MCID: PNC019
MIFTS: 48

Pancreatoblastoma malady

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

Summaries for Pancreatoblastoma

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Wikipedia:64 Pancreatoblastoma is a rare type of pancreatic cancer. more...

MalaCards based summary: 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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Sources:
9Disease Ontology, 42NIH Rare Diseases, 48Orphanet, 61UMLS, 39NCIt, 56SNOMED-CT, 34MeSH, 35MESH via Orphanet, 27ICD10 via Orphanet, 62UMLS via Orphanet
See all sources

Pancreatoblastoma, Aliases & Descriptions:

Name: Pancreatoblastoma 9 42 48 61


Classifications:



Characteristics (Orphanet epidemiological data):

48
pancreatoblastoma:
Prevalence: <1/1000000; Age of onset: Childhood


External Ids:

Disease Ontology9 DOID:6823
NCIt39 C4265
MeSH34 C537162
SNOMED-CT56 53618008, 189814006
MESH via Orphanet35 C537162
ICD10 via Orphanet27 C25.1
UMLS via Orphanet62 C0334489

Related Diseases for Pancreatoblastoma

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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 polyposis29.7SMAD4, APC, CTNNB1
3adenoma29.0SMAD4, APC, CLDN3, CHGA, SYP, CTNNB1
4adenocarcinoma28.2SMAD4, AFP, APC, CLDN3, CLDN7, CLDN5
5pancreatitis10.5
6sertoli cell tumor10.5SYP
7dysembryoplastic neuroepithelial tumor10.4SYP
8beckwith-wiedemann syndrome10.4
9central neurocytoma10.2CHGA, SYP
10pancreatic islet cell tumors10.2SYP, CHGA
11endocrine gland cancer10.2CHGA, SYP
12pineocytoma10.2CHGA, SYP
13merkel cell carcinoma10.2SYP, CHGA
14ganglioglioma10.2CHGA, SYP
15oxyphilic adenoma10.2CLDN7, SYP
16malignant mixed mullerian tumor10.2CTNNB1, AFP
17lung cancer10.2SYP, CHGA
18ganglioneuroma10.2SYP, CHGA
19large cell carcinoma10.2CHGA, SYP
20pancreatic ductal carcinoma10.1SMAD4, CHGA
21neuroendocrine carcinoma10.1CHGA, SYP
22vipoma10.1CHGA, SMAD4
23endometrial carcinoma10.1CHGA, CTNNB1
24gardner syndrome10.1APC, CTNNB1
25intestinal neoplasm10.1APC, CTNNB1
26paraganglioma10.1CHGA, SYP
27cushing's syndrome10.1SYP, CHGA
28desmoid tumor10.1APC, CTNNB1
29turcot syndrome10.1CTNNB1, APC
30colorectal cancer, somatic10.0APC, CTNNB1
31neuroblastoma10.0
32juvenile polyposis syndrome10.0APC, SMAD4
33fibromatosis10.0APC, CTNNB1
34peutz-jeghers syndrome10.0APC, CTNNB1
35adrenocortical carcinoma10.0SMAD4, CHGA, SYP
36hepatocellular adenoma9.9AFP, APC, CTNNB1
37pituitary adenoma9.9SYP, CHGA, APC
38hepatoblastoma9.9CTNNB1, APC, AFP
39medulloblastoma9.9CTNNB1, SYP, APC
40papillary carcinoma9.9SMAD4, CLDN3, CHGA, SYP
41tubular adenocarcinoma9.8AFP, CHGA, SYP, CTNNB1
42prostate cancer9.8SMAD4, CHGA, CTNNB1
43pancreatic cancer9.8SMAD4, CHGA, SYP, CTNNB1
44ovarian cancer9.7SMAD4, CLDN3, CTNNB1
45adrenal cortical adenoma9.7CTNNB1, SYP, CHGA, APC
46colonic benign neoplasm9.7SMAD4, APC, CLDN3, CTNNB1
47colorectal cancer9.6CTNNB1, APC, SMAD4
48gastrinoma9.6SYP, CHGA, APC, SMAD4
49lung benign neoplasm9.6SMAD4, APC, CHGA, SYP
50retinoblastoma9.6SMAD4, 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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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:

32
Pancreas, Liver, Brain

Animal Models for Pancreatoblastoma or affiliated genes

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MGI Mouse Phenotypes related to Pancreatoblastoma:

36
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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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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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 NetPath37
9.2CTNNB1, APC, SMAD4
119.2CTNNB1, APC, SMAD4
12
Show member pathways
DNA damage response (only ATM dependent)37
Wnt Signaling Pathway and Pluripotency37
9.2CTNNB1, APC, SMAD4
139.2SMAD4, APC, CTNNB1
14
Show member pathways
Signal transduction PTEN pathway59
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 motility59
Cytoskeleton remodeling Integrin outside in signaling59
Cytoskeleton remodeling Regulation of actin cytoskeleton by Rho GTPases59
Cell adhesion Tight junctions59
Development MAG dependent inhibition of neurite outgrowth59
8.1CTNNB1, CLDN5, CLDN7, CLDN3

Compounds for genes affiliated with Pancreatoblastoma

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Compounds related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show top 50)    (show all 64)
idCompoundScoreTop Affiliating Genes
1smcc4410.2CHGA, SYP
2111in-pentetreotide4410.2CHGA, SYP
3s 1004410.2SYP, CHGA
4lithium chloride4410.1CHGA, CTNNB1
51-hydroxyanthraquinone4410.0CTNNB1, APC
6methylazoxymethanol acetate4410.0CTNNB1, APC
7samp4410.0CTNNB1, APC
81,2-dimethylhydrazine4410.0APC, CTNNB1
9azoxymethane4410.0CTNNB1, APC
10b 723449.9AFP, CHGA, SYP
112-amino-3-methylimidazo(4,5-f)quinoline449.9APC, CTNNB1
12silver449.9SYP, CHGA, AFP
13exisulind449.9CTNNB1, APC
14sodium bisulfite449.9APC, CTNNB1
15l-amino acid449.8SYP, CHGA
16gold449.8SYP, CHGA, AFP
17phip449.8CTNNB1, APC
18haloperidol44 50 29 3 1213.7CTNNB1, SYP, CHGA
19lithium44 50 25 1212.7CTNNB1, SYP, APC
20crcs449.7CTNNB1, APC, SMAD4
21acth449.7APC, CHGA, SYP
22tgf beta1449.6CTNNB1, AFP, SMAD4
23sucrose44 25 1211.6CTNNB1, SYP, CHGA
24chloramphenicol44 3 50 1212.5CHGA, AFP, SMAD4
25gnrh449.5SMAD4, CHGA, CTNNB1
26hydrogen44 2510.5AFP, APC, CHGA, SYP
27agar449.4SMAD4, AFP, CTNNB1
28epinephrine44 25 1211.4AFP, APC, CHGA
29guanine44 25 1211.4APC, CHGA, SYP, CTNNB1
30cisplatin44 50 60 1212.4AFP, CHGA, SYP, CTNNB1
31glutamine449.4AFP, APC, SYP, CTNNB1
32vitamin d449.4AFP, APC, CHGA, CTNNB1
33dopamine44 29 25 1212.3APC, CHGA, SYP, CTNNB1
34fatty acid449.3AFP, APC, CHGA, CTNNB1
35dexamethasone44 50 29 1212.3CTNNB1, SYP, CHGA, AFP
36cholesterol44 29 25 1212.2SYP, CHGA, CLDN3, AFP
37etoposide44 50 60 1212.2AFP, CHGA, SYP
38steroid449.1SMAD4, AFP, CHGA, SYP, CTNNB1
39glycogen44 2510.1AFP, APC, CHGA, SYP, CTNNB1
40progesterone44 29 60 25 1213.1AFP, APC, CHGA, SYP, CTNNB1
41threonine448.8CTNNB1, APC, AFP, SMAD4
42paraffin448.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
43testosterone44 60 25 1211.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
44estrogen448.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
45serine448.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
46vegf448.6SMAD4, AFP, CLDN3, CHGA, SYP, CTNNB1
47retinoic acid44 259.6AFP, APC, CHGA, SYP, KRT15, CTNNB1
48calcium44 50 25 1211.2AFP, APC, CLDN3, CLDN5, CHGA, SYP
49tyrosine448.2SMAD4, AFP, APC, CHGA, SYP, KRT15
50oligonucleotide447.6CTNNB1, SMAD4, AFP, APC, CLDN7, CLDN5

GO Terms for genes affiliated with Pancreatoblastoma

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Cellular components related to Pancreatoblastoma according to GeneCards/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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Sources for Pancreatoblastoma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
29IUPHAR
30KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet