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Pancreatoblastoma in adults

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
Ayodeji Oluwarotimi Omiyale
Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases in adults. This is a review of the case reports of the adult pancreatoblastoma in the literature. A total of 35 cases were identified and reviewed with the mean age of 41 years (range, 18-78 years) and the male sex accounted for 51.4% of the cases. Adult Pancreatoblastoma seem to have a predilection for the head of the pancreas which accounted for approximately 49% of the cases reviewed with an average size of 8 cm (range, 1...
August 2015: Gland Surgery
Efstratios Zouros, Dimitrios K Manatakis, Spiros G Delis, Christos Agalianos, Charina Triantopoulou, Christos Dervenis
The present study describes the case of a 24-year-old patient who presented with obstructive jaundice and weight loss, and was diagnosed with pancreatoblastoma (PB). Abdominal imaging studies revealed a heterogenous lesion of the pancreatic head with dilatation of the common bile duct. The patient underwent pancreaticoduodenectomy, however, three months after surgery multiple liver and bone metastases were identified on follow-up computed tomography scans. Despite treatment with four cycles of systemic chemotherapy and five courses of radiofrequency ablation, the patient succumbed due to tumour dissemination 13 months after initial diagnosis...
May 2015: Oncology Letters
Di Zhang, Na Tang, Yang Liu, En-Hua Wang
Pancreatoblastoma is a malignant pancreatic tumor that rarely occurs in adults. We report a case of an adult female with pancreatoblastoma. A mass was detected in the pancreatic head using computed tomography and ultrasonography. The clinical diagnosis was a solid-pseudopapillary neoplasm of the pancreas. However, after the operation, the final diagnosis was pancreatoblastoma, which showed two lines of differentiation: Acinar differentiation and squamoid corpuscles. The patient is currently in good condition...
January 2015: Indian Journal of Pathology & Microbiology
Laura D Wood, David S Klimstra
Pancreatic neoplasms with acinar differentiation, including acinar cell carcinoma, pancreatoblastoma, and carcinomas with mixed differentiation, are distinctive pancreatic neoplasms with a poor prognosis. These neoplasms are clinically, pathologically, and genetically unique when compared to other more common pancreatic neoplasms. Most occur in adults, although pancreatoblastomas usually affect children under 10 years old. All of these neoplasms exhibit characteristic histologic features including a solid or acinar growth pattern, dense neoplastic cellularity, uniform nuclei with prominent nucleoli, and granular eosinophilic cytoplasm...
November 2014: Seminars in Diagnostic Pathology
Bulent Salman, Gabriel Brat, Yoo-Seok Yoon, Ralph H Hruban, Aatur D Singhi, Elliot K Fishman, Joseph M Herman, Christopher L Wolfgang
INTRODUCTION: Pancreatoblastoma is an extremely rare pancreatic neoplasm in adults. The aim of this study is to report our experience with adult pancreatoblastoma as well as review the cases reported in the literature in order to provide guidelines for the management of patients with this rare neoplasm. METHODS: We have encountered three cases of pancreatoblastoma in adults at our institution in addition to the 30 cases reported to date in literature. RESULTS: The median age of pancreatoblastoma in adults is 37 years (range, 18-78 years); men and women are similarly affected (male/female = 16/17)...
December 2013: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Suntrea T G Hammer, Scott R Owens
Pancreatoblastomas are malignant epithelial neoplasms of the pancreas that are heterogeneous and have variable cellular differentiation, complicating the diagnosis. We report a case of pancreatoblastoma occurring in an adult patient, presenting as a pancreatic head mass with liver metastasis and jaundice. The initial liver biopsy diagnosis was metastatic neuroendocrine carcinoma based on morphology and synaptophysin positivity. At pancreatic resection, the diagnostic features of pancreatoblastoma were recognized...
September 2013: Archives of Pathology & Laboratory Medicine
Carlie S Sigel, David S Klimstra
BACKGROUND: Acinar cell neoplasms of the pancreas are rare but when encountered, the diagnosis is often established based on cytology specimens. Diagnostic accuracy is important because acinar cell carcinomas are aggressive yet may mimic tumors with different outcomes and management. METHODS: The authors identified all patients with a diagnosis of acinar cell neoplasm in the institutional database; assessed cytomorphology and immunocytochemistry for trypsin, chymotrypsin, synaptophysin, chromogranin A, and MIB-1; and compared all cytology and final histological diagnoses for diagnostic discrepancies...
August 2013: Cancer Cytopathology
Ryo Sueyoshi, Tadaharu Okazaki, Geoffrey J Lane, Atsushi Arakawa, Takashi Yao, Atsuyuki Yamataka
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...
2013: International Journal of Surgery Case Reports
E Gringeri, M Polacco, F E D'Amico, D Bassi, R Boetto, F Tuci, P Bonsignore, G Noaro, F D'Amico, A Vitale, P Feltracco, S Barbieri, D Neri, G Zanus, U Cillo
Ex situ ex vivo liver surgery represents a method to expand the surgical indications to treat otherwise unresectable liver tumors. We report the case of a 38-year old woman with hepatic metastasis from a pancreatoblastoma that was judged to be unresectable due to the involvement of the three hepatic veins. To treat the primary tumor, she underwent a pancreaticoduodenectomy, adjuvant chemotherapy, and thermal ablation of a liver metastasis. After appropriate preoperative study and with the permission of the ethics committee, she underwent ex situ ex vivo liver resection...
September 2012: Transplantation Proceedings
Chitra Balasundaram, Munish Luthra, Disaya Chavalitdhamrong, Jonathan Chow, Hina Khan, Paul J H Endres
CONTEXT: Pancreatoblastoma is a rare neoplasm in adults with a total of only 24 cases that have been reported in the literature. Adult pancreatoblastomas are large tumors and majority are larger than 8 cm at the time of diagnosis. Metastasis is seen in 26% of adults and usually involves the liver and then the lymph nodes. Metastasis is usually observed in cases where the primary tumor measures more than 10 cm. Pancreatoblastoma is named after its resemblance to fetal pancreatic tissue in the seventh week of life...
May 2012: JOP: Journal of the Pancreas
Richard D Glick, Farzana D Pashankar, Alberto Pappo, Michael P Laquaglia
Pancreatoblastoma is a very rare childhood tumor originating from the epithelial exocrine cells of the pancreas. It is the most common malignant pancreatic tumor in young children and has a mean age of diagnosis of 5 years. It is slow growing and its presentation is varied and often non-specific. Tumors tend to be quite large and appropriate cross sectional imaging is very important to assess for extent, metastatic disease, and resectability. Biopsy for tissue diagnosis is essential. Complete surgical resection is the goal of therapy although many patients are unresectable at initial diagnosis and require neoadjuvant chemotherapy...
May 2012: Journal of Pediatric Hematology/oncology
Gemma Gatta, Andrea Ferrari, Charles A Stiller, Guido Pastore, Gianni Bisogno, Annalisa Trama, Riccardo Capocaccia
Embryonal cancers are a heterogeneous group of rare cancers which mainly occur in children and adolescents. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal embryonal cancers in Europe (EU27), using population-based data from cancer registries (CRs) participating in RARECARE. We identified 3322 cases diagnosed from 1995 to 2002 (latest period for which data are available): 44% neuroblastoma, 35% nephroblastoma, 13% retinoblastoma and 6% hepatoblastoma...
July 2012: European Journal of Cancer
Birgit Geoerger, Edward J Estlin, Isabelle Aerts, Pamela Kearns, Brenda Gibson, Nadège Corradini, François Doz, Pilar Lardelli, Bernardo De Miguel, Arturo Soto, Raquel Prados, Gilles Vassal
AIMS: To determine the maximum tolerated dose, the recommended dose (RD) for phase II studies, dose-limiting toxicities and pharmacokinetics (PK) for plitidepsin administered as a 3-h intravenous infusion every 2weeks (one cycle) to children with refractory or relapsed solid tumours. METHODS: Consecutive cohorts of patients were treated according to a standard '3+3' design with escalating doses of plitidepsin at 4, 5 and 6mg/m(2). Additional 15 patients were recruited at the RD to further evaluate safety and pharmacokinetic associations with respect to age, dose level and toxicity...
February 2012: European Journal of Cancer
I B Brecht, D T Schneider, G Klöppel, D von Schweinitz, W Barthlen, M R Hamre
BACKGROUND: Malignant pancreatic tumors are rare in young patients, few epidemiologic data are available. We reviewed prognostic factors and outcome of 228 patients <30 years with malignant pancreatic tumors identified through the U.S. National Cancer Institute's SEER (Surveillance, Epidemiology, and End Results) Public-use Database from 1973 to 2004. METHODS: Cases were grouped using the ICD-O-3. 5-year overall survival (OAS) was assessed by gender, ethnicity, SEER stage, and 5-year age intervals using univariate and Cox regression analysis...
November 2011: Klinische Pädiatrie
Asmaa Gaber Abdou, Nancy Youssef Asaad, Ahmed Elkased, Hala Said, Marwa Dawoud
In this report, we describe a classic case of stroma rich neuroblastoma, nodular type in a 22 year old female presented with a pancreatic mass. This rare and unusual presentation elicits several differential diagnostic categories including solid pseudopapillary tumor, pancreatic endocrine tumor, pancreatoblastoma and PNET. In this report, we tried to differentiate between them depending on the histopathological features and using panel of epithelial and neuroendocrine markers. Although of the rarity of pancreatic neuroblastoma as a primary site of origin, however it should be considered in the differential diagnosis of pancreatic masses in children and young adult...
April 2012: Pathology Oncology Research: POR
Yan Guo, Fei Yuan, Huan Deng, Hua-Feng Wang, Xiao-Long Jin, Jia-Cheng Xiao
Solid-pseudopapillary neoplasm of the pancreas is a rare tumor of uncertain histogenesis that is characterized by a cystic and solid growth pattern with pseudopapillary structures. The differentiation of solid-pseudopapillary neoplasm from other pancreatic tumors is of great importance. However, it is sometimes difficult because of similarities in morphologic features and immunophenotype. CD99 is a diagnostically useful marker for Ewing sarcoma/primitive neuroectodermal tumor. The aim of this study was to investigate the diagnostic value of CD99 in solid-pseudopapillary neoplasm...
June 2011: American Journal of Surgical Pathology
Jason Y Park, Seung-Mo Hong, David S Klimstra, Michael G Goggins, Anirban Maitra, Ralph H Hruban
Pancreatic and duodenal homeobox (Pdx1) is a homeobox transcription factor required for the embryonic development of the pancreas. Pdx1 expression has been earlier identified in pancreatic ductal adenocarcinomas and endocrine neoplasms. This study characterizes Pdx1 protein expression in pancreatic precursor lesions and neoplasms, including pancreatic intraepithelial neoplasia (PanIN, n=32), intraductal papillary mucinous neoplasm (IPMN, n=88), mucinous cystic neoplasm (MCN, n=3), acinar cell carcinoma (ACC, n=8), pancreatic endocrine neoplasm (PEN, n=44), pancreatoblastoma (PB, n=1), solid pseudopapillary neoplasm (n=8), invasive ductal adenocarcinoma (n=67), and nondysplastic ductal epithelium...
October 2011: Applied Immunohistochemistry & Molecular Morphology: AIMM
Stefania Sorrentino, Massimo Conte, Paolo Nozza, Claudio Granata, Valeria Capra, Stefano Avanzini, Alberto Garaventa
Beckwith-Wiedemann syndrome is associated with an increased risk of tumors in the pediatric age. We report the case of a newborn with Beckwith-Wiedemann syndrome presenting the simultaneous occurrence of a cystic pancreatoblastoma and an adrenal neuroblastoma. Surgery was required to treat the pancreatoblastoma, and a "wait and see" policy was adopted for the neuroblastoma, which spontaneously regressed within a few months.
July 2010: Journal of Pediatric Hematology/oncology
Patrizia Dall'igna, Giovanni Cecchetto, Gianni Bisogno, Massimo Conte, Pierluigi Lelli Chiesa, Paolo D'Angelo, Francesco De Leonardis, Gianluca De Salvo, Francesca Favini, Andrea Ferrari
INTRODUCTION: Malignant pancreatic tumors are exceedingly rare in pediatric age and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons. METHODS: From January 2000 to July 2009, 21 patients <18 years old with pancreatic tumors were prospectively registered in the Italian cooperative TREP project dedicated to very rare pediatric tumors. RESULTS: Tumor types were 4 pancreatoblastomas, 2 pancreatic carcinomas, 3 neoplasms of the endocrine pancreas, and 12 solid pseudopapillary tumors...
May 2010: Pediatric Blood & Cancer
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