MCID: PNC019
MIFTS: 48

Pancreatoblastoma malady

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

Summaries for Pancreatoblastoma

About this section


Wikipedia:65 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 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:
10Disease Ontology, 43NIH Rare Diseases, 49Orphanet, 62UMLS, 57SNOMED-CT, 40NCIt, 35MeSH, 36MESH via Orphanet, 28ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Pancreatoblastoma, Aliases & Descriptions:

Name: Pancreatoblastoma 10 43 49 62


Classifications:



Characteristics (Orphanet epidemiological data):

49
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 Ontology10 DOID:6823
NCIt40 C4265
MeSH35 C537162
Orphanet49 677
SNOMED-CT57 189814006, 53618008
MESH via Orphanet36 C537162
ICD10 via Orphanet28 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 53)
idRelated DiseaseScoreTop Affiliating Genes
1acinar cell carcinoma30.4CTNNB1, CHGA, AFP
2adenoma29.9SMAD4, APC, CLDN3, CHGA, SYP, CTNNB1
3adenocarcinoma29.8SMAD4, AFP, APC, CLDN3, CLDN7, CLDN5
4pancreatitis10.5
5beckwith-wiedemann syndrome10.4
6sertoli cell tumor10.1SYP
7dysembryoplastic neuroepithelial tumor10.0SYP
8neuroblastoma10.0
9central neurocytoma10.0CHGA, SYP
10islet cell tumor10.0SYP, CHGA
11endocrine gland cancer10.0CHGA, SYP
12pineocytoma10.0CHGA, SYP
13merkel cell carcinoma10.0SYP, CHGA
14ganglioglioma10.0CHGA, SYP
15oxyphilic adenoma10.0CLDN7, SYP
16malignant mixed mullerian tumor10.0CTNNB1, AFP
17lung cancer10.0SYP, CHGA
18ganglioneuroma10.0SYP, CHGA
19large cell carcinoma10.0CHGA, SYP
20pancreatic ductal carcinoma10.0SMAD4, CHGA
21neuroendocrine tumor10.0CHGA, SYP
22vipoma10.0CHGA, SMAD4
23endometrial cancer10.0CHGA, CTNNB1
24intestinal neoplasm10.0APC, CTNNB1
25paraganglioma10.0CHGA, SYP
26cushing's syndrome10.0SYP, CHGA
27desmoid disease, hereditary10.0APC, CTNNB1
28mismatch repair cancer syndrome10.0CTNNB1, APC
29polyposis, juvenile intestinal10.0APC, SMAD4
30fibromatosis10.0APC, CTNNB1
31peutz-jeghers syndrome10.0APC, CTNNB1
32adrenocortical carcinoma10.0SMAD4, CHGA, SYP
33hepatocellular adenoma10.0AFP, APC, CTNNB1
34pituitary adenoma10.0SYP, CHGA, APC
35hepatoblastoma10.0CTNNB1, APC, AFP
36medulloblastoma10.0CTNNB1, SYP, APC
37papillary carcinoma10.0SMAD4, CLDN3, CHGA, SYP
38tubular adenocarcinoma10.0AFP, CHGA, SYP, CTNNB1
39prostate cancer10.0SMAD4, CHGA, CTNNB1
40adenomatous polyposis coli10.0SMAD4, APC, CTNNB1
41pancreatic cancer10.0SMAD4, CHGA, SYP, CTNNB1
42ovarian cancer, somatic10.0SMAD4, CLDN3, CTNNB1
43adrenal cortical adenoma10.0CTNNB1, SYP, CHGA, APC
44colorectal cancer9.9CTNNB1, APC, SMAD4
45gastrinoma9.9SYP, CHGA, APC, SMAD4
46lung benign neoplasm9.9SMAD4, APC, CHGA, SYP
47retinoblastoma9.9SMAD4, APC, SYP
48hepatocellular carcinoma9.9SMAD4, AFP, APC, CTNNB1
49breast cancer9.9CTNNB1, CLDN7, CLDN3, APC, SMAD4
50gastric cancer, somatic9.9SMAD4, AFP, APC, CLDN3, CLDN7, CTNNB1

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:

33
Pancreas, Liver, Brain

Animal Models for Pancreatoblastoma or affiliated genes

About this section

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

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

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 NetPath38
9.2CTNNB1, SMAD4, APC
119.2CTNNB1, APC, SMAD4
12
Show member pathways
DNA damage response (only ATM dependent)38
Wnt Signaling Pathway and Pluripotency38
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
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 30 3 1313.7CTNNB1, SYP, CHGA
19lithium45 51 26 1312.7CTNNB1, SYP, APC
20crcs459.7CTNNB1, APC, SMAD4
21acth459.7APC, CHGA, SYP
22tgf beta1459.6CTNNB1, AFP, SMAD4
23sucrose45 26 1311.6CTNNB1, SYP, CHGA
24chloramphenicol45 3 51 1312.5CHGA, AFP, SMAD4
25gnrh459.5SMAD4, CHGA, CTNNB1
26hydrogen45 2610.5AFP, APC, CHGA, SYP
27agar459.4SMAD4, AFP, CTNNB1
28epinephrine45 26 1311.4AFP, APC, CHGA
29guanine45 26 1311.4APC, CHGA, SYP, CTNNB1
30cisplatin45 51 61 1312.4AFP, CHGA, SYP, CTNNB1
31glutamine459.4AFP, APC, SYP, CTNNB1
32vitamin d459.4AFP, APC, CHGA, CTNNB1
33dopamine45 30 26 1312.3APC, CHGA, SYP, CTNNB1
34fatty acid459.3AFP, APC, CHGA, CTNNB1
35dexamethasone45 51 30 1312.3CTNNB1, SYP, CHGA, AFP
36cholesterol45 30 26 1312.2SYP, CHGA, CLDN3, AFP
37etoposide45 51 61 1312.2AFP, CHGA, SYP
38steroid459.1SMAD4, AFP, CHGA, SYP, CTNNB1
39glycogen45 2610.1AFP, APC, CHGA, SYP, CTNNB1
40progesterone45 30 61 26 1313.1AFP, APC, CHGA, SYP, CTNNB1
41threonine458.8CTNNB1, APC, AFP, SMAD4
42paraffin458.7SMAD4, AFP, APC, CHGA, SYP, CTNNB1
43testosterone45 61 26 1311.7SMAD4, 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 269.6AFP, APC, CHGA, SYP, KRT15, CTNNB1
48calcium45 51 26 1311.2AFP, 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

About this section

Cellular components related to Pancreatoblastoma according to GeneCards/GeneDecks:

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/GeneDecks:

(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/GeneDecks:

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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Pancreatoblastoma

About this section
4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet