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Cureus Oct 2023In 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,...
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. Biopsy and resultant histology are essential in diagnosis and surgical resection remains the preferred modality of treatment. However, the use of chemotherapy and its efficacy in adults remains unclear, and the prognosis documented in existing literature for adults is worse when compared to paediatric presentations. This case emphasises the need to consider pancreatoblastoma as a differential diagnosis when suspecting pancreatic or abdominal malignancies to achieve early detection and diagnosis, in order to provide optimal treatment and improve patient outcomes.
PubMed: 38046486
DOI: 10.7759/cureus.48063 -
EJC Paediatric Oncology Dec 2023While all childhood cancers are rare, tumors that are particularly infrequent or underrepresented within pediatrics are studied under the umbrella of the Children's...
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. Pleuropulmonary blastoma, gonadal stromal tumors, pancreatic tumors including pancreatoblastoma, gastrointestinal stromal tumor, nonmelanoma skin cancers, neuroendocrine tumors, and desmoplastic small round cell tumors, as well as other carcinomas are also included under the heading of the Children's Oncology Group Rare Tumor committee. While substantial challenges exist in rare cancers, inclusion and global collaboration remain key priorities to ensure high quality research to advance care.
PubMed: 37829670
DOI: 10.1016/j.ejcped.2023.100024 -
Asian Journal of Surgery Nov 2023
Topics: Humans; Child; Pancreatic Neoplasms
PubMed: 37290981
DOI: 10.1016/j.asjsur.2023.05.104 -
Clinical Case Reports May 2023The differential diagnoses of solid pseudopapillary neoplasm of the pancreas include cystic pancreatic neuroendocrine tumor, acinar cell carcinoma, and pancreatoblastoma.
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. Herein, we report a case of SPN in a male patient.
PubMed: 37220514
DOI: 10.1002/ccr3.7387 -
Cancers Apr 2023The 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... (Review)
Review
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). We distinguished their epidemiology, clinical and gross features, covered the newest reports about courses of treatment and systematised differential diagnoses. Although the most common pancreatic tumour, PDAC, has the highest malignant potential, it is still essential to properly classify and differentiate less-common lesions. It is vital to continue the search for new biomarkers, genetic mutations and the development of more specific biochemical tests for determining malignancy in rare pancreatic neoplasms.
PubMed: 37190144
DOI: 10.3390/cancers15082216 -
Journal of Indian Association of... 2022Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by...
Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by radiological suggestions and histopathology. The presence of metastatic disease and inoperable/incomplete excision remains the poor prognostic markers. We present a rare instance of an adolescent who has survived metastatic pancreatoblastoma after neoadjuvant chemoreduction/complete surgical excision.
PubMed: 36714488
DOI: 10.4103/jiaps.jiaps_4_22 -
BMC Medical Imaging Dec 2022This study mainly analysed the imaging data for seven cases of adult pancreatoblastoma (PB) and summarized additional imaging features of this disease based on a... (Review)
Review
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 F-FDG PET/CT.
RESULTS
The tumours were located in the head of the pancreas in three cases, in the tail of the pancreas in two cases, and in the gastric antrum and neck of the pancreas in one case. Six tumours showed blurred edges, and an incomplete envelope was observed in only two cases when enhanced, which showed extruded growth and cyst-solid masses; one tumour was a solid mass with ossification. Showing mild or significant enhancement in the arterial phase (AP) for six cases. In the MRI sequence, isointensity was found on suppressed T1-weighted imaging, and hyperintensity was noted on suppressed T2-weighted imaging in two cases, with significant enhancement. Pancreatic duct dilatation was found in four cases. Tumour F-FDG PET/CT imaging exhibited high uptake in five cases.
CONCLUSION
Adult PB involves a single tumour and commonly manifests as cystic-solid masses with blurred edges. Capsules are rare, ossification is an important feature, tumours can also present in ectopic pancreatic tissues, with mild or strengthening in the AP, and F-FDG uptake is high. These features are relatively specific characteristics in adult PB.
Topics: Humans; Adult; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Retrospective Studies; Positron-Emission Tomography; Tomography, X-Ray Computed; Pancreatic Neoplasms; Magnetic Resonance Imaging; Radiopharmaceuticals
PubMed: 36581821
DOI: 10.1186/s12880-022-00958-4 -
Frontiers in Pediatrics 2022Pancreatic tumors in children are infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often... (Review)
Review
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.
PubMed: 36507125
DOI: 10.3389/fped.2022.966943 -
World Journal of Pediatric Surgery 2022
PubMed: 36475046
DOI: 10.1136/wjps-2021-000398 -
Pediatric Blood & Cancer Jun 2023Primary 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...
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.
Topics: Child; Humans; Surface Plasmon Resonance; Pancreatic Neoplasms; Tomography, X-Ray Computed; Carcinoma, Papillary; Pancreas
PubMed: 36215203
DOI: 10.1002/pbc.29975