MCID: PNC019
MIFTS: 38

Pancreatoblastoma

Categories: Cancer diseases, Endocrine diseases, Gastrointestinal diseases, Rare diseases

Aliases & Classifications for Pancreatoblastoma

MalaCards integrated aliases for Pancreatoblastoma:

Name: Pancreatoblastoma 12 74 20 58 44 15 17 71

Characteristics:

Orphanet epidemiological data:

58
pancreatoblastoma
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),<1/1000000 (Worldwide); Age of onset: Adult,Childhood; Age of death: adult,early childhood,late childhood;

Classifications:

Orphanet: 58  
Rare gastroenterological diseases


External Ids:

Disease Ontology 12 DOID:6823
MeSH 44 C537162
NCIt 50 C4265
SNOMED-CT 67 189814006
MESH via Orphanet 45 C537162
ICD10 via Orphanet 33 C25.1
UMLS via Orphanet 72 C0334489
Orphanet 58 ORPHA677
UMLS 71 C0334489

Summaries for Pancreatoblastoma

MalaCards based summary : Pancreatoblastoma is related to acinar cell carcinoma and adrenal neuroblastoma, and has symptoms including abdominal pain, pruritus and icterus. An important gene associated with Pancreatoblastoma is AFP (Alpha Fetoprotein), and among its related pathways/superpathways are Relaxin signaling pathway and Cell adhesion molecules. Affiliated tissues include pancreas, liver and lymph node, and related phenotypes are vomiting and abdominal pain

Wikipedia : 74 Pancreatoblastoma is a rare type of pancreatic cancer.It occurs mainly in childhood and has a relatively... more...

Related Diseases for Pancreatoblastoma

Diseases related to Pancreatoblastoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 291)
# Related Disease Score Top Affiliating Genes
1 acinar cell carcinoma 30.3 SERPINA3 SERPINA1 CHGA AFP
2 adrenal neuroblastoma 30.2 SYP CHGA
3 ectopic cushing syndrome 30.2 SST CHGA
4 neuroendocrine carcinoma 29.9 SYP SST MUC1 CHGA
5 neuroendocrine tumor 29.7 SYP SST NTS CHGA
6 pancreatic acinar cell adenocarcinoma 29.6 SYP SERPINA3 CHGA AFP
7 pilomatrixoma 29.3 MUC1 KRT15 KRT14
8 rare tumor 10.5
9 thymus sarcomatoid carcinoma 10.4 SYP AFP
10 rare tumor of pancreas 10.4
11 pineal region teratoma 10.4 SYP AFP
12 polyembryoma 10.4 SERPINA1 AFP
13 adult teratoma 10.3 CHGA AFP
14 primary hepatic neuroendocrine carcinoma 10.3 SYP CHGA
15 auditory system benign neoplasm 10.3 SYP CHGA
16 ovarian germ cell teratoma 10.3 SYP AFP
17 small cell carcinoma of the bladder 10.3 SYP CHGA
18 auditory system cancer 10.3 SYP CHGA
19 ovarian mixed germ cell neoplasm 10.3 SERPINA1 AFP
20 pancreatic serous cystadenocarcinoma 10.3 SYP CHGA
21 cauda equina neoplasm 10.3 SYP CHGA
22 appendix carcinoid tumor 10.3 SYP CHGA
23 vaginal tubulovillous adenoma 10.3 SYP CHGA
24 mixed ductal-endocrine carcinoma 10.3 SYP CHGA
25 laryngeal small cell carcinoma 10.3 SYP CHGA
26 gallbladder small cell carcinoma 10.3 SYP CHGA
27 anal neuroendocrine tumor 10.3 SYP SST
28 ureter small cell carcinoma 10.3 SYP CHGA
29 papillary thymic adenocarcinoma 10.3 SYP CHGA
30 urinary bladder small cell neuroendocrine carcinoma 10.3 SYP CHGA
31 gastric small cell carcinoma 10.3 SYP CHGA
32 breast papillary carcinoma 10.3 SYP CHGA
33 extracranial neuroblastoma 10.3 SYP CHGA
34 sensory organ benign neoplasm 10.3 SYP CHGA
35 cervical neuroblastoma 10.3 SYP CHGA
36 ovarian large-cell neuroendocrine carcinoma 10.3 SYP CHGA
37 sclerosing hepatic carcinoma 10.3 CHGA AFP
38 appendix adenocarcinoma 10.3 SYP CHGA
39 intrahepatic biliary papillomatosis 10.3 MUC1 AFP
40 subependymoma 10.3 SYP CHGA
41 pineocytoma 10.3 SYP CHGA
42 cutaneous ganglioneuroma 10.3 SYP CHGA
43 cervix small cell carcinoma 10.3 SYP CHGA
44 sinonasal undifferentiated carcinoma 10.3 SYP MUC1
45 papillary tumor of the pineal region 10.3 SYP MUC1
46 bile duct cystadenocarcinoma 10.3 MUC1 AFP
47 bile duct mucinous adenocarcinoma 10.3 SYP MUC1
48 beckwith-wiedemann syndrome 10.3
49 colonic lymphangioma 10.3 MUC1 AFP
50 glomangiomatosis 10.3 SYP CHGA

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to Pancreatoblastoma

Symptoms & Phenotypes for Pancreatoblastoma

Human phenotypes related to Pancreatoblastoma:

58 31 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002013
2 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
3 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
4 diarrhea 58 31 frequent (33%) Frequent (79-30%) HP:0002014
5 abdominal distention 58 31 frequent (33%) Frequent (79-30%) HP:0003270
6 pancreatic calcification 58 31 frequent (33%) Frequent (79-30%) HP:0005213
7 elevated maternal serum alpha-fetoprotein 58 31 frequent (33%) Frequent (79-30%) HP:0005984
8 jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0000952
9 abnormality of the lymph nodes 58 31 occasional (7.5%) Occasional (29-5%) HP:0002733

UMLS symptoms related to Pancreatoblastoma:


abdominal pain, pruritus, icterus

Drugs & Therapeutics for Pancreatoblastoma

Search Clinical Trials , NIH Clinical Center for Pancreatoblastoma

Cochrane evidence based reviews: pancreatoblastoma

Genetic Tests for Pancreatoblastoma

Anatomical Context for Pancreatoblastoma

MalaCards organs/tissues related to Pancreatoblastoma:

40
Pancreas, Liver, Lymph Node

Publications for Pancreatoblastoma

Articles related to Pancreatoblastoma:

(show top 50) (show all 264)
# Title Authors PMID Year
1
Medulloblastoma and familial adenomatous polyposis: Good prognosis and good quality of life in the long-term? 61
33459525 2021
2
Successful treatment of metastatic pancreatoblastoma in an adult with autologous hematopoietic cell transplant. 61
33199137 2021
3
Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review. 61
33323880 2020
4
A Rare Case of Pancreatoblastoma with Intracranial Seeding. 61
32622062 2020
5
Pancreatoblastoma: A rare indication for liver transplantation in children. 61
32736920 2020
6
Pancreatectomies for pediatric pancreatic tumors: A single institute experience from 2007 to 2018. 61
31575410 2020
7
Malignant epithelial/exocrine tumors of the pancreas. 61
33179623 2020
8
Pancreatoblastoma: A Case Report. 61
33431369 2020
9
Differential diagnosis of pancreatoblastoma (PB) and solid pseudopapillary neoplasms (SPNs) in children by CT and MR imaging. 61
32997172 2020
10
Adult pancreatoblastoma: clinical features and Imaging findings. 61
32647222 2020
11
Rare case of adult pancreatoblastoma. 61
32265211 2020
12
Metastatic adult pancreatoblastoma: Multimodal treatment and molecular characterization of a very rare disease. 61
32156527 2020
13
Treatment patterns and outcomes for pancreatic tumors in children: an analysis of the National Cancer Database. 61
31989243 2020
14
Differential diagnosis of solid pancreatic masses. 61
31994370 2020
15
Living donor liver transplantation with total pancreatectomy and portal vein homograft replacement in a pediatric patient with advanced pancreatoblastoma. 61
32181434 2020
16
[Pancreatoblastoma metachronously found in the stomach and in the pancreas: report of a case]. 61
31914546 2020
17
Adult Pancreatoblastoma With Liver Metastasis Diagnosed by 18F-FDG PET/CT and 2 Years' Postoperative Follow-up. 61
31274558 2020
18
A Rare Case of Pancreatoblastoma in a Pediatric Patient. 61
32015939 2020
19
Non-Ductal Tumors of the Pancreas. 61
32987393 2020
20
Tissue variations of mosaic genome-wide paternal uniparental disomy and phenotype of multi-syndromal congenital hyperinsulinism. 61
30797057 2020
21
Pancreatic Neoplasms With Acinar Differentiation: A Review of Pathologic and Molecular Features. 61
31869246 2019
22
Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center. 61
31338582 2019
23
Pancreatoblastoma: Cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics. 61
31581358 2019
24
Mosaic paternal haploidy in a patient with pancreatoblastoma and Beckwith-Wiedemann spectrum. 61
31231953 2019
25
The Italian Rare Pancreatic Exocrine Cancer Initiative. 61
30967031 2019
26
[Pancreatoblastoma in a pediatric patient: a case report]. 61
31688859 2019
27
Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors. 61
30785015 2019
28
FDG-PET CT in the evaluation of primary and secondary pancreatic malignancies. 61
29750397 2018
29
Pancreatoblastoma: an Atypical Presentation and a Literature Review. 61
28194556 2018
30
Adult pancreatoblastoma: A case report and clinicopathological review of the literature. 61
29753278 2018
31
Molecular Diagnostics in the Neoplasms of the Pancreas, Liver, Gallbladder, and Extrahepatic Biliary Tract: 2018 Update. 61
29776636 2018
32
Pancreatoblastoma: an unusual diagnosis in an adult patient. 61
29285688 2018
33
A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children. 61
28693851 2018
34
Pancreatectomies for pancreatic neoplasms in pediatric and adolescent age: A single institution experience. 61
29277262 2018
35
Integrated Molecular Characterization of the Lethal Pediatric Cancer Pancreatoblastoma. 61
29233928 2018
36
Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant. 61
29235221 2018
37
Identification and characterization of a novel adenomatous polyposis coli mutation in adult pancreatoblastoma. 61
29535845 2018
38
Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases. 61
30174723 2018
39
[Clinicopathologic characteristics of pancreatoblastoma]. 61
29224282 2017
40
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors. 61
29259370 2017
41
Pathologic classification of "pancreatic cancers": current concepts and challenges. 61
29307199 2017
42
Pancreatoblastoma With Metastatic Retroperitoneal Lymph Node and PET/CT. 61
28872552 2017
43
Genetic basis of a common tumor origin in the development of pancreatic mixed acinar-neuroendocrine-ductal carcinoma: A case report. 61
29085438 2017
44
Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report. 61
28538503 2017
45
Pancreaticoduodenectomy for pediatric and adolescent pancreatic malignancy: A single-center retrospective analysis. 61
27894759 2017
46
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. 61
28082787 2017
47
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. 61
25110242 2017
48
Fine needle aspiration cytology diagnosis of a pancreatoblastoma in an infant: case report with a summary of prior published cases. 61
27038102 2016
49
Acinar neoplasms of the pancreas-A summary of 25 years of research. 61
27320062 2016
50
Pancreatic surgery for tumors in children and adolescents. 61
27364750 2016

Variations for Pancreatoblastoma

Expression for Pancreatoblastoma

Search GEO for disease gene expression data for Pancreatoblastoma.

Pathways for Pancreatoblastoma

Pathways related to Pancreatoblastoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.85 SST KRT15 KRT14 KRT10
2 11.61 CLDN7 CLDN5 CLDN3
3
Show member pathways
11.48 CLDN7 CLDN5 CLDN3
4 10.78 KRT15 KRT14 KRT10

GO Terms for Pancreatoblastoma

Cellular components related to Pancreatoblastoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.8 AFP CHGA KRT10 MUC1 SERPINA1 SERPINA3
2 extracellular region GO:0005576 9.76 AFP CHGA KRT10 MUC1 NTS SERPINA1
3 bicellular tight junction GO:0005923 9.5 CLDN7 CLDN5 CLDN3
4 tight junction GO:0070160 9.37 CLDN5 CLDN3
5 lateral plasma membrane GO:0016328 9.13 CLDN7 CLDN5 CLDN3
6 apicolateral plasma membrane GO:0016327 8.8 CLDN3 CLDN5 CLDN7

Biological processes related to Pancreatoblastoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 response to ethanol GO:0045471 9.5 CLDN7 CLDN5 CLDN3
2 cornification GO:0070268 9.43 KRT15 KRT14 KRT10
3 positive regulation of bicellular tight junction assembly GO:1903348 9.16 CLDN5 CLDN3
4 bicellular tight junction assembly GO:0070830 9.13 CLDN7 CLDN5 CLDN3
5 calcium-independent cell-cell adhesion via plasma membrane cell-adhesion molecules GO:0016338 8.8 CLDN3 CLDN5 CLDN7

Molecular functions related to Pancreatoblastoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 9.1 KRT15 KRT14 KRT10 CLDN7 CLDN5 CLDN3

Sources for Pancreatoblastoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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