MCID: PNC019
MIFTS: 48

Pancreatoblastoma malady

Rare diseases, Gastrointestinal diseases, Cancer diseases, Endocrine diseases categories
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Summaries for Pancreatoblastoma

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65Wikipedia, 33MalaCards
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Wikipedia:65 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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8Disease Ontology, 43NIH Rare Diseases, 49Orphanet, 62UMLS, 58SNOMED-CT, 40NCIt, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

49
pancreatoblastoma:
Prevalence: <1/1000000; Age of onset: Childhood


Aliases & Descriptions:

pancreatoblastoma 8 43 49 62


External Ids:

Disease Ontology8 DOID:6823
NCIt40 C4265
MeSH35 C537162
MESH via Orphanet36 C537162
SNOMED-CT58 189814006, 53618008
ICD10 via Orphanet26 C25.1
SNOMED-CT via Orphanet59 53618008
UMLS via Orphanet63 C0334489

Related Diseases for Pancreatoblastoma

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17GeneCards, 18GeneDecks
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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:
42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Pancreatoblastoma

Search NIH Clinical Center for Pancreatoblastoma

Genetic Tests for Pancreatoblastoma

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Anatomical Context for Pancreatoblastoma

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33MalaCards
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MalaCards organs/tissues related to Pancreatoblastoma:

33
Pancreas, Liver, Brain

Animal Models for Pancreatoblastoma or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Pancreatoblastoma:

37
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:
52PubMed
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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, 15Gene 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:
50PathCards, 38NCBI BioSystems Database, 55Reactome, 12EMD Millipore, 5Cell Signaling Technology, 54R&D Systems, 30KEGG, 60Thomson Reuters, 53QIAGEN
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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 NetPath38
9.2CTNNB1, APC, SMAD4
119.2CTNNB1, APC, SMAD4
12
Show member pathways
DNA damage response (only ATM dependent)38
Wnt Signaling Pathway and Pluripotency38
9.2CTNNB1, APC, SMAD4
139.2SMAD4, APC, CTNNB1
14
Show member pathways
Signal transduction PTEN pathway60
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 motility60
Cytoskeleton remodeling Integrin outside in signaling60
Cytoskeleton remodeling Regulation of actin cytoskeleton by Rho GTPases60
Cell adhesion Tight junctions60
Development MAG dependent inhibition of neurite outgrowth60
8.1CTNNB1, CLDN5, CLDN7, CLDN3

Compounds for genes affiliated with Pancreatoblastoma

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Sources:
45Novoseek, 51PharmGKB, 29IUPHAR, 3BitterDB, 11DrugBank, 24HMDB, 61Tocris Bioscience
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Compounds related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show top 50)    (show all 64)
idCompoundScoreTop Affiliating Genes
1smcc4510.2CHGA, SYP
2111in-pentetreotide4510.2CHGA, SYP
3s 1004510.2SYP, CHGA
4lithium chloride4510.1CHGA, CTNNB1
51-hydroxyanthraquinone4510.0CTNNB1, APC
6methylazoxymethanol acetate4510.0CTNNB1, APC
7samp4510.0CTNNB1, APC
81,2-dimethylhydrazine4510.0APC, CTNNB1
9azoxymethane4510.0CTNNB1, APC
10b 723459.9AFP, CHGA, SYP
112-amino-3-methylimidazo(4,5-f)quinoline459.9APC, CTNNB1
12silver459.9SYP, CHGA, AFP
13exisulind459.9CTNNB1, APC
14sodium bisulfite459.9APC, CTNNB1
15l-amino acid459.8SYP, CHGA
16gold459.8SYP, CHGA, AFP
17phip459.8CTNNB1, APC
18haloperidol45 51 29 3 1113.7CTNNB1, SYP, CHGA
19lithium45 51 24 1112.7CTNNB1, SYP, APC
20crcs459.7CTNNB1, APC, SMAD4
21acth459.7APC, CHGA, SYP
22tgf beta1459.6CTNNB1, AFP, SMAD4
23sucrose45 24 1111.6CTNNB1, SYP, CHGA
24chloramphenicol45 3 51 1112.5CHGA, AFP, SMAD4
25gnrh459.5SMAD4, CHGA, CTNNB1
26hydrogen45 2410.5AFP, APC, CHGA, SYP
27agar459.4SMAD4, AFP, CTNNB1
28epinephrine45 24 1111.4AFP, APC, CHGA
29guanine45 24 1111.4APC, CHGA, SYP, CTNNB1
30cisplatin45 51 61 1112.4AFP, CHGA, SYP, CTNNB1
31glutamine459.4AFP, APC, SYP, CTNNB1
32vitamin d459.4AFP, APC, CHGA, CTNNB1
33dopamine45 29 24 1112.3APC, CHGA, SYP, CTNNB1
34fatty acid459.3AFP, APC, CHGA, CTNNB1
35dexamethasone45 51 29 1112.3CTNNB1, SYP, CHGA, AFP
36cholesterol45 29 24 1112.2SYP, CHGA, CLDN3, AFP
37etoposide45 51 61 1112.2AFP, CHGA, SYP
38steroid459.1SMAD4, AFP, CHGA, SYP, CTNNB1
39glycogen45 2410.1AFP, APC, CHGA, SYP, CTNNB1
40progesterone45 29 61 24 1113.1AFP, APC, CHGA, SYP, CTNNB1
41threonine458.8CTNNB1, APC, AFP, SMAD4
42paraffin458.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
43testosterone45 61 24 1111.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
44estrogen458.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
45serine458.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
46vegf458.6SMAD4, AFP, CLDN3, CHGA, SYP, CTNNB1
47retinoic acid45 249.6AFP, APC, CHGA, SYP, KRT15, CTNNB1
48calcium45 51 24 1111.2AFP, APC, CLDN3, CLDN5, CHGA, SYP
49tyrosine458.2SMAD4, AFP, APC, CHGA, SYP, KRT15
50oligonucleotide457.6CTNNB1, SMAD4, AFP, APC, CLDN7, CLDN5

GO Terms for genes affiliated with Pancreatoblastoma

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Sources:
16Gene 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
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet