Pancreatoblastoma

The diagnosis of solid pseudopapillary neoplasm of the pancreas (SPN) can be challenging due to potential confusion with other pancreatic neoplasms, particularly pancreatic neuroendocrine tumors (NETs), using current pathological diagnostic markers. We conducted a comprehensive analysis of bulk RNA sequencing data from SPNs, NETs, and normal pancreas, followed by experimental validation. This analysis revealed an increased accumulation of peroxisomes in SPNs. Moreover, we observed significant upregulation of the peroxisome marker ABCD1 in both primary and metastatic SPN samples compared with normal pancreas and NETs...

PURPOSE: Pancreatic 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. METHODS: The 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...

Pancreatoblastoma (PB) is a rare malignant pancreatic epithelial tumor that mostly occurs in children and occasionally occurs in adults. The tumor has acinar cell differentiation and squamous corpuscles/squamous epithelial islands, which are frequently separated by fibrous bundles. Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease characterized by the presence of numerous adenomatous polyps in the colon and rectum. Cases of pancreatoblastoma combined with familial adenomatous polyposis (FAP) are rarely reported...

CONTEXT.—: The examination of small pancreatic biopsies is a difficult task for pathologists. This is due to the scant and fragmented material often obtained from diagnostic procedures as well as the significant overlap between different neoplastic and nonneoplastic entities. In the upcoming neoadjuvant era, biopsies could become even more important, representing the only possibility to look at the real histomorphology of tumors before chemotherapy-induced modifications. OBJECTIVES...

BACKGROUND AND AIMS: Cytological and histopathological diagnosis of non-ductal pancreatic neoplasms can be challenging in daily clinical practice while it is crucial for therapy and prognosis. The cancer methylome is successfully used as a diagnostic tool in other cancer entities. Here, we investigate if methylation profiling can improve the diagnostic work-up of pancreatic neoplasms. METHODS: DNA methylation data were obtained for 301 primary tumors spanning six primary pancreatic neoplasms and 20 normal pancreas controls...

BACKGROUND: Adult pancreatoblastoma (PBL) is a rare pancreatic malignancy, with recent evidence suggesting a possible link to familial adenomatous polyposis (FAP). This study aims to review the latest evidence and explore a possible association between adult PBL and FAP. METHODS: Two independent literature reviews were conducted: (1) on PBL and FAP, and (2) on PBL in the adult population not diagnosed with FAP. RESULTS: Out of 26 articles on PBL and FAP screened, 5 were selected for systematic review, including 1 additional case...

In this report we present a rare case of pancreatoblastoma in an adult patient. Whilst they are amongst the most common malignant pancreatic tumours in children, presentations in adults are exceedingly rare, with a small number of reported cases. Its presentation is often non-specific in terms of clinical examination, and subsequent imaging can show similar findings to those seen in benign neoplasms. This report highlights the difficulty of achieving a diagnosis and subsequent treatment of such an uncommon disease...

While all childhood cancers are rare, tumors that are particularly infrequent or underrepresented within pediatrics are studied under the umbrella of the Children's Oncology Group Rare Tumor committee, divided into the Retinoblastoma and Infrequent Tumor subcommittees. The Infrequent Tumor subcommittee has traditionally included an emphasis on globally rare tumors such as adrenocortical carcinoma, nasopharyngeal carcinoma, or those tumors that are rare in young children, despite being common in adolescents and young adults, such as colorectal carcinoma, thyroid carcinoma, and melanoma...

No abstract text is available yet for this article.

Pancreatoblastoma is a rare malignant epithelial neoplasm of the pancreas. It primarily occurs in the pediatric population and is extremely uncommon in adults. A 64-year-old male patient with no known systemic disease presented to our clinic with abdominal pain and dyspeptic complaints. On physical examination, a tender epigastric mass was palpated. The patient was operated on with a preliminary diagnosis of gastrointestinal stromal tumor. Enbloc resection of the mass was performed. The transverse colon was segmentally resected with wedge resection of the gastric corpus...

KEY CLINICAL MESSAGE: The differential diagnoses of solid pseudopapillary neoplasm of the pancreas include cystic pancreatic neuroendocrine tumor, acinar cell carcinoma, and pancreatoblastoma. ABSTRACT: Solid pseudopapillary neoplasm (SPN) is a low-grade malignant pancreatic tumor which accounts for 0.9%-2.7% of all exocrine pancreatic neoplasms. It predominantly affects young females (90%) and less frequently occurs in male patients. Its prognosis after surgical resection remains excellent...

The most common tumour of the pancreas is ductal adenocarcinoma (PDAC). It remains one of the most lethal non-neuroendocrine solid tumours despite the use of a multi-approach strategy. Other, less-common neoplasms, which are responsible for 15% of pancreatic lesions, differ in treatment and prognosis. Due to the low incidence rate, there is a lack of information about the rarest pancreatic tumours. In this review, we described six rare pancreatic tumours: intraductal papillary mucinous neoplasm (IPMN), mucinous cystadenoma (MCN), serous cystic neoplasm (SCN), acinar cell carcinoma (ACC), solid pseudopapillary neoplasm (SPN) and pancreatoblastoma (PB)...

Pancreatoblastomas are rare pediatric tumors. In adults, they are exceedingly rare and seem to have a worse prognosis. Most are sporadic, though rare, cases occur in patients with familial adenomatous polyposis. Unlike pancreatic ductal adenocarcinomas, pancreatoblastomas are not believed to arise from dysplastic precursor lesions. Clinical history, along with endoscopic, pathological, and molecular findings, was reviewed for a 57-year-old male patient with an ampullary mass who presented with obstructive jaundice...

Acinar cell carcinoma (ACC) is a rare and highly malignant pancreatic tumor. Owing to histologic similarity, ACC is often difficult to distinguish from other solid medullary pancreatic tumors, particularly neuroendocrine neoplasm (NEN) and intraductal tubulopapillary neoplasm (ITPN). We aimed to identify new immunohistochemical markers commonly expressed in tumor cells with acinar cell differentiation and useful for both surgical and small biopsy specimens. Candidate molecules exclusively expressed in neoplastic or non-neoplastic acinar cells in pancreatic tissues with specific and available antibodies suitable for immunohistochemistry were selected...

A woman in her 80s was evaluated for a liver mass. She had a history of what was reported as pancreatic acinar cell carcinoma resected with a pancreatoduodenectomy 10 years prior at another institution. Liver biopsy showed metastatic pancreatoblastoma (PB) and staging imaging showed no evidence of additional metastatic disease. She underwent laparoscopic non-anatomic partial hepatectomy and recovered uneventfully. The liver pathology was reviewed along with slides from her initial pancreatoduodenectomy, and both were noted to be consistent with PB...

OBJECTIVE: This study mainly analysed the imaging data for seven cases of adult pancreatoblastoma (PB) and summarized additional imaging features of this disease based on a literature review, aiming to improve the understanding and diagnosis rate of this disease. MATERIALS AND METHODS: The imaging data for seven adult patients pathologically diagnosed with adult PB were retrospectively analysed. Among the seven patients, six underwent computed tomography (CT) scans, two patients underwent abdominal magnetic resonance imaging (MRI), and five patients underwent 18 F-FDG PET/CT...

We report the histopathological, immunohistochemical (IHC), and molecular findings in 3 patients with adult pancreatoblastoma, including 2 with autopsy features. The tumors were located in the tail and body of the pancreas, and the 2 autopsy examinations revealed liver and lung metastases. Histopathologically the neoplasms were composed of solid epithelial elements with nested or trabecular growth patterns, fibrous stroma, and squamoid clusters. Keratin 19 was positive mainly in squamoid corpuscles, and trypsin or chymotrypsin was positive in the acinar component...

Pancreatoblastoma (PB), a rare malignant epithelial neoplasm, is the most common pancreatic neoplasm of childhood. It is exceptionally rare in the adult population and its occurrence is limited to case reports. Although the neoplastic cells of PB can have a number of different directions of differentiation, PB is defined by the combination of neoplastic cells with acinar differentiation and squamoid morules. We report a case of a female patient in her seventies who presented with elevated creatinine level, concerning for a kidney disorder, and was found to have an abdominal mass on CT scan...

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...

No abstract text is available yet for this article.