MCID: PNC019
MIFTS: 39

Pancreatoblastoma

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

Aliases & Classifications for Pancreatoblastoma

MalaCards integrated aliases for Pancreatoblastoma:

Name: Pancreatoblastoma 12 74 52 58 43 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 43 C537162
NCIt 49 C4265
SNOMED-CT 67 53618008
MESH via Orphanet 44 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 ectopic cushing syndrome and neuroendocrine carcinoma, and has symptoms including pruritus, abdominal pain and icterus. An important gene associated with Pancreatoblastoma is AFP (Alpha Fetoprotein), and among its related pathways/superpathways are Cell junction organization and Blood-Brain Barrier and Immune Cell Transmigration: VCAM-1/CD106 Signaling. Affiliated tissues include pancreas, liver and lymph node, and related phenotypes are vomiting and weight loss

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 299)
# Related Disease Score Top Affiliating Genes
1 ectopic cushing syndrome 30.7 SYP SST
2 neuroendocrine carcinoma 30.4 SYP SST MUC1 CHGA
3 neuroendocrine tumor 30.1 SYP SST NTS CHGA
4 pancreatic acinar cell adenocarcinoma 29.9 SYP SERPINA3 CHGA AFP
5 hepatoblastoma 29.7 SERPINA3 CTNNB1 AFP
6 pilomatrixoma 29.5 MUC1 KRT14 CTNNB1
7 acinar cell carcinoma 29.3 SYP SERPINA3 SERPINA1 CTNNB1 CHGA AFP
8 adenoma 29.3 SYP SST MUC1 CTNNB1 CHGA
9 wilms tumor 1 29.2 SYP SERPINA3 CTNNB1 AFP
10 rare tumor 10.7
11 polyembryoma 10.5 SERPINA1 AFP
12 pineal region teratoma 10.5 SYP AFP
13 thymus sarcomatoid carcinoma 10.5 SYP AFP
14 rare tumor of pancreas 10.5
15 breast neuroendocrine neoplasm 10.5 SYP CHGA
16 primary hepatic neuroendocrine carcinoma 10.5 SYP CHGA
17 beckwith-wiedemann syndrome 10.5
18 small cell carcinoma of the bladder 10.5 SYP CHGA
19 cervical large cell neuroendocrine carcinoma 10.5 SYP CHGA
20 auditory system benign neoplasm 10.5 SYP CHGA
21 appendix carcinoid tumor 10.5 SYP CHGA
22 auditory system cancer 10.5 SYP CHGA
23 mixed ductal-endocrine carcinoma 10.5 SYP CHGA
24 anal neuroendocrine tumor 10.5 SYP SST
25 pancreatic serous cystadenocarcinoma 10.5 SYP CHGA
26 cauda equina neoplasm 10.5 SYP CHGA
27 vaginal tubulovillous adenoma 10.5 SYP CHGA
28 adult teratoma 10.5 CHGA AFP
29 urinary bladder small cell neuroendocrine carcinoma 10.5 SYP CHGA
30 ureter small cell carcinoma 10.5 SYP CHGA
31 breast papillary carcinoma 10.5 SYP CHGA
32 intrahepatic biliary papillomatosis 10.5 MUC1 AFP
33 laryngeal neuroendocrine tumor 10.5 SYP CHGA
34 cystadenofibroma 10.5 MUC1 AFP
35 ovarian mixed germ cell neoplasm 10.5 SERPINA1 AFP
36 laryngeal small cell carcinoma 10.5 SYP CHGA
37 gallbladder small cell carcinoma 10.5 SYP CHGA
38 gastric small cell carcinoma 10.5 SYP CHGA
39 esophageal adenoid cystic carcinoma 10.5 SYP CHGA
40 sinonasal undifferentiated carcinoma 10.5 SYP MUC1
41 sensory organ benign neoplasm 10.5 SYP CHGA
42 papillary tumor of the pineal region 10.5 SYP MUC1
43 bile duct mucinous adenocarcinoma 10.5 SYP MUC1
44 subependymoma 10.5 SYP CHGA
45 ovarian germ cell teratoma 10.5 SYP AFP
46 pineocytoma 10.5 SYP CHGA
47 ovarian large-cell neuroendocrine carcinoma 10.5 SYP CHGA
48 appendix adenocarcinoma 10.5 SYP CHGA
49 bile duct cystadenocarcinoma 10.5 MUC1 AFP
50 mucinous cystadenofibroma 10.5 SERPINA1 MUC1

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 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
3 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
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:


pruritus, abdominal pain, 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, Brain

Publications for Pancreatoblastoma

Articles related to Pancreatoblastoma:

