MCID: PNC019
MIFTS: 41

Pancreatoblastoma malady

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

Aliases & Classifications for Pancreatoblastoma

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Sources:
9Disease Ontology, 41NIH Rare Diseases, 47Orphanet, 60UMLS, 55SNOMED-CT, 38NCIt, 33MeSH, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Pancreatoblastoma, Aliases & Descriptions:

Name: Pancreatoblastoma 9 41 47 60


Classifications:



Characteristics (Orphanet epidemiological data):

47
pancreatoblastoma:
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),<1/1000000 (Worldwide); Age of onset: Adult,Childhood; Age of death: adult,early childhood,late childhood


External Ids:

Disease Ontology9 DOID:6823
NCIt38 C4265
MeSH33 C537162
Orphanet47 677
SNOMED-CT55 189814006, 53618008
MESH via Orphanet34 C537162
ICD10 via Orphanet26 C25.1
UMLS via Orphanet61 C0334489

Summaries for Pancreatoblastoma

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Wikipedia:63 Pancreatoblastoma is a rare type of pancreatic cancer. more...

MalaCards based summary: Pancreatoblastoma is related to acinar cell carcinoma and adenoma. 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.

Related Diseases for Pancreatoblastoma

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

(show top 50)    (show all 53)
idRelated DiseaseScoreTop Affiliating Genes
1acinar cell carcinoma30.4CTNNB1, CHGA, AFP
2adenoma29.0SMAD4, APC, CLDN3, CHGA, CTNNB1, SYP
3adenocarcinoma28.2CLDN5, CLDN3, SMAD4, APC, CLDN7, CHGA
4pancreatitis10.5
5sertoli cell tumor10.4SYP
6dysembryoplastic neuroepithelial tumor10.4SYP
7beckwith-wiedemann syndrome10.4
8central neurocytoma10.2SYP, CHGA
9islet cell tumor10.2SYP, CHGA
10pineocytoma10.2CHGA, SYP
11endocrine gland cancer10.2CHGA, SYP
12merkel cell carcinoma10.2SYP, CHGA
13ganglioglioma10.2CHGA, SYP
14oxyphilic adenoma10.2SYP, CLDN7
15malignant mixed mullerian tumor10.2CTNNB1, AFP
16lung cancer10.2CHGA, SYP
17ganglioneuroma10.2CHGA, SYP
18large cell carcinoma10.1CHGA, SYP
19pancreatic ductal carcinoma10.1SMAD4, CHGA
20neuroendocrine tumor10.1SYP, CHGA
21vipoma10.1CHGA, SMAD4
22endometrial cancer10.1CTNNB1, CHGA
23intestinal neoplasm10.1APC, CTNNB1
24paraganglioma10.1SYP, CHGA
25cushing's syndrome10.1SYP, CHGA
26desmoid disease, hereditary10.0APC, CTNNB1
27mismatch repair cancer syndrome10.0CTNNB1, APC
28neuroblastoma10.0
29polyposis, juvenile intestinal10.0SMAD4, APC
30fibromatosis10.0CTNNB1, APC
31peutz-jeghers syndrome10.0APC, CTNNB1
32adrenocortical carcinoma10.0CHGA, SYP, SMAD4
33hepatocellular adenoma9.9AFP, CTNNB1, APC
34pituitary adenoma9.9APC, CHGA, SYP
35hepatoblastoma9.9AFP, CTNNB1, APC
36medulloblastoma9.9APC, SYP, CTNNB1
37papillary carcinoma9.8CLDN3, CHGA, SMAD4, SYP
38tubular adenocarcinoma9.8CHGA, AFP, SYP, CTNNB1
39prostate cancer9.8CTNNB1, CHGA, SMAD4
40adenomatous polyposis coli9.8APC, CTNNB1, SMAD4
41pancreatic cancer9.7SMAD4, CHGA, CTNNB1, SYP
42ovarian cancer, somatic9.7CLDN3, CTNNB1, SMAD4
43adrenal cortical adenoma9.7CTNNB1, SYP, APC, CHGA
44colorectal cancer9.6CTNNB1, SMAD4, APC
45gastrinoma9.6CHGA, APC, SMAD4, SYP
46lung benign neoplasm9.6CHGA, APC, SMAD4, SYP
47retinoblastoma9.6SMAD4, APC, SYP
48hepatocellular carcinoma9.5APC, SMAD4, CTNNB1, AFP
49breast cancer9.4CTNNB1, CLDN7, APC, SMAD4, CLDN3
50gastric cancer, somatic9.3SMAD4, AFP, APC, CLDN7, CTNNB1, CLDN3

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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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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MalaCards organs/tissues related to Pancreatoblastoma:

31
Pancreas, Liver, Brain

Animal Models for Pancreatoblastoma or affiliated genes

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MGI Mouse Phenotypes related to Pancreatoblastoma:

35
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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Articles related to Pancreatoblastoma:

(show top 50)    (show all 113)
idTitleAuthorsYear
1
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. (25110242)
2014
2
CT-guided radiofrequency ablation following high-dose chemotherapy of a liver-metastasizing pancreatoblastoma with tumor thrombus in the portal vein. (23828484)
2013
3
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. (24081396)
2013
4
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. (23991735)
2013
5
Congenital pancreatoblastoma: report of an atypical case and review of the literature. (22278199)
2012
6
Management of pancreatoblastoma in children and young adults. (22525406)
2012
7
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. (22572137)
2012
8
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). (21696948)
2011
9
Pancreatoblastoma in an adult patient. A case report. (19287117)
2009
10
Imprint cytology of pancreatoblastoma: a case report and review of the literature. (19229998)
2009
11
Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. (19194274)
2009
12
Childhood pancreatoblastoma: clinical features and immunohistochemistry analysis. (18308466)
2008
13
Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. (18981556)
2008
14
Long-term survival of pancreatoblastoma in children. (18090935)
2007
15
Adrenocorticotropin-secreting pancreatoblastoma. (17642425)
2007
16
Pancreatoblastoma in an adult: case report and review of the literature. (16769539)
2006
17
Pediatric pancreatoblastoma: histopathologic and cytogenetic characterization of tumor and derived cell line. (15721631)
2005
18
Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature. (16080945)
2005
19
Fine-needle aspiration cytology of pancreatoblastoma in a young woman: report of a case and review of the literature. (16138370)
2005
20
Pancreatoblastoma in an adult woman: sonography, CT, and dynamic gadolinium-enhanced MRI features. (15728031)
2005
21
Diagnosis, treatment and outcome of pancreatoblastoma. (15256806)
2004
22
The fine-needle aspiration biopsy cytology of pancreatoblastoma. (15540188)
2004
23
Antenatal manifestation of congenital pancreatoblastoma in a fetus with Beckwith-Wiedemann syndrome. (12673632)
2003
24
Ampullary pancreatoblastoma in an elderly patient: a case report and review of the literature. (14567752)
2003
25
Significance of aberrant (cytoplasmic/nuclear) expression of beta-catenin in pancreatoblastoma. (12533831)
2003
26
Pancreatoblastoma. A case report with special emphasis on squamoid corpuscles with optically clear nuclei rich in biotin. (12920766)
2003
27
Pancreatoblastoma is associated with chromosome 11p loss of heterozygosity and IGF2 overexpression. (12116082)
2002
28
Fine-needle aspiration cytology of pancreatoblastoma. (11477717)
2001
29
Pancreatoblastoma. (11770246)
2001
30
Imaging features of pancreatoblastoma. (11486805)
2001
31
Pancreatoblastoma treated by delayed operation after effective chemotherapy. (11083448)
2000
32
Pancreatoblastoma in an adolescent girl: case report and review of 26 Japanese cases. (9641374)
1998
33
Pancreatoblastoma. (9396538)
1997
34
Pancreatoblastoma in children. Case report and review of the literature. (9007475)
1996
35
Pancreatoblastoma: a histochemical and immunohistochemical analysis. (8944621)
1996
36
Adult pancreatoblastoma: a case report and review of the literature. (8792711)
1996
37
Pancreatoblastoma: case report and review of treatment in the literature. (8699999)
1996
38
Cytogenetic and flow cytometric analysis of a pancreatoblastoma. (7889500)
1995
39
Adenocarcinoma of the pancreas in childhood (pancreatoblastoma): report of a case with good response to chemotherapy. (8804461)
1995
40
Pancreatoblastoma in an older adult. (7605173)
1995
41
Pancreatoblastoma: a case report. (7954063)
1994
42
Pancreatoblastoma and solid and cystic papillary tumor: two tumors related to pancreatic ontogeny. (7809509)
1994
43
Pancreatoblastoma with marked elevation of serum alpha-fetoprotein. An autopsy case report with immunocytochemical study. (1689089)
1990
44
Pancreatoblastoma: MR appearance. (2159494)
1990
45
Use of enzyme histochemistry in the diagnosis of pancreatoblastoma. (2553578)
1989
46
Recurrent pancreatoblastoma with inappropriate adrenocorticotrophic hormone secretion. (2852926)
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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Search GEO for disease gene expression data for Pancreatoblastoma.

