 case (5,6%) a pancreatic cyst was diagnosed intraoperatively and radical operation was not use. Surgical treatment was achieved in 12 patients (70,6%). RO resection was achieved in all cases. There was no postoperative mortality, but postoperative complications occurred in 1 case (8,3%) and was treated conservatively. At a median follow-up of 43 months (range 12-842 months) there were 2 tumors recurrence (16,7%). No patients had postoperative pancreatic exocrine or endocrine insufficiencies. Conclusion: In the setting of a high-volume surgical center, radical resection of pancreatic tumors in children is associated with acceptable postoperative morbidity and favorable long-term outcome.

Surgical treatment of pancreatic tumors in children

Surgical treatment of pancreatic tumors in children

Outcome after surgery for solid pseudopapillary pancreatic tumors in children: Report from the TREP project-Italian Rare Tumors Study Group.

Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow-up after pancreatic resection in children are scant. A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short-term (<30 days) and long-term complications of different surgical resections, as well as long-term follow-up were evaluated. From January 2000 to present, 43 patients (male:female=8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7-18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n=14) or from body/tail (n=29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow-up (median, 8.4 years; range, 0-17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula). Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long-term follow-up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.

P-179 - Surgical management of pancreatic tumors in children

P-179 - Surgical management of pancreatic tumors in children

A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children

To examine the clinicopathological characteristics and prognosis of pediatric patients with malignant pancreatic tumors in a population-based cohort. The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify all pediatric patients with malignant pancreatic tumors, diagnosed between 1973 and 2013. Kaplan-Meier analysis was performed to determine median and five-year overall survival (OS) rates. Univariate survival analysis was executed using the log-rank test. Cox proportional hazards model was used to identify variables independently associated with mortality. A total of 114 patients with pancreatic malignancies were identified. Median patient age was 16years and the majority of patients were white (64%) females (61.4%). The most prevalent histologic subtype was neuroendocrine tumors (35.1%), whereas pancreatoblastoma was more common during the first decade of life (P<0.001). Distant metastases were noted in 41.7% of the patients, while 33.3% and 25% had localized and regional disease respectively. Five-year OS rates were 77%, 66.4% and 64.8% for patients with pancreatoblastoma, neuroendocrine and epithelial tumors respectively. No death was observed in the solid pseudopapillary tumor group. Only history of having cancer-directed surgery (CDS) was significantly associated with lower overall mortality (HR: 5.1, 95% CI: 2.1, 12.4). Pancreatic malignancies are rare in children. Their prognosis is variable and only CDS was independently associated with superior survival. Prognosis study, Level II.

P-228 - Surgical treatment of pancreatic tumors in children

P-228 - Surgical treatment of pancreatic tumors in children