MCID: PNC019
MIFTS: 48

Pancreatoblastoma malady

Rare diseases, Gastrointestinal diseases, Cancer diseases, Endocrine diseases categories

Summaries for Pancreatoblastoma

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66Wikipedia, 34MalaCards
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Wikipedia:66 Pancreatoblastoma is a rare type of pancreatic cancer. more...

MalaCards: Pancreatoblastoma is related to acinar cell carcinoma and familial adenomatous polyposis. An important gene associated with Pancreatoblastoma is SYP (synaptophysin), and among its related pathways are Heart Development and TGF Beta Signaling Pathway. The compounds smcc and 111in-pentetreotide have been mentioned in the context of this disorder. Affiliated tissues include pancreas, liver and brain, and related mouse phenotypes are tumorigenesis and endocrine/exocrine gland.

Aliases & Classifications for Pancreatoblastoma

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9Disease Ontology, 44NIH Rare Diseases, 50Orphanet, 63UMLS, 59SNOMED-CT, 41NCIt, 36MeSH, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

50
pancreatoblastoma:
Prevalence: <1/1000000; Age of onset: Childhood


Aliases & Descriptions:

pancreatoblastoma 9 44 50 63


External Ids:

Disease Ontology9 DOID:6823
NCIt41 C4265
MeSH36 C537162
MESH via Orphanet37 C537162
SNOMED-CT59 189814006, 53618008
ICD10 via Orphanet27 C25.1
SNOMED-CT via Orphanet60 53618008
UMLS via Orphanet64 C0334489

Related Diseases for Pancreatoblastoma

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18GeneCards, 19GeneDecks
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Diseases related to Pancreatoblastoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 56)
idRelated DiseaseScoreTop Affiliating Genes
1acinar cell carcinoma30.4CTNNB1, CHGA, AFP
2familial adenomatous polyposis30.0APC, CTNNB1, SMAD4
3adenoma29.8SMAD4, APC, CLDN3, CHGA, CTNNB1, SYP
4adenocarcinoma29.6CLDN5, CLDN3, SMAD4, APC, CLDN7, CHGA
5pancreatitis10.5
6beckwith-wiedemann syndrome10.4
7sertoli cell tumor10.1SYP
8dysembryoplastic neuroepithelial tumor10.1SYP
9central neurocytoma10.1SYP, CHGA
10pancreatic islet cell tumors10.1SYP, CHGA
11pineocytoma10.1CHGA, SYP
12endocrine gland cancer10.1CHGA, SYP
13merkel cell carcinoma10.0SYP, CHGA
14ganglioglioma10.0CHGA, SYP
15oxyphilic adenoma10.0SYP, CLDN7
16malignant mixed mullerian tumor10.0CTNNB1, AFP
17lung cancer10.0CHGA, SYP
18ganglioneuroma10.0CHGA, SYP
19large cell carcinoma10.0CHGA, SYP
20pancreatic ductal carcinoma10.0SMAD4, CHGA
21neuroendocrine carcinoma10.0SYP, CHGA
22vipoma10.0CHGA, SMAD4
23neuroblastoma10.0
24endometrial carcinoma10.0CTNNB1, CHGA
25gardner syndrome10.0APC, CTNNB1
26intestinal neoplasm10.0APC, CTNNB1
27paraganglioma10.0SYP, CHGA
28cushing's syndrome10.0SYP, CHGA
29desmoid tumor10.0APC, CTNNB1
30turcot syndrome10.0CTNNB1, APC
31colorectal cancer, somatic10.0APC, CTNNB1
32juvenile polyposis syndrome10.0SMAD4, APC
33fibromatosis10.0CTNNB1, APC
34peutz-jeghers syndrome10.0APC, CTNNB1
35adrenocortical carcinoma10.0CHGA, SYP, SMAD4
36hepatocellular adenoma10.0AFP, CTNNB1, APC
37pituitary adenoma10.0APC, CHGA, SYP
38hepatoblastoma10.0AFP, CTNNB1, APC
39medulloblastoma10.0APC, SYP, CTNNB1
40papillary carcinoma10.0CLDN3, CHGA, SMAD4, SYP
41tubular adenocarcinoma10.0CHGA, AFP, SYP, CTNNB1
42prostate cancer10.0CTNNB1, CHGA, SMAD4
43pancreatic cancer9.9SMAD4, CHGA, CTNNB1, SYP
44ovarian cancer9.9CLDN3, CTNNB1, SMAD4
45adrenal adenoma9.9CTNNB1, SYP, APC, CHGA
46colon cancer9.9CTNNB1, SMAD4, CLDN3, APC
47colorectal cancer9.9CTNNB1, SMAD4, APC
48gastrinoma9.9CHGA, APC, SMAD4, SYP
49lung benign neoplasm9.9CHGA, APC, SMAD4, SYP
50retinoblastoma9.9SMAD4, APC, SYP

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to pancreatoblastoma

Symptoms for Pancreatoblastoma

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Drugs & Therapeutics for Pancreatoblastoma

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

Search CenterWatch for Pancreatoblastoma

Drug clinical trials:

Search ClinicalTrials for Pancreatoblastoma

Search NIH Clinical Center for Pancreatoblastoma

Search CenterWatch for Pancreatoblastoma

Genetic Tests for Pancreatoblastoma

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Anatomical Context for Pancreatoblastoma

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34MalaCards
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MalaCards organs/tissues related to Pancreatoblastoma:

34
Pancreas, Liver, Brain

Animal Models for Pancreatoblastoma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Pancreatoblastoma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.8CTNNB1, APC, AFP, SMAD4
2MP:00053798.6SMAD4, AFP, APC, CHGA, CTNNB1
3MP:00053898.6SMAD4, AFP, APC, CHGA, CTNNB1
4MP:00053768.3SMAD4, AFP, APC, CHGA, CTNNB1
5MP:00053858.3CTNNB1, CHGA, CLDN5, APC, SMAD4
6MP:00053678.2CTNNB1, CHGA, CLDN7, APC, SMAD4
7MP:00107687.2SMAD4, AFP, APC, CLDN7, CLDN5, CHGA

Publications for Pancreatoblastoma

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Sources:
53PubMed
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Articles related to Pancreatoblastoma:

(show top 50)    (show all 110)
idTitleAuthorsYear
1
CT-guided radiofrequency ablation following high-dose chemotherapy of a liver-metastasizing pancreatoblastoma with tumor thrombus in the portal vein. (23828484)
2013
2
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. (24081396)
2013
3
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. (23991735)
2013
4
Congenital pancreatoblastoma: report of an atypical case and review of the literature. (22278199)
2012
5
Management of pancreatoblastoma in children and young adults. (22525406)
2012
6
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. (22572137)
2012
7
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). (21696948)
2011
8
Pancreatoblastoma in an adult patient. A case report. (19287117)
2009
9
Imprint cytology of pancreatoblastoma: a case report and review of the literature. (19229998)
2009
10
Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. (19194274)
2009
11
Childhood pancreatoblastoma: clinical features and immunohistochemistry analysis. (18308466)
2008
12
Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. (18981556)
2008
13
Long-term survival of pancreatoblastoma in children. (18090935)
2007
14
Adrenocorticotropin-secreting pancreatoblastoma. (17642425)
2007
15
Pancreatoblastoma in an adult: case report and review of the literature. (16769539)
2006
16
Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature. (16080945)
2005
17
Fine-needle aspiration cytology of pancreatoblastoma in a young woman: report of a case and review of the literature. (16138370)
2005
18
Pancreatoblastoma in an adult woman: sonography, CT, and dynamic gadolinium-enhanced MRI features. (15728031)
2005
19
Diagnosis, treatment and outcome of pancreatoblastoma. (15256806)
2004
20
The fine-needle aspiration biopsy cytology of pancreatoblastoma. (15540188)
2004
21
Pancreatoblastoma (infantile pancreatic carcinoma)--a case report. (16295493)
2004
22
Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome. (12673632)
2003
23
Ampullary pancreatoblastoma in an elderly patient: a case report and review of the literature. (14567752)
2003
24
Significance of aberrant (cytoplasmic/nuclear) expression of beta-catenin in pancreatoblastoma. (12533831)
2003
25
Pancreatoblastoma. A case report with special emphasis on squamoid corpuscles with optically clear nuclei rich in biotin. (12920766)
2003
26
Pancreatoblastoma is associated with chromosome 11p loss of heterozygosity and IGF2 overexpression. (12116082)
2002
27
Fine-needle aspiration cytology of pancreatoblastoma. (11477717)
2001
28
Pancreatoblastoma. (11770246)
2001
29
Imaging features of pancreatoblastoma. (11486805)
2001
30
Pancreatoblastoma treated by delayed operation after effective chemotherapy. (11083448)
2000
31
Pancreatoblastoma in an adolescent girl: case report and review of 26 Japanese cases. (9641374)
1998
32
Pancreatoblastoma. (9396538)
1997
33
Pancreatoblastoma in children. Case report and review of the literature. (9007475)
1996
34
Pancreatoblastoma: a histochemical and immunohistochemical analysis. (8944621)
1996
35
Adult pancreatoblastoma: a case report and review of the literature. (8792711)
1996
36
Pancreatoblastoma: case report and review of treatment in the literature. (8699999)
1996
37
Cytogenetic and flow cytometric analysis of a pancreatoblastoma. (7889500)
1995
38
Adenocarcinoma of the pancreas in childhood (pancreatoblastoma): report of a case with good response to chemotherapy. (8804461)
1995
39
Pancreatoblastoma in an older adult. (7605173)
1995
40
Pancreatoblastoma: a case report. (7954063)
1994
41
Pancreatoblastoma and solid and cystic papillary tumor: two tumors related to pancreatic ontogeny. (7809509)
1994
42
Pancreatoblastoma with marked elevation of serum alpha-fetoprotein. An autopsy case report with immunocytochemical study. (1689089)
1990
43
Pancreatoblastoma: MR appearance. (2159494)
1990
44
Use of enzyme histochemistry in the diagnosis of pancreatoblastoma. (2553578)
1989
45
Recurrent pancreatoblastoma with inappropriate adrenocorticotrophic hormone secretion. (2852926)
1988
46
Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association. (2845376)
1988
47
Immunocytochemical markers of uncommon pancreatic tumors. Acinar cell carcinoma, pancreatoblastoma, and solid cystic (papillary-cystic) tumor. (3542187)
1987
48
Immunohistochemical study of pancreatoblastoma. (2449037)
1987
49
Pancreatoblastoma in Japan, with differential diagnosis from papillary cystic tumor (ductuloacinar adenoma) of the pancreas. (3554891)
1987
50
Pancreatoblastoma. A histochemical and ultrastructural analysis. (6141001)
1984