(show top 50) (show all 255)
# Title Authors PMID Year
1
Rare case of adult pancreatoblastoma. 61
32265211 2020
2
Metastatic adult pancreatoblastoma: Multimodal treatment and molecular characterization of a very rare disease. 61
32156527 2020
3
Differential diagnosis of solid pancreatic masses. 61
31994370 2020
4
Treatment patterns and outcomes for pancreatic tumors in children: an analysis of the National Cancer Database. 61
31989243 2020
5
Living donor liver transplantation with total pancreatectomy and portal vein homograft replacement in a pediatric patient with advanced pancreatoblastoma. 61
32181434 2020
6
Adult Pancreatoblastoma With Liver Metastasis Diagnosed by 18F-FDG PET/CT and 2 Years' Postoperative Follow-up. 61
31274558 2020
7
[Pancreatoblastoma metachronously found in the stomach and in the pancreas: report of a case]. 61
31914546 2020
8
A Rare Case of Pancreatoblastoma in a Pediatric Patient. 61
32015939 2020
9
Tissue variations of mosaic genome-wide paternal uniparental disomy and phenotype of multi-syndromal congenital hyperinsulinism. 61
30797057 2020
10
Pancreatic Neoplasms With Acinar Differentiation: A Review of Pathologic and Molecular Features. 61
31869246 2019
11
Pancreatoblastoma: Cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics. 61
31581358 2019
12
Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center. 61
31338582 2019
13
Pancreatectomies for Pediatric Pancreatic Tumors: A Single Institute Experience from 2007 to 2018. 61
31575410 2019
14
Mosaic paternal haploidy in a patient with pancreatoblastoma and Beckwith-Wiedemann spectrum. 61
31231953 2019
15
The Italian Rare Pancreatic Exocrine Cancer Initiative. 61
30967031 2019
16
[Pancreatoblastoma in a pediatric patient: a case report]. 61
31688859 2019
17
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
18
FDG-PET CT in the evaluation of primary and secondary pancreatic malignancies. 61
29750397 2018
19
Pancreatoblastoma: an Atypical Presentation and a Literature Review. 61
28194556 2018
20
Adult pancreatoblastoma: A case report and clinicopathological review of the literature. 61
29753278 2018
21
Molecular Diagnostics in the Neoplasms of the Pancreas, Liver, Gallbladder, and Extrahepatic Biliary Tract: 2018 Update. 61
29776636 2018
22
Pancreatoblastoma: an unusual diagnosis in an adult patient. 61
29285688 2018
23
A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children. 61
28693851 2018
24
Pancreatectomies for pancreatic neoplasms in pediatric and adolescent age: A single institution experience. 61
29277262 2018
25
Identification and characterization of a novel adenomatous polyposis coli mutation in adult pancreatoblastoma. 61
29535845 2018
26
Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant. 61
29235221 2018
27
Integrated Molecular Characterization of the Lethal Pediatric Cancer Pancreatoblastoma. 61
29233928 2018
28
Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases. 61
30174723 2018
29
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors. 61
29259370 2017
30
Pathologic classification of "pancreatic cancers": current concepts and challenges. 61
29307199 2017
31
[Clinicopathologic characteristics of pancreatoblastoma]. 61
29224282 2017
32
Pancreatoblastoma With Metastatic Retroperitoneal Lymph Node and PET/CT. 61
28872552 2017
33
Genetic basis of a common tumor origin in the development of pancreatic mixed acinar-neuroendocrine-ductal carcinoma: A case report. 61
29085438 2017
34
The functional assay of a variant unknown significance of APC gene derived from an adult pancreatoblastoma patient. 61
32120693 2017
35
Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report. 61
28538503 2017
36
Pancreaticoduodenectomy for pediatric and adolescent pancreatic malignancy: A single-center retrospective analysis. 61
27894759 2017
37
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. 61
28082787 2017
38
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. 61
25110242 2017
39
Fine needle aspiration cytology diagnosis of a pancreatoblastoma in an infant: case report with a summary of prior published cases. 61
27038102 2016
40
Acinar neoplasms of the pancreas-A summary of 25 years of research. 61
27320062 2016
41
Pancreatic surgery for tumors in children and adolescents. 61
27364750 2016
42
Adult pancreatoblastoma - Case report and review of literature. 61
27761191 2016
43
Surgical and molecular pathology of pancreatic neoplasms. 61
27267993 2016
44
Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. 61
27046982 2016
45
Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report. 61
27134883 2016
46
Multi-Visceral Transplantation in a 21-Year-Old Man with Prior Pancreatoblastoma. 61
27721967 2016
47
[Report of 14 cases with pancreatoblastoma]. 61
26791924 2016
48
Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt. 61
26881602 2015
49
Pancreatic tumours in children: diagnosis, treatment and outcome. 61
26174862 2015
50
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. 61
26056794 2015

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
12.33 CTNNB1 CLDN7 CLDN5 CLDN3
2
Show member pathways
12.25 CTNNB1 CLDN7 CLDN5 CLDN3
3
Show member pathways
12.01 CTNNB1 CLDN7 CLDN5 CLDN3
4 11.59 MUC1 CTNNB1 CLDN3
5
Show member pathways
11.41 CTNNB1 CLDN7 CLDN5 CLDN3
6 10.37 SST CTNNB1
7 10.3 CTNNB1 CLDN5 CLDN3

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 9.87 SST SERPINA3 SERPINA1 MUC1 KRT10 CHGA
2 extracellular region GO:0005576 9.86 SST SERPINA3 SERPINA1 NTS MUC1 KRT10
3 bicellular tight junction GO:0005923 9.46 CTNNB1 CLDN7 CLDN5 CLDN3
4 tight junction GO:0070160 9.37 CLDN5 CLDN3
5 apicolateral plasma membrane GO:0016327 9.13 CLDN7 CLDN5 CLDN3
6 lateral plasma membrane GO:0016328 8.92 CTNNB1 CLDN7 CLDN5 CLDN3

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

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

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

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 9.02 KRT14 KRT10 CLDN7 CLDN5 CLDN3

Sources for Pancreatoblastoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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