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Radiology Case Reports 2009The case of a six-month-old child with pancreatoblastoma is presented. Pancreatoblastoma is a rare primary neoplasm of childhood, usually presenting in children ranging...
The case of a six-month-old child with pancreatoblastoma is presented. Pancreatoblastoma is a rare primary neoplasm of childhood, usually presenting in children ranging from 2 -8 years old. The radiologic findings vary from solid to complex masses. This case is unusual due to the young age of the patient.
PubMed: 27307808
DOI: 10.2484/rcr.v4i2.284 -
Modern Pathology : An Official Journal... Apr 2020Pancreatic acinar cell carcinoma is relatively rare (1 to 2% of pancreatic malignancies) but may be under-recognized. In contrast to pancreatic ductal adenocarcinoma,...
Pancreatic acinar cell carcinoma is relatively rare (1 to 2% of pancreatic malignancies) but may be under-recognized. In contrast to pancreatic ductal adenocarcinoma, most acinar cell carcinomas lack mutations in KRAS, DPC, CDKN2A or TP53, but appear to have a high incidence of gene rearrangements, with up to 20% reported to be driven by BRAF fusions. With the development of a new class of RET-specific tyrosine kinase inhibitors, which appear to have particularly strong activity against RET gene rearranged tumours, there is now considerable interest in identifying RET gene rearrangements across a wide range of cancers. RET rearrangements have been reported to occur at a very low incidence (<1%) in all pancreatic carcinomas. We postulated that given its unique molecular profile, RET gene rearrangements may be common in acinar cell carcinomas. We performed fluorescent in-situ hybridization (FISH) studies on a cohort of 40 acinar cell spectrum tumours comprising 36 pure acinar cell carcinomas, three pancreatoblastomas and one mixed acinar-pancreatic neuroendocrine tumour. RET gene rearrangements were identified in 3 (7.5%) cases and BRAF gene rearrangements in 5 (12.5%). All gene rearranged tumours were pure acinar cell carcinomas. Our findings indicate that amongst all pancreatic carcinomas, acinar carcinomas are highly enriched for potentially actionable gene rearrangements in RET or BRAF. FISH testing is inexpensive and readily available in the routine clinical setting and may have a role in the assessment of all acinar cell carcinomas-at this stage to recruit patients for clinical trials of new targeted therapies, but perhaps in the near future as part of routine care.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Carcinoma, Acinar Cell; Databases, Factual; Europe; Female; Gene Rearrangement; Genetic Predisposition to Disease; Humans; In Situ Hybridization, Fluorescence; Male; Middle Aged; Pancreatic Neoplasms; Proto-Oncogene Proteins B-raf; Proto-Oncogene Proteins c-ret; Young Adult
PubMed: 31558784
DOI: 10.1038/s41379-019-0373-y -
Frontiers in Oncology 2021Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy...
Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy (FOLFIRINOX regimen) was associated with a disease control in one case and a partial response in the second with an improvement of general status for both. A high-throughput sequencing of the tumor described in both cases alteration in the Wnt/β-catenin pathway: a mutation in (exon 3, c.110C>G, p.S37C, reported as a hotspot in COSMIC) in one case and a homozygous loss associated with breakage targeting (5q22.2) in the second.
PubMed: 33796447
DOI: 10.3389/fonc.2021.564506 -
Ecancermedicalscience 2018Pancreatoblastoma is a rare paediatric malignant neoplasm. The treatment of choice is complete surgical resection. However, it is often unresectable due to its large...
Pancreatoblastoma is a rare paediatric malignant neoplasm. The treatment of choice is complete surgical resection. However, it is often unresectable due to its large size, local infiltration or distant metastasis. Since the condition is rare, there is currently no standard treatment regimen. We outline the case of a 4-year-old child who presented with abdominal pain and distention, together with an enlarged liver and elevated serum α-fetoprotein levels. Imaging studies showed the presence of an abnormal pancreatic tumour and multiple nodular lesions in the liver, the biopsies from which led to a diagnosis of pancreatoblastoma. In this case, the patient received cycles of neoadjuvant chemotherapy, combining cisplatin and doxorubicin. The patient subsequently underwent scheduled surgery in which the primary pancreatic lesion was resected, obtaining a circumscribed and nodular specimen measuring 7 × 6 cm and weighing 150 g. Given the extent of the metastasis, the child is currently awaiting a liver transplant.
PubMed: 30174723
DOI: 10.3332/ecancer.2018.861 -
Journal of Korean Medical Science Jun 2011Pancreatoblastoma is a rare primary pancreatic neoplasm of children that may arise in any portion of the pancreas. We report a case of a 3-yr-old boy who presented to...
