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BMJ Case Reports Apr 2020
Topics: Aged; Carcinoma, Acinar Cell; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Humans; Male; Mesenteric Veins; Neoadjuvant Therapy; Pancreatic Neoplasms; Thrombosis; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 32265211
DOI: 10.1136/bcr-2019-233884 -
Annals of Hepato-biliary-pancreatic... Feb 2020Pancreatoblastoma is a malignant exocrine pancreatic tumor that is usually present in childhood. We herein present one case of pediatric living donor liver...
Pancreatoblastoma is a malignant exocrine pancreatic tumor that is usually present in childhood. We herein present one case of pediatric living donor liver transplantation (LDLT) combined with spleen-preserving regional total pancreatectomy and portal vein homograft interposition in a 4-year-old boy with advanced pancreatoblastoma invading the portal and superior mesenteric veins. The size of the pancreatoblastoma was gradually reduced along systemic chemotherapy, thus we decided to perform surgery to remove it completely. A cold-stored fresh iliac vein homograft was prepared. Initially, a spleen-preserving distal pancreatectomy was performed. Thereafter, a completion regional total pancreatectomy was performed under superior mesenteric vein-vena cava bypass. A left liver graft from his mother was implanted according to the standardized procedures with portal vein interposition. This patient recovered uneventfully and is currently undergoing scheduled adjuvant chemotherapy. To our knowledge, this is the world-second case of pediatric LDLT for advanced pancreatoblastoma. Availability of fresh vein homografts is helpful to expand the indication of pediatric LDLT.
PubMed: 32181434
DOI: 10.14701/ahbps.2020.24.1.78 -
Pancreatology : Official Journal of the... Apr 2020Pancreatoblastoma is a rare malignancy that occurs predominantly in children. Less than 50 adult cases, including 17 patients with metastatic disease, have been...
BACKGROUND
Pancreatoblastoma is a rare malignancy that occurs predominantly in children. Less than 50 adult cases, including 17 patients with metastatic disease, have been published to date. Recent outcome data from children with advanced-stage disease suggest an intensive multimodal treatment approach; however, little is known about the most beneficial therapy in adults. Molecular characterization of pancreatoblastoma is limited to a small number of pediatric cases and revealed few recurrent genetic events without immediate clinical relevance.
METHODS
Patients were treated between 2013 and 2018 at a high-volume German university cancer center. Molecular analyses included whole genome, exome, transcriptome, and fusion gene panel sequencing. Molecularly guided treatment recommendations were discussed within a dedicated molecular tumor board (MTB) embedded in a precision oncology program (NCT MASTER).
RESULTS
We identified four adult patients with metastatic pancreatoblastoma. In three patients, local approaches were combined with systemic treatment. Oxaliplatin-containing protocols showed an acceptable tumor control as well as an adequate toxicity profile. Overall survival was 15, 17, 18 and 24 months, respectively. Three tumors harbored genetic alterations involving the FGFR pathway that included an oncogenic FGFR2 fusion.
CONCLUSION
Oxaliplatin-containing chemotherapy seems to be a reasonable approach in adult patients with advanced pancreatoblastoma, whereas the benefit of intensified treatment including local ablative techniques or surgical resection remains unclear. Our finding of FGFR alterations in three of four cases indicates a potential role of FGFR signaling in adult pancreatoblastoma whose clinical significance warrants further study.
Topics: Adult; Antineoplastic Agents; Chromosome Mapping; Combined Modality Therapy; Exome; Female; Gene Fusion; Genome, Human; Humans; Male; Neoplasm Metastasis; Oxaliplatin; Pancreatic Neoplasms; Pancreaticoduodenectomy; Precision Medicine; Receptor, Fibroblast Growth Factor, Type 2; Survival Analysis; Transcriptome; Young Adult
PubMed: 32156527
DOI: 10.1016/j.pan.2020.02.017 -
Cureus Jan 2020Pancreatoblastoma (PB) is a rare pancreatic neoplasm which arises when a group of pancreatic cells start to go through uncontrollable growth. The diagnosis of PB is...
