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Annales de Pathologie Nov 2022
Topics: Humans; Pancreatic Neoplasms
PubMed: 35701283
DOI: 10.1016/j.annpat.2022.04.002 -
Pediatric Blood & Cancer Apr 2023
Topics: Humans; Pancreatic Neoplasms; Guanine Nucleotide Exchange Factors
PubMed: 36519595
DOI: 10.1002/pbc.30155 -
Journal of Radiology Case Reports Aug 2016Most cases of pancreatoblastoma, a rare malignant epithelial tumor of the pancreas, are seen in the pediatric population. The rarity of pancreatoblastoma, the similar... (Review)
Review
Most cases of pancreatoblastoma, a rare malignant epithelial tumor of the pancreas, are seen in the pediatric population. The rarity of pancreatoblastoma, the similar radiologic findings to those seen in other pancreatic lesions, and its histopathologic heterogeneity, make its preoperative diagnosis in adults a real challenge. We report ultrasound, computed tomography and magnetic resonance imaging correlative findings of a histologically proven pancreatoblastoma in a 37-year-old woman. Pancreatoblastoma should be considered in the differential diagnosis of a pancreatic mass presenting uncommon imaging features.
Topics: Adult; Biopsy; Diagnosis, Differential; Fatal Outcome; Female; Humans; Magnetic Resonance Imaging; Pancreatic Neoplasms; Tomography, X-Ray Computed; Ultrasonography
PubMed: 27761191
DOI: 10.3941/jrcr.v10i8.2737 -
Zhonghua Yi Xue Za Zhi = Chinese... Sep 1994Primary neoplasms of the pancreas are rare in children. One variant of these tumors is pancreatoblastoma, comprising 0.5% of epithelial tumors of the pancreas. It... (Review)
Review
Primary neoplasms of the pancreas are rare in children. One variant of these tumors is pancreatoblastoma, comprising 0.5% of epithelial tumors of the pancreas. It usually affects children at 1-8 years of age, with quite equal sex ratio. The tumor can be found at any site of pancreas, but it most commonly arises in the head of pancreas. Histopathologically, pancreatoblastoma is an encapsulated tumor with distinct organoid structures and sometimes squamoid corpuscles. Acinar cells with zymogen granules are occasionally found. The tumor has favorable prognosis. We present a 14-year-old female who was admitted due to prolonged jaundice for about 2 months. Physical examination revealed pale conjunctiva, yellowish skin color and hepatosplenomegaly. Abdominal sonography and CT scan showed dilated common bile duct and a tumor mass about 2 x 2 cm in dimension located a pancreatic head. She received surgical operation to relieve obstructive jaundice and later Whipple's operation for radical resection. Six months after operation, the patient received the examination of abdominal sonography, CT scan and gallium tumor scan but there was no evidence of local recurrence or distant metastasis. Now the patient is living well for more than one year. Because of its rare occurrence, we demonstrate this case and review the literature.
Topics: Adolescent; Female; Humans; Pancreatic Neoplasms
PubMed: 7954063
DOI: No ID Found -
World Journal of Pediatric Surgery 2022
PubMed: 36475046
DOI: 10.1136/wjps-2021-000398 -
The Journal of International Medical... Aug 2021Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of...
Pancreatoblastoma (PB) is a rare epithelial malignancy usually occurring in the paediatric population. Adult PB is rare, and its imaging findings are similar to those of other tumours, making preoperative diagnosis a considerable challenge. We report correlative ultrasound, contrast-enhanced ultrasonography, contrast-enhanced magnetic resonance imaging, and positron emission tomography-computed tomography findings in a 60-year-old woman with PB. PB often presents with uncommon imaging features and should be considered in the differential diagnosis of pancreatic masses. It is important for clinicians to be aware of these differences to provide effective treatment.
Topics: Adult; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Pancreatic Neoplasms; Ultrasonography
PubMed: 34461770
DOI: 10.1177/03000605211039565 -
Translational Oncology Apr 2022The aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or...
PURPOSE
The aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or metastatic disease.
METHODS
Four cases treated in our institution and 59 cases reported previously in the literature from the PubMed biomedical database (2000-2020) were reviewed and analyzed.
RESULTS
Four cases with PB presented with abdominal pain and palpable abdominal masses, with the tumor size ranging from 5.2 to 18 cm in diameter. The invasion of the splenic vein and superior mesenteric artery, duodenum, and lymph nodes were risk factors for PB. Three cases were treated with combination therapy and showed favorable outcomes, while one case was treated with chemotherapy alone due to tumor progression and died of the disease. Squamous corpuscles were revealed in the tumor samples and considered a defining component for histological diagnosis.
CONCLUSIONS
Multidisciplinary diagnosis plays an important role in clinical management. The risk factors should be considered in the therapeutic stratification of PB before surgery.
PubMed: 35180620
DOI: 10.1016/j.tranon.2022.101359 -
Archives of Pathology & Laboratory... Nov 2003Pancreatoblastoma, generally regarded as a pediatric malignant tumor, is rarely found in the adult population. Only 13 adults with pancreatoblastoma, ranging in age from... (Review)
Review
Pancreatoblastoma, generally regarded as a pediatric malignant tumor, is rarely found in the adult population. Only 13 adults with pancreatoblastoma, ranging in age from 19 to 68 years, have previously been reported in the world literature. A diagnosis of pancreatoblastoma relies on characteristic histologic features, including epithelial differentiation and, more importantly, squamoid differentiation. Despite aggressive therapy, adults with pancreatoblastoma have a poor outcome. We describe a 78-year-old woman who presented with painless jaundice and was found on abdominal computed tomographic scan to have a 2.7-cm ampullary mass. The patient underwent successful pancreaticoduodenectomy. Pathologic examination of the resected tumor revealed findings characteristic of pancreatoblastoma. The tumor formed acinar and glandular structures, solid areas, and contained many "squamoid corpuscles," a defining feature of pancreatoblastoma. The tumor cells also showed acinar and ductal phenotype by immunohistochemistry. To the best of our knowledge, this case represents the oldest patient with pancreatoblastoma to be described in the literature to date and the first to occur in the ampulla of Vater. We review previously published cases and discuss the clinical and histopathologic features of adult pancreatoblastoma.
Topics: Aged; Ampulla of Vater; Female; Humans; Pancreatic Neoplasms
PubMed: 14567752
DOI: 10.5858/2003-127-1501-APIAEP -
Genes Dec 2023Adult pancreatoblastoma (PBL) is a rare pancreatic malignancy, with recent evidence suggesting a possible link to familial adenomatous polyposis (FAP). This study aims... (Review)
Review
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. We identified eight FAP-related PBL cases, with a median age of 40 (IQR: 34-50). Of these, seven (87%) occurred in adults. We found 65 cases of adult PBL not FAP-related; thus, 7 out of 65 cases (10.7%) of adult PBL reported in the literature are associated with a clinical diagnosis of FAP or were carriers of germline pathogenic variants (GPVs).
CONCLUSION
Data suggest a non-random association between adult PBL and FAP. Further research is essential to optimise surveillance protocols and develop more effective treatment strategies.
Topics: Adult; Humans; Adenomatous Polyposis Coli; Germ-Line Mutation; Pancreatic Neoplasms
PubMed: 38254934
DOI: 10.3390/genes15010044 -
Pediatric Blood & Cancer Jun 2021Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by...
Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network - European Registry) project.
Topics: Chemotherapy, Adjuvant; Child; Child, Preschool; Humans; Neoadjuvant Therapy; Pancreatectomy; Pancreatic Neoplasms; Rare Diseases
PubMed: 34174157
DOI: 10.1002/pbc.29112