Pancreatoblastoma is a rare primary pancreatic neoplasm of children that may arise in any portion of the pancreas. We report a case of a 3-yr-old boy who presented to with abdominal pain our hospital and a progressive bulge in his right abdomen. Biochemical evaluation and serum levels of tumoral markers were within reference limits. On the computed tomography, two tumors were found. One located in the head of the pancreas; however, a laparotomy revealed that the head of pancreas was compressed but normal. The other was in the left abdomen near the spleen and the tail of the pancreas. The diagnosis of two synchronous pancreatoblastoma originating from the omentum was confirmed by pathology. Therefore, a pancreatoblastoma should be considered when a large well-defined, lobulated, and heterogeneous mass is identified in the pancreas of children. In addition, an ectopic pancreatoblastoma should be considered when identified within or near the ectopic pancreatic tissue.
Topics: Antineoplastic Agents; Child, Preschool; Drug Therapy, Combination; Humans; Laparotomy; Male; Pancreatic Neoplasms; Peritoneal Neoplasms; Tomography, X-Ray Computed
PubMed: 21655073
DOI: 10.3346/jkms.2011.26.6.832 -
BMJ Case Reports 2009Immature teratoma of the stomach in the neonate is extremely rare.1(,)2 This report outlines a case of giant immature teratoma of the stomach, which was detected by...
Immature teratoma of the stomach in the neonate is extremely rare.1(,)2 This report outlines a case of giant immature teratoma of the stomach, which was detected by prenatal ultrasonography in the third trimester as an echogenic mass contiguous with the stomach bubble. It increased from 4.5 cm in diameter to 7 cm between 34 and 37 weeks gestation. The baby was delivered by elective caesarean section at 37 weeks gestation. Neonatal imaging highlighted a differential diagnosis of nephroblastoma, neuroblastoma, pancreatoblastoma and teratoma. The infant underwent surgical excision of the abdominal mass on the 10th day of life. Histology revealed grade III immature gastric teratoma arising from the posterior wall of stomach, outlining the unknown implications of such a designation in an extraovarian site. The infant made a good postoperative recovery and is currently well 9 months later, without adjuvant therapy, and with no evidence of recurrent disease.
PubMed: 21686482
DOI: 10.1136/bcr.10.2008.1050 -
Annals of Hepato-biliary-pancreatic... Feb 2020Pancreatoblastoma is a malignant exocrine pancreatic tumor that is usually present in childhood. We herein present one case of pediatric living donor liver...
Pancreatoblastoma is a malignant exocrine pancreatic tumor that is usually present in childhood. We herein present one case of pediatric living donor liver transplantation (LDLT) combined with spleen-preserving regional total pancreatectomy and portal vein homograft interposition in a 4-year-old boy with advanced pancreatoblastoma invading the portal and superior mesenteric veins. The size of the pancreatoblastoma was gradually reduced along systemic chemotherapy, thus we decided to perform surgery to remove it completely. A cold-stored fresh iliac vein homograft was prepared. Initially, a spleen-preserving distal pancreatectomy was performed. Thereafter, a completion regional total pancreatectomy was performed under superior mesenteric vein-vena cava bypass. A left liver graft from his mother was implanted according to the standardized procedures with portal vein interposition. This patient recovered uneventfully and is currently undergoing scheduled adjuvant chemotherapy. To our knowledge, this is the world-second case of pediatric LDLT for advanced pancreatoblastoma. Availability of fresh vein homografts is helpful to expand the indication of pediatric LDLT.
PubMed: 32181434
DOI: 10.14701/ahbps.2020.24.1.78 -
BMC Gastroenterology Mar 2024Pancreatic 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...
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. Cox proportional-hazards regression was used to determine the variables related to OS.
RESULTS
We 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.
CONCLUSIONS
Pancreatic 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.
Topics: Humans; Child; Female; Adolescent; Prognosis; Survival Analysis; Kaplan-Meier Estimate; Pancreatic Neoplasms
PubMed: 38486208
DOI: 10.1186/s12876-024-03194-y -
Modern Pathology : An Official Journal... Jun 2005Solid pseudopapillary tumor, pancreatoblastoma, undifferentiated carcinoma with osteoclastic-like giant cells, and acinar cell carcinomas are rare pancreatic nonductal... (Comparative Study)
Comparative Study
Solid pseudopapillary tumor, pancreatoblastoma, undifferentiated carcinoma with osteoclastic-like giant cells, and acinar cell carcinomas are rare pancreatic nonductal neoplasms. Compared to the significant advances in our understanding of the pathogenesis of pancreatic ductal adenocarcinomas in the last decades, the molecular mechanisms underlying pancreatic nonductal neoplasms are poorly understood. In order to elucidate their molecular pathogenesis, we constructed tissue microarrays to study the expression of some novel pancreatic ductal adenocarcinoma-associated tumor markers in these nonductal pancreatic neoplasms. We analyzed nine markers including tumor suppressor gene (14-3-3 sigma), proliferation marker (topoisomerase II alpha), epithelial markers (prostate stem cell antigen, mesothelin and cytokeratin 19), stromal markers (fascin, hsp47 and fibronectin), and gamma-synuclein whose function is not delineated. In addition, we included tumor suppressor gene DPC4 and oncogene Beta-catenin to further confirm their expression in pancreatic nonductal tumors. Our results showed that in contrast to pancreatic ductal adenocarcinomas that show loss of Dpc4 protein in 55% of cases, loss of Dpc4 expression is absent in pancreatic nonductal neoplasms. Expression of 14-3-3 sigma is frequently seen in both pancreatic nonductal neoplasms (25-100%) and ductal adenocarcinomas (89%). Aberrant nuclear expression of beta-catenin is common in pancreatic nonductal neoplasms, specifically in solid pseudopapillary tumors (88%) and pancreatoblastomas (100%) but is rarely seen in pancreatic ductal adenocarcinomas (<5%). Expression of topoisomerase II alpha is not seen in solid pseudopapillary tumors and undifferentiated carcinomas with osteoclastic-like giant cells but is focally seen in pancreatoblastomas (50%) and acinar cell carcinomas (85%). Expression of PSCA and mesothelin was observed in pancreatic nonductal neoplasms but their expression was seen less frequently (0-50%) and weaker than that in pancreatic ductal adenocarcinomas (60-100%). CK19, a marker of pancreatic ductal adenocarcinomas, is not expressed in pancreatic nonductal neoplasms. Expression of gamma-synuclein as well as stromal markers (fascin, hsp47 and fibronectin) is frequently seen in both. Our findings indicate pancreatic nonductal neoplasms have distinctive patterns of protein expression relative to pancreatic ductal adenocarcinomas and suggest that pancreatic nonductal neoplasms have different genetic pathways from the more common pancreatic ductal adenocarcinomas.
Topics: 14-3-3 Proteins; Antigens, Neoplasm; Biomarkers, Tumor; Carcinoma, Acinar Cell; Carcinoma, Pancreatic Ductal; Carrier Proteins; Cytoskeletal Proteins; DNA-Binding Proteins; Exonucleases; Exoribonucleases; Fibronectins; GPI-Linked Proteins; HSP47 Heat-Shock Proteins; Heat-Shock Proteins; Humans; Immunohistochemistry; Keratins; Membrane Glycoproteins; Mesothelin; Microfilament Proteins; Neoplasm Proteins; Nerve Tissue Proteins; Pancreatic Neoplasms; Serpins; Smad4 Protein; Synucleins; Trans-Activators; beta Catenin; gamma-Synuclein
PubMed: 15696124
DOI: 10.1038/modpathol.3800363 -
International Journal of Surgery Case... 2013Pancreatic tumor is a rare condition in children, but reasonably common in adults. Histopathology in children also differs from that in adults, with most cases being...
INTRODUCTION
Pancreatic tumor is a rare condition in children, but reasonably common in adults. Histopathology in children also differs from that in adults, with most cases being pancreatoblastoma, solid pseudopapillary tumor, or pancreatic endocrine tumors.
PRESENTATION OF CASE
A 14-month-old boy was noticed abdominal distension and referred to our hospital. Laboratory findings revealed leukocytosis and elevation of serum level of C-reactive protein and pancreatic enzymes. Radiological findings at admission were the huge abdominal cyst in abdominal computed tomography. As the levels of pancreatic enzyme elevated synchronous to oral feeding, total parenteral nutrition was needed. Besides radiographically the abdominal lesion changed from multiple large cystic type to multiple microcystic lesion including solid component over time. It was considered different diagnosis was pancreatic blastoma, pancreatic pseudo cyst, and lymphangioma and he was performed operation. The huge multicystic and partially solid tumor arising from the tail of pancreas existed from posterior of stomach to pelvic cavity. The tumor was completely resected without pancreatectomy and residual pancreas can be kept without tumor invasion. Histopathological finding was pancreatic hamartoma.
DISCUSSION
Pancreatic hamartoma was extremely rare, and only 17 cases were previously reported in the literature. This is the first case that the change of radiographic findings overtime was shown. We reviewed 17 cases (4 cases in children) of pancreatic hamartoma including our case.
CONCLUSION
Although extremely rare, pancreatic hamartoma should be considered in the differential diagnosis of cystic abdominal mass in children.
PubMed: 23143293
DOI: 10.1016/j.ijscr.2012.10.001