Pancreatoblastoma (PB) is a rare pancreatic neoplasm which arises when a group of pancreatic cells start to go through uncontrollable growth. The diagnosis of PB is challenging due to its vague symptoms. The initial diagnosis is made by imaging, afterwards the management is usually by resection of the tumor with or without chemotherapy which depends on the size and grade of the tumor. We report a case of a nine-year-old girl who was diagnosed with pancreotoblastoma and underwent complete resection with chemotherapy.
PubMed: 32015939
DOI: 10.7759/cureus.6779 -
Minerva Gastroenterologica E Dietologica Mar 2020Solid pancreatic lesions include mainly adenocarcinoma, neuroendocrine tumors pancreatic cystic neoplasms with solid component, solid pseudopapillary tumor,... (Review)
Review
Solid pancreatic lesions include mainly adenocarcinoma, neuroendocrine tumors pancreatic cystic neoplasms with solid component, solid pseudopapillary tumor, pancreatoblastoma, pancreatic lymphoma, and pancreatic metastasis. The most frequent pancreatic lesion is the adenocarcinoma, representing between 70% and 95% of all solid pancreatic neoplasm. The diagnosis of these lesions can be a challenge and currently, there are different imaging techniques such as CT scan, EUS and MRI with high sensitivity and specificity. The most widely used technique for the initial evaluation is the CT scan with a sensitivity between 76% and 92% for the diagnosis of pancreatic cancer. The EUS has a sensitivity for the detection of pancreatic lesions of around 98% and is accepted to be the most sensitive technique for the detection of small pancreatic tumors (<2 cm). The MRI, with a very high soft-tissue contrast resolution, provides an accuracy in the detection and staging of adenocarcinoma of 90-100%. A multimodality approach is usually necessary in patients with clinical suspicion of pancreatic lesion. The EUS is required for the local evaluation of the relation of the lesion with vessels and for tissue acquisition and the CT scan and/or MRI is usually required for the local and distance staging in case of pancreatic cancer. The purpose of this review is to provide an overview of solid pancreatic lesions and the role of the different imaging techniques in their evaluation.
Topics: Diagnosis, Differential; Diagnostic Imaging; Humans; Pancreatic Neoplasms
PubMed: 31994370
DOI: 10.23736/S1121-421X.20.02646-X -
Pediatric Surgery International Mar 2020Pancreatic tumors are rare in children and limited data are available regarding incidence, treatment, and outcomes. We aim to describe patient and tumor characteristics...
PURPOSE
Pancreatic tumors are rare in children and limited data are available regarding incidence, treatment, and outcomes. We aim to describe patient and tumor characteristics and to report on survival of these diseases.
METHODS
Children with pancreatic tumors were queried from the National Cancer Database (2004-2014). The association between treatment and hazard of death was assessed using Kaplan-Meier method and Cox regression model.
RESULTS
We identified 109 children with pancreatic tumors; 52% were male and median age at diagnosis was 14 years. Tumors were distributed as follows: pseudopapillary neoplasm (30%), endocrine tumors (27%), pancreatoblastoma (16%), pancreatic adenocarcinoma (16%), sarcoma (6%) and neuroblastoma (5%). Seventy-nine patients underwent surgery, of which 76% achieved R0 resection. Most patients (85%) had lymph nodes examined, of which 22% had positive nodes. Five-year overall survival by tumor histology was 95% (pseudopapillary neoplasm), 75% (neuroblastoma), 70% (pancreatoblastoma), 51% (endocrine tumors), 43% (sarcoma), and 34% (adenocarcinoma). On multivariable analysis, surgical resection was the strongest predictor of survival (HR 0.26, 95% CI 0.10-0.68, p < 0.01).
CONCLUSION
Overall survival of children with pancreatic tumors is grim, with varying survival rates among different tumors. Surgical resection is associated with improved long-term survival.
Topics: Adenocarcinoma; Adolescent; Aged; Child; Child, Preschool; Combined Modality Therapy; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; Neoplasm Staging; Pancreatectomy; Pancreatic Neoplasms; Survival Rate; United States
PubMed: 31989243
DOI: 10.1007/s00383-020-04617-z -
Zhonghua Bing Li Xue Za Zhi = Chinese... Jan 2020
Topics: Humans; Pancreas; Pancreatic Neoplasms; Stomach; Stomach Neoplasms
PubMed: 31914546
DOI: 10.3760/cma.j.issn.0529-5807.2020.01.021 -
Archives of Pathology & Laboratory... Jul 2020Pancreatic acinar lesions encompass a broad spectrum of malignant tumors and benign reactive processes affecting both adults and children, with clinical, pathologic, and... (Review)
Review
CONTEXT.—
Pancreatic acinar lesions encompass a broad spectrum of malignant tumors and benign reactive processes affecting both adults and children, with clinical, pathologic, and molecular features that are distinct from more common ductal neoplasms. Accurate morphologic diagnosis and molecular assessment of these uncommon neoplasms is critical for effective patient care.
OBJECTIVE.—
To review the clinical, pathologic, and molecular features of pancreatic neoplasms with acinar differentiation, the most common of which is acinar cell carcinoma but which also includes mixed carcinomas with acinar components, cystic acinar lesions, and pancreatoblastoma.
DATA SOURCES.—
We assessed the current primary literature, as well as recently updated diagnostic manuals.
CONCLUSIONS.—
Pancreatic acinar neoplasms are a morphologically and molecularly heterogeneous group of diseases that are characterized by acinar differentiation of at least a subset of the neoplastic cells, defined either morphologically (granular cytoplasm, single prominent nucleoli) or immunohistochemically. Squamoid nests are a key morphologic feature of pancreatoblastoma. Alterations in WNT signaling and chromosomal 11p loss are common molecular features of both acinar cell carcinoma and pancreatoblastoma. Targetable molecular alterations in acinar carcinoma include BRAF rearrangements and DNA repair defects, including mismatch repair deficiency and BRCA pathway defects. For practicing pathologists, morphologic recognition of such acinar neoplasms is critical, and in the future, molecular diagnostics to identify lesions susceptible to targeted therapy will likely form an important component of patient care.
Topics: Biomarkers, Tumor; Biopsy; Carcinoma, Acinar Cell; Cell Differentiation; Genetic Predisposition to Disease; Humans; Immunohistochemistry; Molecular Diagnostic Techniques; Neoplasms, Complex and Mixed; Pancreatic Neoplasms; Phenotype; Predictive Value of Tests
PubMed: 31869246
DOI: 10.5858/arpa.2019-0472-RA -
Revista de Gastroenterologia Del Peru :... 2019Pancreoblastoma is a very rare tumor that originates from the exocrine epithelial cells of the pancreas. However, it is the most frequent pancreatic tumor in children....
Pancreoblastoma is a very rare tumor that originates from the exocrine epithelial cells of the pancreas. However, it is the most frequent pancreatic tumor in children. It usually appears in the first decade of life with an average of 5 years old. The clinical manifestations are usually unspecific, being the abdominal pain and the abdominal mass the most frequent. The radiological signs are not very well described in medical literature. The definite diagnosis should always be established with biopsia and histological examination. Here it is presented a clinical case of pancreoblastoma with unusual involvement of a major duodenal papilla in a pediatric patient.
Topics: Ampulla of Vater; Child, Preschool; Female; Humans; Pancreatic Neoplasms
PubMed: 31688859
DOI: No ID Found -
The American Journal of Medicine Jun 2020
Topics: Abdominal Wall; Adult; Anticoagulants; Enoxaparin; Hematoma; Humans; Injections, Subcutaneous; Male; Pancreatic Neoplasms; Physical Examination; Venous Thrombosis
PubMed: 31677935
DOI: 10.1016/j.amjmed.2019.09.027