Variations for Pancreatoblastoma

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Expression for genes affiliated with Pancreatoblastoma

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pancreatoblastoma

Search GEO for disease gene expression data for Pancreatoblastoma.

Pathways for genes affiliated with Pancreatoblastoma

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Sources:
51PathCards, 39NCBI BioSystems Database, 56Reactome, 13EMD Millipore, 5Cell Signaling Technology, 55R&D Systems, 31KEGG, 61Thomson Reuters, 54QIAGEN
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Pathways related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show all 25)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6CTNNB1, SMAD4
29.6CTNNB1, SMAD4
39.6CTNNB1, SMAD4
49.6SMAD4, CTNNB1
59.6CTNNB1, APC
6
Show member pathways
9.6CTNNB1, APC
79.6CTNNB1, APC
89.5KRT15, APC
99.3CTNNB1, APC, AFP
10
Show member pathways
Wnt Signaling Pathway NetPath39
9.2CTNNB1, APC, SMAD4
119.2CTNNB1, APC, SMAD4
12
Show member pathways
DNA damage response (only ATM dependent)39
Wnt Signaling Pathway and Pluripotency39
9.2CTNNB1, APC, SMAD4
139.2SMAD4, APC, CTNNB1
14
Show member pathways
Signal transduction PTEN pathway61
9.2SMAD4, APC, CTNNB1
159.2SMAD4, APC, CTNNB1
168.9CTNNB1, APC, AFP, SMAD4
178.9CLDN3, CLDN5, CTNNB1
188.5CLDN5, CLDN7, CLDN3
198.5CLDN3, CLDN7, CLDN5
208.1CLDN3, CLDN7, CLDN5, CTNNB1
21
Show member pathways
8.1CLDN3, CLDN7, CLDN5, CTNNB1
22
Show member pathways
8.1CLDN3, CLDN7, CLDN5, CTNNB1
238.1CTNNB1, CLDN5, CLDN7, CLDN3
24
Show member pathways
8.1CTNNB1, CLDN5, CLDN7, CLDN3
25
Show member pathways
Cytoskeleton remodeling Fibronectin binding integrins in cell motility61
Cytoskeleton remodeling Integrin outside in signaling61
Cytoskeleton remodeling Regulation of actin cytoskeleton by Rho GTPases61
Cell adhesion Tight junctions61
Development MAG dependent inhibition of neurite outgrowth61
8.1CTNNB1, CLDN5, CLDN7, CLDN3

Compounds for genes affiliated with Pancreatoblastoma

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Sources:
46Novoseek, 52PharmGKB, 30IUPHAR, 3BitterDB, 12DrugBank, 25HMDB, 62Tocris Bioscience
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Compounds related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show top 50)    (show all 64)
idCompoundScoreTop Affiliating Genes
1smcc4610.2CHGA, SYP
2111in-pentetreotide4610.2CHGA, SYP
3s 1004610.2SYP, CHGA
4lithium chloride4610.1CHGA, CTNNB1
51-hydroxyanthraquinone4610.0CTNNB1, APC
6methylazoxymethanol acetate4610.0CTNNB1, APC
7samp4610.0CTNNB1, APC
81,2-dimethylhydrazine4610.0APC, CTNNB1
9azoxymethane4610.0CTNNB1, APC
10b 723469.9AFP, CHGA, SYP
112-amino-3-methylimidazo(4,5-f)quinoline469.9APC, CTNNB1
12silver469.9SYP, CHGA, AFP
13exisulind469.9CTNNB1, APC
14sodium bisulfite469.9APC, CTNNB1
15l-amino acid469.8SYP, CHGA
16gold469.8SYP, CHGA, AFP
17phip469.8CTNNB1, APC
18haloperidol46 52 30 3 1213.7CTNNB1, SYP, CHGA
19lithium46 52 25 1212.7CTNNB1, SYP, APC
20crcs469.7CTNNB1, APC, SMAD4
21acth469.7APC, CHGA, SYP
22tgf beta1469.6CTNNB1, AFP, SMAD4
23sucrose46 25 1211.6CTNNB1, SYP, CHGA
24chloramphenicol46 3 52 1212.5CHGA, AFP, SMAD4
25gnrh469.5SMAD4, CHGA, CTNNB1
26hydrogen46 2510.5AFP, APC, CHGA, SYP
27agar469.4SMAD4, AFP, CTNNB1
28epinephrine46 25 1211.4AFP, APC, CHGA
29guanine46 25 1211.4APC, CHGA, SYP, CTNNB1
30cisplatin46 52 62 1212.4AFP, CHGA, SYP, CTNNB1
31glutamine469.4AFP, APC, SYP, CTNNB1
32vitamin d469.4AFP, APC, CHGA, CTNNB1
33dopamine46 30 25 1212.3APC, CHGA, SYP, CTNNB1
34fatty acid469.3AFP, APC, CHGA, CTNNB1
35dexamethasone46 52 30 1212.3CTNNB1, SYP, CHGA, AFP
36cholesterol46 30 25 1212.2SYP, CHGA, CLDN3, AFP
37etoposide46 52 62 1212.2AFP, CHGA, SYP
38steroid469.1SMAD4, AFP, CHGA, SYP, CTNNB1
39glycogen46 2510.1AFP, APC, CHGA, SYP, CTNNB1
40progesterone46 30 62 25 1213.1AFP, APC, CHGA, SYP, CTNNB1
41threonine468.8CTNNB1, APC, AFP, SMAD4
42paraffin468.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
43testosterone46 62 25 1211.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
44estrogen468.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
45serine468.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
46vegf468.6SMAD4, AFP, CLDN3, CHGA, SYP, CTNNB1
47retinoic acid46 259.6AFP, APC, CHGA, SYP, KRT15, CTNNB1
48calcium46 52 25 1211.2AFP, APC, CLDN3, CLDN5, CHGA, SYP
49tyrosine468.2SMAD4, AFP, APC, CHGA, SYP, KRT15
50oligonucleotide467.6CTNNB1, SMAD4, AFP, APC, CLDN7, CLDN5

GO Terms for genes affiliated with Pancreatoblastoma

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Sources:
17Gene Ontology
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Cellular components related to Pancreatoblastoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1beta-catenin destruction complexGO:0308779.5APC, CTNNB1
2cell-cell adherens junctionGO:0059139.3CTNNB1, APC
3centrosomeGO:0058139.2CTNNB1, APC, SMAD4
4lateral plasma membraneGO:0163288.8CTNNB1, CLDN7, APC
5tight junctionGO:0059238.2CLDN5, CLDN7, CLDN3, APC

Biological processes related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1SMAD protein signal transductionGO:06039510.0SMAD4, AFP
2canonical Wnt signaling pathway involved in negative regulation of apoptotic processGO:0443369.8CTNNB1, APC
3gastrulation with mouth forming secondGO:0017029.8CTNNB1, SMAD4
4proximal/distal pattern formationGO:0099549.7CTNNB1, APC
5T cell differentiation in thymusGO:0330779.7APC, CTNNB1
6thymus developmentGO:0485389.6CTNNB1, APC
7cellular component disassembly involved in execution phase of apoptosisGO:0069219.5CTNNB1, APC
8branching involved in ureteric bud morphogenesisGO:0016589.3SMAD4, CTNNB1
9negative regulation of cell proliferationGO:0082859.2CTNNB1, APC, SMAD4
10calcium-independent cell-cell adhesionGO:0163388.7CLDN5, CLDN7, CLDN3

Molecular functions related to Pancreatoblastoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cadherin bindingGO:0452969.6CTNNB1, APC
2I-SMAD bindingGO:0704119.5CTNNB1, SMAD4
3R-SMAD bindingGO:0704129.3CTNNB1, SMAD4
4structural molecule activityGO:0051988.1CLDN3, CLDN7, CLDN5, CTNNB1
5identical protein bindingGO:0428027.9SYP, CLDN5, CLDN7, CLDN3, SMAD4

Products for genes affiliated with Pancreatoblastoma

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  • Antibodies
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Sources for Pancreatoblastoma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet