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Pancreatoblastoma

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https://www.readbyqxmd.com/read/29535845/identification-and-characterization-of-a-novel-adenomatous-polyposis-coli-mutation-in-adult-pancreatoblastoma
#1
Shigeo Yamaguchi, Tomoaki Fujii, Yuki Izumi, Yuki Fukumura, Min Han, Hideki Yamaguchi, Tomomi Akita, Chikamasa Yamashita, Shunsuke Kato, Takao Sekiya
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 adenomatous polyposis coli ( APC ) gene. We also found a heterogeneous mutation in the 1835 codon of the APC gene in the patient's germline by Sanger sequencing...
February 13, 2018: Oncotarget
https://www.readbyqxmd.com/read/29307199/pathologic-classification-of-pancreatic-cancers-current-concepts-and-challenges
#2
Mohamed E Mostafa, Ipek Erbarut-Seven, Burcin Pehlivanoglu, Volkan Adsay
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...
December 2017: Chinese Clinical Oncology
https://www.readbyqxmd.com/read/29285688/pancreatoblastoma-an-unusual-diagnosis-in-an-adult-patient
#3
Gonçalo Nunes, Hélder Coelho, Marta Patita, Rita Barosa, Pedro Pinto Marques, Lídia Roque Ramos, Maria José Brito, Aurora Tomaz, Jorge Fonseca
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...
April 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29277262/pancreatectomies-for-pancreatic-neoplasms-in-pediatric-and-adolescent-age-a-single-institution-experience
#4
Chiara Scandavini, Roberto Valente, Elena Rangelova, Ralf Segersvärd, Urban Arnelo, Johan Permert, Pär-Johan Svensson, Jakob Stenman, Marco Del Chiaro
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...
March 2018: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/29259370/pancreatic-acinar-cell-carcinoma-a-review-on-molecular-profiling-of-patient-tumors
#5
REVIEW
Ahmad Al-Hader, Rami N Al-Rohil, Haiyong Han, Daniel Von Hoff
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...
December 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29235221/liver-transplant-for-metastatic-pancreatoblastoma-7-year-event-free-survival-after-chemotherapy-pancreatectomy-complete-hepatectomy-and-liver-transplant
#6
A A Ghaffarian, L Book, R L Meyers
Pancreatoblastoma is a rare malignant tumor in children. Surgical resection of the tumor is necessary for cure; however, due to its aggressive nature, it is often unresectable at presentation due to tumor size, local invasion, and/or metastasis. Because it is a rare tumor, there is currently no standard treatment regimen. We report a case of a 4-year-old boy who presented with metastatic pancreatoblastoma with multiple large metastases involving all four sectors of the liver. We began treatment with chemotherapy (cisplatin, 5FU, vincristine, and doxorubicin), which significantly reduced the tumor burden in both the pancreas and liver...
February 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29233928/integrated-molecular-characterization-of-the-lethal-pediatric-cancer-pancreatoblastoma
#7
Tomoya Isobe, Masafumi Seki, Kenichi Yoshida, Masahiro Sekiguchi, Yusuke Shiozawa, Yuichi Shiraishi, Shunsuke Kimura, Misa Yoshida, Yoshikage Inoue, Akira Yokoyama, Nobuyuki Kakiuchi, Hiromichi Suzuki, Keisuke Kataoka, Yusuke Sato, Tomoko Kawai, Kenichi Chiba, Hiroko Tanaka, Teppei Shimamura, Motohiro Kato, Akihiro Iguchi, Asahito Hama, Tomoaki Taguchi, Masaharu Akiyama, Junya Fujimura, Akiko Inoue, Tsuyoshi Ito, Takao Deguchi, Chikako Kiyotani, Tomoko Iehara, Hajime Hosoi, Akira Oka, Masashi Sanada, Yukichi Tanaka, Kenichiro Hata, Satoru Miyano, Seishi Ogawa, Junko Takita
Pancreatoblastoma is a rare pediatric pancreatic malignancy for which the molecular pathogenesis is not understood. In this study, we report the findings of an integrated multiomics study of whole-exome and RNA sequencing as well as genome-wide copy number and methylation analyses of ten pancreatoblastoma cases. The pancreatoblastoma genome was characterized by a high frequency of aberrant activation of the Wnt signaling pathway, either via somatic mutations of CTNNB1 (90%) and copy-neutral loss of heterozygosity (CN-LOH) of APC (10%)...
February 15, 2018: Cancer Research
https://www.readbyqxmd.com/read/29224282/-clinicopathologic-characteristics-of-pancreatoblastoma
#8
N Hou, W B Guan, R F Wang, W W Yu, M Qiao, Y Yan, Y Q Sun, L F Wang
No abstract text is available yet for this article.
December 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28872552/pancreatoblastoma-with-metastatic-retroperitoneal-lymph-node-and-pet-ct
#9
Giuseppe Corrias, Monica Ragucci, Olca Basturk, Luca Saba, Lorenzo Mannelli
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...
September 1, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28693851/a-population-based-analysis-of-a-rare-oncologic-entity-malignant-pancreatic-tumors-in-children
#10
Konstantinos S Mylonas, Dimitrios Nasioudis, Diamantis I Tsilimigras, Ilias P Doulamis, Peter T Masiakos, Cassandra M Kelleher
PURPOSE: To examine the clinicopathological characteristics and prognosis of pediatric patients with malignant pancreatic tumors in a population-based cohort. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify all pediatric patients with malignant pancreatic tumors, diagnosed between 1973 and 2013. Kaplan-Meier analysis was performed to determine median and five-year overall survival (OS) rates. Univariate survival analysis was executed using the log-rank test...
June 30, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28538503/complete-resection-of-pancreatoblastoma-with-portal-vein-obstruction-after-high-dose-chemotherapy-a-case-report
#11
Souji Ibuka, Shuichiro Uehara, Takehisa Ueno, Takaharu Oue, Takako Miyamura, Yoshiko Hashii, Hiroomi Okuyama
BACKGROUND: As no standard chemotherapy exists for pancreatoblastoma (PB), surgical resection is the most effective way of achieving complete remission. CASE REPORT: A 3-year-old girl with PB causing portal vein obstruction was referred to our hospital. Because of the portal vein involvement, she initially received 5 cycles of preoperative chemotherapy after biopsy, consisting of cyclophosphamide, vincristine, pirarubicin, and cisplatin. After chemotherapy, she underwent distal pancreatectomy, and the tumor was resected completely...
July 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28194556/pancreatoblastoma-an-atypical-presentation-and-a-literature-review
#12
Marisa Terino, Eileen Plotkin, Raffi Karagozian
No abstract text is available yet for this article.
February 14, 2017: Journal of Gastrointestinal Cancer
https://www.readbyqxmd.com/read/27926361/nonductal-pancreatic-cancers
#13
REVIEW
Sun-Young Jun, Seung-Mo Hong
Nonductal pancreatic neoplasms, including solid pseudopapillary neoplasms, acinar cell carcinomas, and pancreatoblastomas, are uncommon. These entities share overlapping gross, microscopic, and immunohistochemical features, such as well-demarcated solid neoplasms, monotonous cellular tumor cells with little intervening stroma, and abnormal beta-catenin expression. Each tumor also has unique clinicopathologic characteristics with diverse clinical behavior. To differentiate nonductal pancreatic neoplasms, identification of histologic findings, such as pseudopapillae, acinar cell features, and squamoid corpuscles, is important...
December 2016: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/27894759/pancreaticoduodenectomy-for-pediatric-and-adolescent-pancreatic-malignancy-a-single-center-retrospective-analysis
#14
Erika B Lindholm, Abdulaziz K Alkattan, Sara J Abramson, Anita P Price, Todd E Heaton, Vinod P Balachandran, Michael P La Quaglia
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...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27761191/adult-pancreatoblastoma-case-report-and-review-of-literature
#15
REVIEW
Filipa Vilaverde, Alcinda Reis, Pedro Rodrigues, Ana Carvalho, Horácio Scigliano
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...
August 2016: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/27721967/multi-visceral-transplantation-in-a-21-year-old-man-with-prior-pancreatoblastoma
#16
R Girlanda, A Pozzi, C S Matsumoto, T M Fishbein
Organ transplantation in patients with prior malignancy increases the risk of tumor recurrence post-transplantation due to immunosuppression. Only two cases of liver transplantation have so far been reported in children with hepatic metastases from pancreatoblastoma, a rare malignant neoplasm originating from the epithelial exocrine cells of the pancreas. Herein, we describe a case of a successful multi-visceral transplant in a man with intestinal failure after surgical resection of pancreatoblastoma.
2016: International Journal of Organ Transplantation Medicine
https://www.readbyqxmd.com/read/27364750/pancreatic-surgery-for-tumors-in-children-and-adolescents
#17
Maria G Sacco Casamassima, Colin D Gause, Seth D Goldstein, Fizan Abdullah, Avner Meoded, Jeffrey R Lukish, Christopher L Wolfgang, John Cameron, David J Hackam, Ralph H Hruban, Paul M Colombani
PURPOSE: Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies. METHODS: We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015. RESULTS: Nineteen patients were identified with a median age at operation of 16...
August 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27320062/acinar-neoplasms-of-the-pancreas-a-summary-of-25-years-of-research
#18
REVIEW
David S Klimstra, Volkan Adsay
Our understanding about the family of acinar neoplasms of the pancreas has grown substantially over the past 25 years. The prototype is acinar cell carcinoma, an uncommon variant of pancreatic carcinoma that demonstrates production of pancreatic exocrine enzymes, verifiable using immunohistochemistry, and exhibits characteristic histologic features. Related neoplasms include mixed acinar carcinomas such as mixed acinar neuroendocrine carcinoma and mixed acinar ductal carcinoma. In the pediatric age group, pancreatoblastoma is also closely related...
September 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/27267993/surgical-and-molecular-pathology-of-pancreatic-neoplasms
#19
REVIEW
Wenzel M Hackeng, Ralph H Hruban, G Johan A Offerhaus, Lodewijk A A Brosens
BACKGROUND: Histologic characteristics have proven to be very useful for classifying different types of tumors of the pancreas. As a result, the major tumor types in the pancreas have long been classified based on their microscopic appearance. MAIN BODY: Recent advances in whole exome sequencing, gene expression profiling, and knowledge of tumorigenic pathways have deepened our understanding of the underlying biology of pancreatic neoplasia. These advances have not only confirmed the traditional histologic classification system, but also opened new doors to early diagnosis and targeted treatment...
June 7, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27134883/pancreatoblastoma-an-unusual-occurrence-of-a-tumour-in-paediatric-age-group-a-case-report
#20
Pankaj Halder, Bedabrata Mukhopadhyay, Chhanda Das, Madhumita Mukhopadhyay
Pancreatoblastoma, is rare exocrine malignant tumour of childhood. We are reporting a case of three-year-old child presented to our hospital suffering from vague abdominal pain for further examination and treatment. Clinical examination revealed only a palpable abdominal mass. CT Scan revealed a huge complex space occupying lesion 9.1x8.8x9.2cm with large central cystic degeneration and lobulated enhancing peripheral solid components with foci of calcification, seem to arise from body and tail regions of pancreas...
March 2016: Journal of Clinical and Diagnostic Research: JCDR
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