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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 -
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 -
World Journal of Gastroenterology Dec 2017Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC),... (Review)
Review
Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. Reports of overall survival range from 18 to 47 mo. A literature review on PACCs included comprehensive genomic profiling and whole exome sequencing on a series of more than 70 patients as well as other diagnostic studies including immunohistochemistry. Surgical resection of PACC is the preferred treatment for localized and resectable tumors. The efficacy of adjuvant treatment is unclear. Metastatic PACCs are generally not curable and treated with systemic chemotherapy. They are moderately responsive to chemotherapy with different regimens showing various degrees of response in case reports/series. Most of these regimens were developed to treat patients with pancreatic ductal adenocarcinomas or colorectal adenocarcinomas. Review of PACC's molecular profiling showed a number of gene alterations such as: , , , , , , , , , and DNA mismatch repair abnormalities. PACCs had multiple somatic mutations with some targetable with available drugs. Therefore, molecular profiling of PACC should be an option for patients with refractory PACC.
Topics: Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Carcinoma, Acinar Cell; Gene Expression Profiling; Humans; Immunohistochemistry; Mutation; Pancreas; Pancreatectomy; Pancreatic Neoplasms; Prognosis; Signal Transduction; Survival Rate; Exome Sequencing
PubMed: 29259370
DOI: 10.3748/wjg.v23.i45.7945 -
Oncology Letters Oct 2017Pancreatic cancer is classified as ductal, acinar, neuroendocrine carcinoma or pancreatoblastoma. Ductal and acinar cells derive from exocrine glands and neuroendocrine...
Pancreatic cancer is classified as ductal, acinar, neuroendocrine carcinoma or pancreatoblastoma. Ductal and acinar cells derive from exocrine glands and neuroendocrine cells from endocrine glands; however, mixed acinar-neuroendocrine-ductal carcinoma has different histological carcinomas coexisting within a nodule. The mixed pancreatic carcinoma forms from different developmental origins and therefore requires investigation. The current case report presents a 50-year-old male who had a tumor within the body of the pancreas. Pathological examination clarified the tumor as a mixed acinar-neuroendocrine-ductal carcinoma. The ductal and acinar/neuroendocrine tumor components were isolated using laser-capture microdissection, and next-generation sequencing analysis was performed. Consequently, frameshift (p.N210fs) and missense (p.G12R) mutations were identified in both ductal and acinar/neuroendocrine tumors. These results suggested a pancreatic mixed acinar-neuroendocrine-ductal carcinoma was derived from a founder tumor clone, and supports the notion that a founder tumor clone may differentiate and transform into a diverse histological type and form a pancreatic mixed carcinoma.
PubMed: 29085438
DOI: 10.3892/ol.2017.6786 -
Clinical Nuclear Medicine Nov 2017A previously healthy 4-year-old girl presented with petechial rash and low platelet count. There were no other symptoms. On abdominal ultrasound, a 4.7-cm heterogeneous...
A previously healthy 4-year-old girl presented with petechial rash and low platelet count. There were no other symptoms. On abdominal ultrasound, a 4.7-cm heterogeneous mass was demonstrated anterior to the left kidney. An abdominal MRI subsequently performed demonstrated a heterogeneously enhancing mass at the same location extending to the pancreas and spleen. A surgical biopsy of the mass was obtained. Pathology reported a malignant epithelioid neoplasm consistent with pancreatoblastoma. The mass demonstrated intense FDG uptake on PET and an FDG avid retrocaval lymph node.
Topics: Child, Preschool; Female; Fluorodeoxyglucose F18; Humans; Lymphatic Metastasis; Magnetic Resonance Imaging; Pancreatic Neoplasms; Positron Emission Tomography Computed Tomography; Retroperitoneal Space; Ultrasonography
PubMed: 28872552
DOI: 10.1097/RLU.0000000000001822 -
Journal of Indian Association of... 2017
PubMed: 28082787
DOI: 10.4103/0971-9261.194633 -
Journal of Pediatric Surgery Feb 2017While pancreaticoduodenectomy (PD) has been extensively studied in adults, there are few data pertaining specifically to pediatric patients. We retrospectively analyzed...
PURPOSE
While pancreaticoduodenectomy (PD) has been extensively studied in adults, there are few data pertaining specifically to pediatric patients. We retrospectively analyzed PD-associated morbidity and mortality in pediatric patients.
METHODS
Our analytic cohort included all consecutive patients ≤18years of age treated at our institution from 1993 to 2015 who underwent PD. Patient data (demographics, disease characteristics, surgical and adjuvant treatment, length of hospital stay, and postoperative course) were extracted from the medical records.
RESULTS
We identified 12 children with a median age of 9years (7 female, 5 male). Final diagnoses were pancreatoblastoma (n=3), solid pseudopapillary tumor (n=3), neuroblastoma (n=2), rhabdomyosarcoma (n=2), and neuroendocrine carcinoma (n=2). Four patients underwent PD for resection of recurrent disease. 75% (9/12 patients) received neoadjuvant therapy. The median operative time was approximately 7hours with a mean blood loss of 590cm. The distal pancreas was invaginated into the posterior stomach (n=3) or into the jejunum (n=5) or was directly sewn to the jejunal mucosa (n=4). There were no operative deaths. There were 4 patients (34%) with grade II complications, 1 with a grade IIIb complication (chest tube), and 1 with a grade IV complication (reexploration). The most common long-term morbidity was pancreas exocrine supplementation (n=10; 83%). Five patients (42%) diagnosed with either solid pseudopapillary tumor or rhabdomyosarcoma are currently alive with a mean survival of 77.4months.
CONCLUSION
Pancreaticoduodenectomy is a feasible management strategy for pediatric pancreatic malignancies and is associated with acceptable morbidity and overall survival. Long-term outcome is mostly dependent on histology of the tumor.
LEVEL OF EVIDENCE
Level IV; retrospective study with no comparison group.
Topics: Adolescent; Carcinoma, Neuroendocrine; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Male; Neuroblastoma; Pancreatic Neoplasms; Pancreaticoduodenectomy; Postoperative Complications; Retrospective Studies; Rhabdomyosarcoma; Survival Analysis; Treatment Outcome
PubMed: 27894759
DOI: 10.1016/j.jpedsurg.2016.11.025 -
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