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 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 (CAMs). Affiliated tissues include pancreas, liver and breast, 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 281)
# Related Disease Score Top Affiliating Genes
1 ectopic cushing syndrome 30.7 SYP SST
2 neuroendocrine carcinoma 30.3 SYP SST MUC1 CHGA
3 acinar cell carcinoma 30.3 SYP SERPINA3 SERPINA1 CHGA AFP
4 neuroendocrine tumor 30.0 SYP SST NTS CHGA
5 pancreatic acinar cell adenocarcinoma 29.9 SYP SERPINA3 CHGA AFP
6 pilomatrixoma 29.4 MUC1 KRT15 KRT14
7 rare tumor 10.7
8 thymus sarcomatoid carcinoma 10.5 SYP AFP
9 polyembryoma 10.5 SERPINA1 AFP
10 mature teratoma of the ovary 10.5 SYP AFP
11 rare tumor of pancreas 10.5
12 ovarian cystic teratoma 10.5 SYP AFP
13 ovarian mixed germ cell neoplasm 10.5 SERPINA1 AFP
14 ureter small cell carcinoma 10.5 SYP CHGA
15 primary hepatic neuroendocrine carcinoma 10.5 SYP CHGA
16 ovarian large-cell neuroendocrine carcinoma 10.5 SYP CHGA
17 beckwith-wiedemann syndrome 10.5
18 pancreas lymphoma 10.5 SYP CHGA
19 ovarian germ cell teratoma 10.5 SYP AFP
20 small cell carcinoma of the bladder 10.5 SYP CHGA
21 urinary bladder small cell neuroendocrine carcinoma 10.5 SYP CHGA
22 laryngeal neuroendocrine tumor 10.5 SYP CHGA
23 appendix carcinoid tumor 10.5 SYP CHGA
24 auditory system cancer 10.5 SYP CHGA
25 mixed ductal-endocrine carcinoma 10.5 SYP CHGA
26 thymus small cell carcinoma 10.5 SYP CHGA
27 anal neuroendocrine tumor 10.5 SYP SST
28 papillary thymic adenocarcinoma 10.5 SYP CHGA
29 breast papillary carcinoma 10.5 SYP CHGA
30 cauda equina neoplasm 10.5 SYP CHGA
31 adult teratoma 10.5 CHGA AFP
32 vaginal tubulovillous adenoma 10.5 SYP CHGA
33 cystadenofibroma 10.5 MUC1 AFP
34 vaginal adenoma 10.5 SYP CHGA
35 nodular ganglioneuroblastoma 10.5 SYP CHGA
36 rectum neuroendocrine neoplasm 10.5 SYP SST
37 intrahepatic biliary papillomatosis 10.5 MUC1 AFP
38 esophageal adenoid cystic carcinoma 10.5 SYP CHGA
39 gastric small cell carcinoma 10.5 SYP CHGA
40 vaginal benign neoplasm 10.5 SYP CHGA
41 sensory organ benign neoplasm 10.5 SYP CHGA
42 cervical neuroblastoma 10.5 SYP CHGA
43 subependymoma 10.5 SYP CHGA
44 pineocytoma 10.5 SYP CHGA
45 sinonasal undifferentiated carcinoma 10.5 SYP MUC1
46 papillary tumor of the pineal region 10.5 SYP MUC1
47 bile duct cystadenocarcinoma 10.5 MUC1 AFP
48 pulmonary large cell neuroendocrine carcinoma 10.5 SYP CHGA
49 colonic lymphangioma 10.5 MUC1 AFP
50 bile duct mucinous adenocarcinoma 10.5 SYP 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 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, Breast, Brain, Lung, Lymph Node, Thymus

Publications for Pancreatoblastoma

Articles related to Pancreatoblastoma:

(show top 50) (show all 251)
# Title Authors PMID Year
1
Tissue variations of mosaic genome-wide paternal uniparental disomy and phenotype of multi-syndromal congenital hyperinsulinism. 61
30797057 2020
2
Differential diagnosis of solid pancreatic masses. 61
31994370 2020
3
Treatment patterns and outcomes for pancreatic tumors in children: an analysis of the National Cancer Database. 61
31989243 2020
4
A Rare Case of Pancreatoblastoma in a Pediatric Patient. 61
32015939 2020
5
[Pancreatoblastoma metachronously found in the stomach and in the pancreas: report of a case]. 61
31914546 2020
6
Adult Pancreatoblastoma With Liver Metastasis Diagnosed by 18F-FDG PET/CT and 2 Years' Postoperative Follow-up. 61
31274558 2020
7
Pancreatic Neoplasms With Acinar Differentiation: A Review of Pathologic and Molecular Features. 61
31869246 2019
8
Pancreatoblastoma: Cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics. 61
31581358 2019
9
Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center. 61
31338582 2019
10
Pancreatectomies for Pediatric Pancreatic Tumors: A Single Institute Experience from 2007 to 2018. 61
31575410 2019
11
Mosaic paternal haploidy in a patient with pancreatoblastoma and Beckwith-Wiedemann spectrum. 61
31231953 2019
12
The Italian Rare Pancreatic Exocrine Cancer Initiative. 61
30967031 2019
13
[Pancreatoblastoma in a pediatric patient: a case report]. 61
31688859 2019
14
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
15
FDG-PET CT in the evaluation of primary and secondary pancreatic malignancies. 61
29750397 2018
16
Pancreatoblastoma: an Atypical Presentation and a Literature Review. 61
28194556 2018
17
Adult pancreatoblastoma: A case report and clinicopathological review of the literature. 61
29753278 2018
18
Molecular Diagnostics in the Neoplasms of the Pancreas, Liver, Gallbladder, and Extrahepatic Biliary Tract: 2018 Update. 61
29776636 2018
19
Pancreatoblastoma: an unusual diagnosis in an adult patient. 61
29285688 2018
20
A population-based analysis of a rare oncologic entity: Malignant pancreatic tumors in children. 61
28693851 2018
21
Pancreatectomies for pancreatic neoplasms in pediatric and adolescent age: A single institution experience. 61
29277262 2018
22
Identification and characterization of a novel adenomatous polyposis coli mutation in adult pancreatoblastoma. 61
29535845 2018
23
Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant. 61
29235221 2018
24
Integrated Molecular Characterization of the Lethal Pediatric Cancer Pancreatoblastoma. 61
29233928 2018
25
Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases. 61
30174723 2018
26
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors. 61
29259370 2017
27
Pathologic classification of "pancreatic cancers": current concepts and challenges. 61
29307199 2017
28
[Clinicopathologic characteristics of pancreatoblastoma]. 61
29224282 2017
29
Pancreatoblastoma With Metastatic Retroperitoneal Lymph Node and PET/CT. 61
28872552 2017
30
Genetic basis of a common tumor origin in the development of pancreatic mixed acinar-neuroendocrine-ductal carcinoma: A case report. 61
29085438 2017
31
Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report. 61
28538503 2017
32
Pancreaticoduodenectomy for pediatric and adolescent pancreatic malignancy: A single-center retrospective analysis. 61
27894759 2017
33
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. 61
28082787 2017
34
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. 61
25110242 2017
35
Fine needle aspiration cytology diagnosis of a pancreatoblastoma in an infant: case report with a summary of prior published cases. 61
27038102 2016
36
Acinar neoplasms of the pancreas-A summary of 25 years of research. 61
27320062 2016
37
Pancreatic surgery for tumors in children and adolescents. 61
27364750 2016
38
Adult pancreatoblastoma - Case report and review of literature. 61
27761191 2016
39
Surgical and molecular pathology of pancreatic neoplasms. 61
27267993 2016
40
Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. 61
27046982 2016
41
Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report. 61
27134883 2016
42
Multi-Visceral Transplantation in a 21-Year-Old Man with Prior Pancreatoblastoma. 61
27721967 2016
43
[Report of 14 cases with pancreatoblastoma]. 61
26791924 2016
44
Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt. 61
26881602 2015
45
Pancreatic tumours in children: diagnosis, treatment and outcome. 61
26174862 2015
46
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. 61
26056794 2015
47
Clinicopathological review of pancreatoblastoma in adults. 61
26312218 2015
48
Uncommon presentations of common pancreatic neoplasms: a pictorial essay. 61
25772002 2015
49
Successful emergency combined therapy with partial splenic arterial embolization and endoscopic injection therapy against a bleeding duodenal varix in a child. 61
25851961 2015
50
Adult pancreatoblastoma: A case report and review of the literature. 61
26137059 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
11.85 SST KRT15 KRT14 KRT10
2 11.6 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 SST SERPINA3 SERPINA1 MUC1 KRT10 CHGA
2 extracellular region GO:0005576 9.76 SST SERPINA3 SERPINA1 NTS MUC1 KRT10
3 bicellular tight junction GO:0005923 9.5 CLDN7 CLDN5 CLDN3
4 lateral plasma membrane GO:0016328 9.13 CLDN7 CLDN5 CLDN3
5 apicolateral plasma membrane GO:0016327 8.8 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 cornification GO:0070268 9.33 KRT15 KRT14 KRT10
3 positive regulation of bicellular tight junction assembly GO:1903348 8.96 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 8.92 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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