MCID: PNC019
MIFTS: 49

Pancreatoblastoma malady

Rare diseases, Gastrointestinal diseases, Cancer diseases, Endocrine diseases categories
Download this MalaCard

Summaries for Pancreatoblastoma

About this section


Fully expand this MalaCard
Wikipedia:65 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 receptor signaling. 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

About this section
Sources:
8Disease Ontology, 42NIH Rare Diseases, 48Orphanet, 62UMLS, 39NCIt, 57SNOMED-CT, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Pancreatoblastoma, Aliases & Descriptions:

Name: Pancreatoblastoma 8 42 48 62


Classifications:



Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology8 DOID:6823
NCIt39 C4265
MeSH34 C537162
SNOMED-CT57 53618008, 189814006
MESH via Orphanet35 C537162
ICD10 via Orphanet26 C25.1
UMLS via Orphanet63 C0334489

Related Diseases for Pancreatoblastoma

About this section

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

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to pancreatoblastoma

Symptoms for Pancreatoblastoma

About this section

Drugs & Therapeutics for Pancreatoblastoma

About this section

Drug clinical trials:

Search ClinicalTrials for Pancreatoblastoma

Search NIH Clinical Center for Pancreatoblastoma

Genetic Tests for Pancreatoblastoma

About this section

Anatomical Context for Pancreatoblastoma

About this section

MalaCards organs/tissues related to Pancreatoblastoma:

32
Pancreas, Liver, Brain

Animal Models for Pancreatoblastoma or affiliated genes

About this section

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

About this section

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

About this section

Expression for genes affiliated with Pancreatoblastoma

About this section
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

About this section

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
39.6SMAD4, CTNNB1
49.6SMAD4, CTNNB1
59.6CTNNB1, APC
6
Show member pathways
9.6APC, CTNNB1
79.6CTNNB1, APC
89.5KRT15, APC
99.3CTNNB1, APC, AFP
10
Show member pathways
Wnt Signaling Pathway NetPath37
9.2CTNNB1, SMAD4, APC
119.2CTNNB1, APC, SMAD4
12
Show member pathways
DNA damage response (only ATM dependent)37
Wnt Signaling Pathway and Pluripotency37
9.2APC, SMAD4, CTNNB1
13
Show member pathways
Signal transduction PTEN pathway60
9.2APC, SMAD4, CTNNB1
149.2SMAD4, CTNNB1, APC
159.2SMAD4, APC, CTNNB1
168.9AFP, SMAD4, APC, CTNNB1
178.9CTNNB1, CLDN5, CLDN3
188.5CLDN3, CLDN7, CLDN5
198.5CLDN3, CLDN7, CLDN5
20
Show member pathways
8.1CLDN7, CLDN3, CTNNB1, CLDN5
218.1CLDN3, CTNNB1, CLDN7, CLDN5
228.1CLDN7, CTNNB1, CLDN5, CLDN3
23
Show member pathways
8.1CTNNB1, CLDN5, CLDN7, CLDN3
24
Show member pathways
8.1CLDN5, CLDN7, CLDN3, CTNNB1
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
8.1CLDN3, CTNNB1, CLDN5, CLDN7

Compounds for genes affiliated with Pancreatoblastoma

About this section

Compounds related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show top 50)    (show all 64)
idCompoundScoreTop Affiliating Genes
1smcc4410.2CHGA, SYP
2111in-pentetreotide4410.2SYP, CHGA
3s 1004410.2CHGA, SYP
4lithium chloride4410.1CHGA, CTNNB1
51-hydroxyanthraquinone4410.0CTNNB1, APC
6methylazoxymethanol acetate4410.0CTNNB1, APC
7samp4410.0CTNNB1, APC
81,2-dimethylhydrazine4410.0CTNNB1, APC
9azoxymethane4410.0CTNNB1, APC
10b 723449.9AFP, CHGA, SYP
112-amino-3-methylimidazo(4,5-f)quinoline449.9CTNNB1, APC
12silver449.9AFP, SYP, CHGA
13exisulind449.9CTNNB1, APC
14sodium bisulfite449.9CTNNB1, APC
15l-amino acid449.8CHGA, SYP
16gold449.8CHGA, AFP, SYP
17phip449.8APC, CTNNB1
18haloperidol44 50 28 2 1113.7CHGA, CTNNB1, SYP
19lithium44 50 24 1112.7CTNNB1, SYP, APC
20crcs449.7APC, CTNNB1, SMAD4
21acth449.7APC, CHGA, SYP
22tgf beta1449.6SMAD4, AFP, CTNNB1
23sucrose44 24 1111.6CHGA, CTNNB1, SYP
24chloramphenicol44 2 50 1112.5SMAD4, AFP, CHGA
25gnrh449.5SMAD4, CTNNB1, CHGA
26hydrogen44 2410.5CHGA, SYP, APC, AFP
27agar449.4SMAD4, AFP, CTNNB1
28epinephrine44 24 1111.4AFP, APC, CHGA
29guanine44 24 1111.4APC, CHGA, SYP, CTNNB1
30cisplatin44 50 61 1112.4AFP, CHGA, SYP, CTNNB1
31glutamine449.4SYP, APC, AFP, CTNNB1
32vitamin d449.4AFP, CTNNB1, APC, CHGA
33dopamine44 28 24 1112.3APC, CHGA, SYP, CTNNB1
34fatty acid449.3CTNNB1, APC, CHGA, AFP
35dexamethasone44 50 28 1112.3CTNNB1, SYP, CHGA, AFP
36cholesterol44 28 24 1112.2CHGA, CLDN3, AFP, SYP
37etoposide44 50 61 1112.2CHGA, SYP, AFP
38steroid449.1AFP, SMAD4, CHGA, SYP, CTNNB1
39glycogen44 2410.1AFP, APC, CTNNB1, SYP, CHGA
40progesterone44 28 61 24 1113.1CTNNB1, SYP, CHGA, AFP, APC
41threonine448.8SMAD4, CTNNB1, APC, AFP
42paraffin448.7SMAD4, AFP, APC, CTNNB1, SYP, CHGA
43testosterone44 61 24 1111.7CHGA, SMAD4, AFP, APC, SYP, CTNNB1
44estrogen448.7SMAD4, APC, SYP, CTNNB1, CHGA, AFP
45serine448.7AFP, SMAD4, CHGA, CTNNB1, SYP, APC
46vegf448.6CTNNB1, SMAD4, AFP, CLDN3, CHGA, SYP
47retinoic acid44 249.6CHGA, SYP, CTNNB1, APC, AFP, KRT15
48calcium44 50 24 1111.2SYP, CLDN3, APC, AFP, CLDN5, CHGA
49tyrosine448.2APC, SYP, AFP, SMAD4, CHGA, KRT15
50oligonucleotide447.6CLDN5, CLDN7, APC, AFP, SMAD4, CHGA

GO Terms for genes affiliated with Pancreatoblastoma

About this section

Cellular components related to Pancreatoblastoma according to GeneCards/GeneDecks:

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

Biological processes related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1SMAD protein signal transductionGO:06039510.0AFP, SMAD4
2canonical Wnt signaling pathway involved in negative regulation of apoptotic processGO:0443369.8APC, CTNNB1
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, SMAD4, APC
10calcium-independent cell-cell adhesionGO:0163388.7CLDN5, CLDN3, CLDN7

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:0051988.1CTNNB1, CLDN3, CLDN7, CLDN5
5identical protein bindingGO:0428027.9CLDN3, SMAD4, CLDN7, SYP, CLDN5

Products for genes affiliated with Pancreatoblastoma

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Pancreatoblastoma

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet