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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 -
Virchows Archiv : An International... Jan 2019CD200 has been recently indicated as a robust marker of well-differentiated neuroendocrine neoplasms. Here, we evaluate its role in differential diagnosis of solid...
CD200 has been recently indicated as a robust marker of well-differentiated neuroendocrine neoplasms. Here, we evaluate its role in differential diagnosis of solid pancreatic neoplasms. We immunostained for CD200 22 solid pseudopapillary neoplasms (SPNs), 8 acinar carcinomas (ACs), 2 pancreatoblastomas (PBs), 138 neuroendocrine tumors (PanNETs), and 48 ductal adenocarcinomas. All SPNs showed strong cytoplasmic and membranous staining for CD200, while only one case of AC had focal positivity. The two PBs showed focal CD200 positivity, mainly located in squamoid nests. The vast majority of PanNETs (96%) showed strong cytoplasmic and membranous staining for CD200, whereas all PDACs were negative. As both PanNETs and SPNs express CD200, it has no role in the differential diagnosis between these two entities.
Topics: Antigens, CD; Biomarkers, Tumor; Carcinoma, Acinar Cell; Carcinoma, Pancreatic Ductal; Carcinoma, Papillary; Diagnosis, Differential; Humans; Immunohistochemistry; Neuroendocrine Tumors; Pancreatic Neoplasms; Predictive Value of Tests
PubMed: 30132130
DOI: 10.1007/s00428-018-2437-7 -
Clinics in Laboratory Medicine Jun 2018Pancreatic neoplasms, including ductal adenocarcinoma, solid pseudopapillary neoplasm, pancreatic endocrine neoplasms, acinar cell carcinoma, and pancreatoblastoma, are... (Review)
Review
Pancreatic neoplasms, including ductal adenocarcinoma, solid pseudopapillary neoplasm, pancreatic endocrine neoplasms, acinar cell carcinoma, and pancreatoblastoma, are associated with different genetic abnormalities. Hepatic adenomas with beta-catenin exon 3 mutation are associated with a high risk of malignancy. Hepatic adenoma with arginosuccinate synthetase 1 expression or sonic hedgehog mutations are associated with a risk of bleeding. Hepatocellular carcinoma and choangiocarcinoma display heterogeneity at both morphologic and molecular levels Cholangiocellular carcinoma is most commonly associated with IDH 1/2 mutations.
Topics: Digestive System Neoplasms; Humans; Molecular Diagnostic Techniques
PubMed: 29776636
DOI: 10.1016/j.cll.2018.03.003 -
Clinical Imaging 2018Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease. (Review)
Review
PURPOSE
Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease.
MATERIALS AND METHODS
The demographic, clinical, and imaging findings of 41 patients with pathologically proven pancreatoblastoma from 1986 to 2017 identified in PubMed were reviewed. The key words used for searching PubMed were: "pancreatoblastoma", "pancreatic tumor", and "adult pancreatoblastoma." We also reported the details of a case of adult pancreatoblastoma treated at our institution.
RESULTS
We identified 41 cases of adult pancreatoblastomas, and the mean age at diagnosis was 41.4 ± 17.4 years. Pancreatoblastomas occurred in the pancreatic head in 48.4% of patients, and in 39.0% of cases the tumor was >8 cm in diameter at diagnosis. Patient age and tumor size were similar between males and females (P = 0.59; P = 0.32, respectively). Metastases was present in 17 of the 41 adult patients (41.5%). No significant difference in age, sex, tumor size, and tumor location was found between patients with and without metastases (P = 0.57, 0.58, 0.64, 0.39, respectively).
CONCLUSION
Preoperative diagnosis of adult pancreatoblastoma is difficult because of the heterogeneous, variable cellular differentiation and atypical clinical and imaging features. A pancreatoblastoma should be considered when tumors in the pancreas are solid and cystic.
Topics: Adult; Fatal Outcome; Female; Humans; Male; Middle Aged; Pancreas; Pancreatic Neoplasms; Young Adult
PubMed: 29753278
DOI: 10.1016/j.clinimag.2018.05.001 -
Pediatric Blood & Cancer Oct 2018Primary pancreatic carcinoma and pancreatic metastases are rare in the pediatric population. Pancreatoblastoma is the most common pancreatic malignant tumor in young...
PURPOSE
Primary pancreatic carcinoma and pancreatic metastases are rare in the pediatric population. Pancreatoblastoma is the most common pancreatic malignant tumor in young children and solid-pseudopapillary tumor in teenagers. Pancreatic adenocarcinoma is extremely rare under the age of 40 and is usually associated with underlying genetic abnormalities. Secondary malignancies of the pancreas occur more frequently than primary pancreatic malignancies in children and are most commonly seen with non-Hodgkin lymphomas (NHL) and mesenchymal sarcomas. The purpose of this study was to characterize the metabolism of primary and secondary tumors of the pancreas in pediatric patients.
MATERIALS AND METHODS
A retrospective analysis of all primary and secondary pancreatic malignancies imaged with 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) computed tomography (CT) was conducted.
RESULTS
Three patients with primary pancreatic cancers were identified, one each with pancreatoblastoma, solid-pseudopapillary tumor, and adenocarcinoma. Each tumor showed elevated uptake of FDG. Metastatic disease in the pancreas was identified in 12 patients-five NHL (including three Burkitt lymphomas), six sarcomas (three osteosarcomas, two rhabdomyosarcomas, and one Ewing sarcoma family tumor), and one malignant rhabdoid tumor. Elevated but variable uptake of FDG was found in each of the tumors of patients with metastatic disease within the pancreas.
CONCLUSION
Both primary malignancies and metastatic disease within the pancreas, though very rare in children, adolescents, and young adults, are metabolically active and can be functionally characterized using FDG-PET CT.
Topics: Adolescent; Child; Female; Fluorodeoxyglucose F18; Humans; Infant; Male; Pancreatic Neoplasms; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Retrospective Studies; Young Adult
PubMed: 29750397
DOI: 10.1002/pbc.27115 -
Pediatric Blood & Cancer Oct 2018A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor...
A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor (SPT) (61.3%). Pancreaticoduodenectomy was the most commonly performed operation (48%). Neoadjuvant chemo/radiotherapy was utilized in 3.9, 36.2, 25, and 27.8% of patients with SPTs, pancreatoblastomas (PBLs), neuroendocrine tumors, and exocrine carcinomas, respectively. Adjuvant chemotherapy (75.6%) and radiation (34%) were most commonly utilized in PBLs. All-cause mortality was highest in exocrine carcinomas (50%). Overall, 98.8% of patients with SPTs survived. PBL exhibited the highest recurrence rate (14.7%) within a mean of 23.5 months.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Pancreatic Neoplasms; Young Adult
PubMed: 29697193
DOI: 10.1002/pbc.27114 -
Oncotarget Feb 2018During next generation sequencing (NGS) analysis, many missense mutations were found in a well-known oncogene, many of which were variant of uncertain significance...
During next generation sequencing (NGS) analysis, many missense mutations were found in a well-known oncogene, many of which were variant of uncertain significance mutations. We recently treated an adult patient with pancreatoblastoma by chemotherapy. Using an NGS cancer panel, we found a previously unreported missense mutation in the 1835 codon of the () gene. We also found a heterogeneous mutation in the 1835 codon of the gene in the patient's germline by Sanger sequencing. Although this patient did not have a history of familial adenomatous polyposis, functional analysis suggested the R1835G mutant showed attenuated repression of Wnt/β-catenin signaling activity. This is the first report showing a novel missense mutation involved in the onset of adult pancreatoblastoma.
PubMed: 29535845
DOI: 10.18632/oncotarget.24017 -
Chinese Clinical Oncology Dec 2017As the most common and most important cancer of the pancreas, with rapid mortality and now also as the third leading cause of cancer-related deaths in the United States,... (Review)
Review
As the most common and most important cancer of the pancreas, with rapid mortality and now also as the third leading cause of cancer-related deaths in the United States, pancreatic ductal adenocarcinoma (PDAC) has become synonymous with "pancreas cancer". PDAC is also the prototype of the "pancreatobiliary-type" adenocarcinomas, along the biliary tract, ampullary and gallbladder cancers with the similar morphology and behavior. Recent molecular profiling studies have identified distinct subsets of PDAC, potentially with different behaviors and targetability. Moreover, while PDAC is by far the most common cancer of the pancreas, there are various other types that occur in this organ and are erroneously classified together with PDAC. Many of these have different molecular and biologic characteristics that warrant their management separately although they are also technically "pancreatic cancers". While some are closely related to PDAC and have as aggressive behavior (such as adenosquamous carcinomas which are recently recognized under "basal" like category in profiling studies, which are actually even worse prognostically than PDACs), in the meantime, others such as colloid carcinoma has a much better behavior than PDAC, and as a carcinoma with intestinal lineage (MUC2/CDX2) colloid carcinoma may require an entirely different treatment approach as well. Similarly, medullary carcinomas also appear to have different biology. Additionally, non-ductal cancers such as acinar, neuroendocrine, solid-pseudopapillary neoplasms and pancreatoblastoma have their respective clinicopathologic and molecular associations and warrant careful elimination in the management and study protocols. Another very problematic aspect in the classification of "pancreas cancer" is its delineation from the cancers of neighboring organs, in particular, ampullary/duodenal and common bile duct (CBD) cancers, for which recently more refined criteria have been provided. Additionally, the possibility of metastasis from another site and lymphomas also need to be considered. In summary, there is a whole host of cancers that occur in the pancreas that ought to be considered carefully before a case is classified as an ordinary "pancreas cancer" (PDAC).
Topics: Humans; Male; Pancreatic Neoplasms
PubMed: 29307199
DOI: 10.21037/cco.2017.12.01 -
Clinical Journal of Gastroenterology Apr 2018The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin...
The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis. The patient underwent an endoscopic ultrasound (EUS) with fine needle aspiration (FNA) from the pancreatic mass and liver metastasis with cytological evaluation consistent with a pancreatoblastoma, later confirmed through a percutaneous US-guided liver biopsy. During the inpatient period, liver function deteriorated and acute kidney injury developed. Severe progressive cachexia was observed. The patient was discharged on renal replacement therapy and palliative care. Death occurred 3 months after diagnosis. Pancreatoblastoma is an uncommon pancreatic malignant epithelial cancer of the pancreas, typically occurring in the paediatric population. Adult pancreatoblastoma is extremely rare, with about 40 cases reported in the literature and generally presenting a more aggressive biologic and clinical behaviour. Surgical resection is the treatment of choice, but most cases are detected in advanced stages. This case underlines the ability to establish a pancreatoblastoma cytology-based diagnosis with EUS-FNA, and confirms the associated poor outcome.
Topics: Adult; Biopsy, Fine-Needle; Endosonography; Fatal Outcome; Humans; Liver Neoplasms; Magnetic Resonance Imaging; Male; Pancreatic Neoplasms; Positron Emission Tomography Computed Tomography
PubMed: 29285688
DOI: 10.1007/s12328-017-0812-6 -
Pancreatology : Official Journal of the... Mar 2018There are very few data in the current literature regarding the short- and long-term outcome of surgery for pediatric pancreatic tumors (PPT). No data are available on...
BACKGROUND
There are very few data in the current literature regarding the short- and long-term outcome of surgery for pediatric pancreatic tumors (PPT). No data are available on the impact of pancreatic surgery on the children's growth.
METHODS
This is a retrospective cohort study on a consecutive series of pediatric/adolescent patients who underwent pediatric surgery at Karolinska University Hospital from January 2005 to July 2017.
RESULTS
Overall 14 pancreatic operations were performed in 13 patients. The median age was 11.4 years (range 3-15). Six pancreaticoduodenectomies (42.8%), 5 distal pancreatectomies (35.7%), and 3 enucleations (21.5%) were performed. The final histology revealed a solid pseudopapillary tumor in 9 cases (69.2%), neuroblastoma in 1 (7.7%), ganglioneuroma in 1 (7.7%), pancreatoblastoma in 1 (7.7%), and insulinoma in 1 (7.7%). Overall, 3 patients developed post-operative complications (23%). There was no peri-operative mortality. All patients are alive after a median follow-up time of 80 months. Exocrine insufficiency was detected post-operatively in 4 patients (30.7%) Endocrine insufficiency requiring insulin treatment developed in one patient (7.7%). No significant impact on growth was detected in any of the patients after pancreatic resection.
CONCLUSIONS
In our series, surgery performed for PPTs seems to be safe and effective. The effect of pancreatic surgery on children's growth does not seem to be significant.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Male; Pancreatectomy; Pancreatic Neoplasms; Pancreaticoduodenectomy; Postoperative Complications; Treatment Outcome
PubMed: 29277262
DOI: 10.1016/j.pan.2017.12.009