Pathways for genes affiliated with Pancreatoblastoma

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Pathways related to Pancreatoblastoma according to GeneCards Suite gene sharing:

(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
89.3CTNNB1, APC, AFP
9
Show member pathways
Wnt Signaling Pathway NetPath36
9.2CTNNB1, APC, SMAD4
109.2CTNNB1, APC, SMAD4
11
Show member pathways
DNA damage response (only ATM dependent)36
Wnt Signaling Pathway and Pluripotency36
9.2CTNNB1, APC, SMAD4
129.2SMAD4, APC, CTNNB1
13
Show member pathways
Signal transduction PTEN pathway58
9.2SMAD4, APC, CTNNB1
149.2SMAD4, APC, CTNNB1
158.9CTNNB1, APC, AFP, SMAD4
168.9CLDN3, CLDN5, CTNNB1
178.5CLDN5, CLDN7, CLDN3
188.5CLDN3, CLDN7, CLDN5
198.1CLDN3, CLDN7, CLDN5, CTNNB1
20
Show member pathways
8.1CLDN3, CLDN7, CLDN5, CTNNB1
21
Show member pathways
8.1CLDN3, CLDN7, CLDN5, CTNNB1
228.1CTNNB1, CLDN5, CLDN7, CLDN3
23
Show member pathways
8.1CTNNB1, CLDN5, CLDN7, CLDN3
24
Show member pathways
Cytoskeleton remodeling Fibronectin binding integrins in cell motility58
Cytoskeleton remodeling Integrin outside in signaling58
Cytoskeleton remodeling Regulation of actin cytoskeleton by Rho GTPases58
Cell adhesion Tight junctions58
Development MAG dependent inhibition of neurite outgrowth58
8.1CTNNB1, CLDN5, CLDN7, CLDN3

Compounds for genes affiliated with Pancreatoblastoma

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Compounds related to Pancreatoblastoma according to GeneCards Suite gene sharing:

(show top 50)    (show all 64)
idCompoundScoreTop Affiliating Genes
1smcc4310.2CHGA, SYP
2111in-pentetreotide4310.2CHGA, SYP
3s 1004310.2SYP, CHGA
4lithium chloride4310.1CHGA, CTNNB1
51-hydroxyanthraquinone4310.0CTNNB1, APC
6methylazoxymethanol acetate4310.0CTNNB1, APC
7samp4310.0CTNNB1, APC
81,2-dimethylhydrazine4310.0APC, CTNNB1
9azoxymethane4310.0CTNNB1, APC
10b 723439.9AFP, CHGA, SYP
112-amino-3-methylimidazo(4,5-f)quinoline439.9APC, CTNNB1
12silver439.9SYP, CHGA, AFP
13exisulind439.9CTNNB1, APC
14sodium bisulfite439.9APC, CTNNB1
15l-amino acid439.8SYP, CHGA
16gold439.8SYP, CHGA, AFP
17phip439.8CTNNB1, APC
18haloperidol43 49 28 2 1213.7CTNNB1, SYP, CHGA
19lithium43 49 24 1212.7CTNNB1, SYP, APC
20crcs439.7CTNNB1, APC, SMAD4
21acth439.7APC, CHGA, SYP
22tgf beta1439.6CTNNB1, AFP, SMAD4
23sucrose43 24 1211.6CTNNB1, SYP, CHGA
24chloramphenicol43 2 49 1212.5CHGA, AFP, SMAD4
25gnrh439.5SMAD4, CHGA, CTNNB1
26hydrogen43 2410.5AFP, APC, CHGA, SYP
27agar439.4SMAD4, AFP, CTNNB1
28epinephrine43 24 1211.4AFP, APC, CHGA
29guanine43 24 1211.4APC, CHGA, SYP, CTNNB1
30cisplatin43 49 59 1212.4AFP, CHGA, SYP, CTNNB1
31glutamine439.4AFP, APC, SYP, CTNNB1
32vitamin d439.4AFP, APC, CHGA, CTNNB1
33dopamine43 28 24 1212.3APC, CHGA, SYP, CTNNB1
34fatty acid439.3AFP, APC, CHGA, CTNNB1
35dexamethasone43 49 28 1212.3CTNNB1, SYP, CHGA, AFP
36cholesterol43 28 24 1212.2SYP, CHGA, CLDN3, AFP
37etoposide43 49 59 1212.2AFP, CHGA, SYP
38steroid439.1SMAD4, AFP, CHGA, SYP, CTNNB1
39glycogen43 2410.1AFP, APC, CHGA, SYP, CTNNB1
40progesterone43 28 59 24 1213.1AFP, APC, CHGA, SYP, CTNNB1
41threonine438.8CTNNB1, APC, AFP, SMAD4
42paraffin438.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
43testosterone43 59 24 1211.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
44estrogen438.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
45serine438.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
46vegf438.6SMAD4, AFP, CLDN3, CHGA, SYP, CTNNB1
47retinoic acid43 249.6AFP, APC, CHGA, SYP, KRT15, CTNNB1
48calcium43 49 24 1211.2AFP, APC, CLDN3, CLDN5, CHGA, SYP
49tyrosine438.2SMAD4, AFP, APC, CHGA, SYP, KRT15
50oligonucleotide437.6CTNNB1, SMAD4, AFP, APC, CLDN7, CLDN5

GO Terms for genes affiliated with Pancreatoblastoma

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

idNameGO IDScoreTop Affiliating Genes
1beta-catenin destruction complexGO:00308779.5APC, CTNNB1
2cell-cell adherens junctionGO:00059139.3CTNNB1, APC
3centrosomeGO:00058139.2CTNNB1, APC, SMAD4
4lateral plasma membraneGO:00163288.8CTNNB1, CLDN7, APC
5tight junctionGO:00059238.2CLDN5, CLDN7, CLDN3, APC

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

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1SMAD protein signal transductionGO:006039510.0SMAD4, AFP
2canonical Wnt signaling pathway involved in negative regulation of apoptotic processGO:00443369.8CTNNB1, APC
3gastrulation with mouth forming secondGO:00017029.8CTNNB1, SMAD4
4proximal/distal pattern formationGO:00099549.7CTNNB1, APC
5T cell differentiation in thymusGO:00330779.7APC, CTNNB1
6thymus developmentGO:00485389.6CTNNB1, APC
7cellular component disassembly involved in execution phase of apoptosisGO:00069219.5CTNNB1, APC
8branching involved in ureteric bud morphogenesisGO:00016589.3SMAD4, CTNNB1
9negative regulation of cell proliferationGO:00082859.2CTNNB1, APC, SMAD4
10calcium-independent cell-cell adhesionGO:00163388.7CLDN5, CLDN7, CLDN3

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

idNameGO IDScoreTop Affiliating Genes
1cadherin bindingGO:00452969.6CTNNB1, APC
2I-SMAD bindingGO:00704119.5CTNNB1, SMAD4
3R-SMAD bindingGO:00704129.3CTNNB1, SMAD4
4structural molecule activityGO:00051988.1CLDN3, CLDN7, CLDN5, CTNNB1
5identical protein bindingGO:00428027.9SYP, CLDN5, CLDN7, CLDN3, SMAD4

Products for genes affiliated with Pancreatoblastoma

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Sources for Pancreatoblastoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet