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International Journal of Organ... 2016Organ transplantation in patients with prior malignancy increases the risk of tumor recurrence post-transplantation due to immunosuppression. Only two cases of liver...
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.
PubMed: 27721967
DOI: No ID Found -
Diagnostic Pathology Jun 2016Histologic 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... (Review)
Review
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.
CONCLUSION
This review discusses the histopathology, genetic and epigenetic alterations and potential treatment targets of the five major malignant pancreatic tumors - pancreatic ductal adenocarcinoma, pancreatic neuroendocrine tumor, solid-pseudopapillary neoplasm, acinar cell carcinoma and pancreatoblastoma.
Topics: Biomarkers, Tumor; Carcinoma, Acinar Cell; Carcinoma, Pancreatic Ductal; Eye Diseases, Hereditary; Humans; Neuroendocrine Tumors; Optic Nerve Diseases; Pancreas; Pancreatic Neoplasms
PubMed: 27267993
DOI: 10.1186/s13000-016-0497-z -
Journal of Clinical and Diagnostic... Mar 2016Pancreatoblastoma, is rare exocrine malignant tumour of childhood. We are reporting a case of three-year-old child presented to our hospital suffering from vague...
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. Surgery was done and mass was removed. By histopathology and immunohistochemistry it was diagnosed as pancreatoblastoma. The prognosis is very good in paediatric age, lacking evidence of metastatic disease at first presentation. Therefore early diagnosis is needed for specific treatment. The case is being reported because of its rarity.
PubMed: 27134883
DOI: 10.7860/JCDR/2016/15336.7413 -
Journal of Indian Association of... 2016Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar...
Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar resemblance. We report a case of a 4-year-old boy with a right hypochondriac region mass, which was diagnosed as hepatoblastoma on the basis of imaging, raised tumor marker, and biopsy; however, pancreatic origin of the mass was ascertained on exploration and pancreatoblastoma was confirmed on histopathology.
PubMed: 27046982
DOI: 10.4103/0971-9261.176969 -
Journal of Cancer Research and... 2015Although rare, pancreatoblastoma is the most common pancreatic tumor in children. Cushing syndrome secondary to ectopic secretion of adrenocorticotropic hormone (ACTH)...
Although rare, pancreatoblastoma is the most common pancreatic tumor in children. Cushing syndrome secondary to ectopic secretion of adrenocorticotropic hormone (ACTH) from a pancreatoblastoma is very rare with only two previously reported cases. We present the management and the lesson learnt in a 3-year-old child with recurrent pancreatoblastoma with Cushing syndrome.
Topics: Child, Preschool; Cushing Syndrome; Humans; Male; Neoplasm Recurrence, Local; Pancreatic Neoplasms; Prognosis
PubMed: 26881602
DOI: 10.4103/0973-1482.151854 -
Gland Surgery Aug 2015Pancreatoblastoma (PB) is a rare malignant neoplasm of the pancreas with unknown etiology. It occurs mostly in the pediatric population with very rare documented cases... (Review)
Review
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, 1.8-20 cm). The median follow up for patients was 15 months (range, 1-108 months) Metastatic disease and local infiltration of surrounding tissues is common with poor prognosis in adult patients. Preoperative diagnosis is difficult because of the unhelpful tumor markers in adults and the cellular heterogeneity of the tumor which makes fine needle aspiration cytology unreliable. Histopathological review of the tumor is essential for diagnosis. Pancreatoblastomas should be considered a differential diagnosis of solid and cystic pancreatic neoplasms. Surgical resection of the tumor is the treatment of choice with a variable combination with radiotherapy and chemotherapy.
PubMed: 26312218
DOI: 10.3978/j.issn.2227-684X.2015.04.05 -
Oncology Letters May 2015The 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)....
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. PB is a malignant tumour of the pancreas that typically occurs in the pediatric population. The aim of the present study was to highlight the aggressive behavior of this rare clinical entity, focusing on the pitfalls of pre-operative diagnosis and the lack of management strategy guidelines in adults. Preoperative diagnosis of PB based on radiographic features may be difficult, as the imaging characteristics are non-specific. Furthermore, cytology may also be misleading, as the neoplasm consists of multiple cell lines (acinar, ductal and neuroendocrine cells) and diagnosis depends largely on the identification of the distinctive histological characteristic of squamoid corpuscles, which present as nests of flattened cells with a squamous appearance. Despite the use of surgical resection and adjuvant chemoradiotherapy for the treatment of this malignancy, its aggressive nature means that PB is associated with a poor prognosis in adult patients.
PubMed: 26137059
DOI: 10.3892/ol.2015.3001 -
Seminars in Diagnostic Pathology Nov 2014Pancreatic neoplasms with acinar differentiation, including acinar cell carcinoma, pancreatoblastoma, and carcinomas with mixed differentiation, are distinctive...
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. Exocrine enzymes are detectable by immunohistochemistry and, for carcinomas with mixed differentiation, neuroendocrine or ductal lineage markers are also expressed. The genetic alterations of this family of neoplasms largely differ from conventional ductal adenocarcinomas, with only rare mutations in TP53, KRAS, and p16, but no single gene or neoplastic pathway is consistently altered in acinar neoplasms. Instead, there is striking genomic instability, and a subset of cases has mutations in the APC/β-catenin pathway, mutations in SMAD4, RAF gene family fusions, or microsatellite instability. Therapeutically targetable mutations are often present. This review summarizes the clinical and pathologic features of acinar neoplasms and reviews the current molecular data on these uncommon tumors.
Topics: Carcinoma; Carcinoma, Acinar Cell; Humans; Pancreatic Neoplasms
PubMed: 25441307
DOI: 10.1053/j.semdp.2014.08.003 -
Turk Patoloji Dergisi 2017Pancreatoblastoma, rarely encountered in the literature, is a malignant exocrine tumor seen in the pancreas. A 5-year-old boy suffering from abdominal pain was sent to...
Pancreatoblastoma, rarely encountered in the literature, is a malignant exocrine tumor seen in the pancreas. A 5-year-old boy suffering from abdominal pain was sent to our institute for further examination and treatment. Clinical examination was normal but for a palpable abdominal tumor mass. Abdominal Doppler ultrasonography showed a mass with well-defined margins within the body of the pancreas. Laboratory tests, including lactic dehydrogenase, alpha-fetoprotein and cancer antigen 125 were abnormal. The tumor invading the splenic vein and transverse colon was removed totally. We observed a hypercellular tumor in histopathological examination. The tumor had epithelial acinar cells and squamoid morules (corpuscles) separated by stromal bands. Adjuvant chemotherapy was used after surgery. However, the patient died 14 months later. All data about pancreatoblastoma have to be collected in order to choose the treatment to elucidate the molecular pathogenesis of the tumor, to diagnose it early and to develop target-specific treatments.
Topics: Child, Preschool; Fatal Outcome; Humans; Male; Pancreatic Neoplasms
PubMed: 25110242
DOI: 10.5146/tjpath.2014.01268 -
Transplant International : Official... Oct 2014Hepatic venous outflow reconstruction is of critical significance in pediatric patients undergoing living donor liver transplantation. Accurate knowledge of the...
Hepatic venous outflow reconstruction is of critical significance in pediatric patients undergoing living donor liver transplantation. Accurate knowledge of the anatomical variations is important to obtain appropriate size segmental grafts. The diameter of the hepatic veins and the potential risk of complications at the level of the anastomosis require an adequate primary vascular reconstruction. We describe a venous outflow reconstruction technique, in a living related left lateral lobe graft, with unfavorable hepatic venous anatomy.
Topics: Child; Female; Follow-Up Studies; Graft Survival; Hepatic Veins; Humans; Liver; Liver Circulation; Liver Neoplasms; Liver Transplantation; Living Donors; Pancreatic Neoplasms; Plastic Surgery Procedures; Risk Assessment; Treatment Outcome; Vascular Surgical Procedures
PubMed: 24963596
DOI: 10.1111/tri.12388