MCID: PNC019
MIFTS: 39

Pancreatoblastoma malady

Gastrointestinal, Cancer, Endocrine categories

Summaries for Pancreatoblastoma

Sources:
64Wikipedia, 33MalaCards
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Wikipedia:64 Pancreatoblastoma is a rare tumor of the pancreas. more...

MalaCards: Pancreatoblastoma is related to acinar cell carcinoma and adenoma. An important gene associated with Pancreatoblastoma is SYP (synaptophysin), and among its related pathways are Platelet sensitization by LDL and Canonical Wnt signaling pathway. The compounds smcc and 111in-pentetreotide have been mentioned in the context of this disorder. Affiliated tissues include liver, brain and pancreas, and related mouse phenotypes are endocrine/exocrine gland and renal/urinary system.

Aliases & Classifications for Pancreatoblastoma

Sources:
8Disease Ontology, 43NIH Rare Diseases, 49Orphanet, 61UMLS, 57SNOMED-CT, 40NCIt, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Gastrointestinal, Endocrine


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

pancreatoblastoma 8 43 49 61


External Ids:

Disease Ontology8 DOID:6823
NCIt40 C4265
MeSH35 C537162
MESH via Orphanet36 C537162
SNOMED-CT57 53618008, 189814006
ICD10 via Orphanet26 C25.1
SNOMED-CT via Orphanet58 53618008
UMLS via Orphanet62 C0334489

Related Diseases for Pancreatoblastoma

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 74)
idRelated DiseaseScoreTop Affiliating Genes
1acinar cell carcinoma30.3CHGA, AFP, CTNNB1
2adenoma30.1APC, CTNNB1, SYP, CHGA, CLDN3, SMAD4
3papillary carcinoma29.9SYP, CHGA, CLDN3, SMAD4
4familial adenomatous polyposis29.9APC, CTNNB1, SMAD4
5adenocarcinoma29.9APC, CTNNB1, AFP, SYP, CHGA, CLDN5
6beckwith-wiedemann syndrome10.4
7n syndrome10.1
8papillary adenoma10.0
9neurofibroma10.0SYP
10rhabdoid tumor10.0SYP
11hepatitis10.0AFP
12neuroblastoma10.0SYP
13ependymoma10.0SYP
14parathyroid adenoma10.0CHGA
15neuroendocrine tumor10.0CHGA
16prolactinoma10.0CHGA
17dysembryoplastic neuroepithelial tumor10.0SYP
18hepatitis c10.0AFP
19hypercalcemia10.0CHGA
20gallbladder cancer10.0APC
21ganglioneuroblastoma10.0SYP
22meningioma10.0SYP
23multiple endocrine neoplasia10.0CHGA
24sertoli-leydig cell tumor10.0AFP
25glioblastoma multiforme10.0SMAD4
26germinoma10.0AFP
27sertoli cell tumor10.0SYP
28pancreatitis10.0SMAD4
29wilms tumor10.0CTNNB1
30nephroblastoma10.0CTNNB1
31granular cell tumor10.0SYP
32lung cancer10.0SYP, CHGA
33central neurocytoma10.0CHGA, SYP
34melanoma10.0CTNNB1
35endocrine gland cancer10.0SYP, CHGA
36pineocytoma10.0SYP, CHGA
37merkel cell carcinoma10.0CHGA, SYP
38pituitary tumors10.0CHGA, APC
39juvenile polyposis syndrome10.0SMAD4, APC
40large cell carcinoma10.0CHGA, SYP
41seminoma10.0SMAD4, AFP
42pancreatic ductal carcinoma10.0CHGA, SMAD4
43vipoma10.0CHGA, SMAD4
44oxyphilic adenoma10.0SYP, CLDN7
45pancreatic islet cell tumors10.0SYP, CHGA
46gardner syndrome10.0CTNNB1, APC
47medulloblastoma10.0CTNNB1, APC
48peutz-jeghers syndrome10.0CTNNB1, APC
49malignant mixed mullerian tumor10.0CTNNB1, AFP
50turcot syndrome10.0APC, CTNNB1

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to pancreatoblastoma

Clinical Features for Pancreatoblastoma

Drugs & Therapeutics for Pancreatoblastoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Pancreatoblastoma

Drug clinical trials:

Search ClinicalTrials for Pancreatoblastoma

Search NIH Clinical Center for Pancreatoblastoma

Search CenterWatch for Pancreatoblastoma

Genetic Tests for Pancreatoblastoma

Anatomical Context for Pancreatoblastoma

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Pancreatoblastoma:

33
Liver, Brain, Pancreas

Animal Models for Pancreatoblastoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Pancreatoblastoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053798.3APC, CTNNB1, AFP, CHGA, SMAD4
2MP:00053678.3SMAD4, CLDN7, CHGA, CTNNB1, APC
3MP:00107687.4SMAD4, CLDN7, CLDN5, CHGA, AFP, CTNNB1

Publications for Pancreatoblastoma

Sources:
51PubMed
See all sources

Articles related to Pancreatoblastoma:

(show top 50)    (show all 114)
idTitleAuthorsYear
1
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. (24081396)
2013
2
Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. (23042010)
2013
3
Congenital pancreatoblastoma: report of an atypical case and review of the literature. (22278199)
2012
4
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). (21696948)
2011
5
Central pancreatectomy for pancreatoblastoma in a 16-year-old girl. (21843703)
2011
6
Synchronous ectopic pancreatoblastoma in a child: a case report. (21655073)
2011
7
Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. (20632017)
2010
8
Imaging findings of pancreatoblastoma in 4 children including a case of ectopic pancreatoblastoma. (20686762)
2010
9
Corticotropin-releasing hormone-secreting pancreatoblastoma in an adult patient. (20664482)
2010
10
Pancreatoblastoma: two case reports from a medical center in Taiwan. (20057322)
2010
11
Pancreatoblastoma in an adult patient. A case report. (19287117)
2009
12
Pancreatoblastoma in adults: a review of the literature. (19077457)
2009
13
A peculiar variant of pancreatoblastoma in an adult. (18362850)
2008
14
Long-term survival of pancreatoblastoma in children. (18090935)
2007
15
Diagnosis and treatment of pancreatoblastoma in China. (17198189)
2007
16
Aggressive surgical and chemotherapeutic treatment of advanced pancreatoblastoma associated with tumor thrombus in portal vein. (16516645)
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
Clinical and biologic analysis of pancreatoblastoma. (15632705)
2005
20
The fine-needle aspiration biopsy cytology of pancreatoblastoma. (15540188)
2004
21
Pancreatoblastoma (infantile pancreatic carcinoma)--a case report. (16295493)
2004
22
Septal enhancement within pancreatoblastoma of the pancreatic head presenting with progressive anaemia. (15205844)
2004
23
Ampullary pancreatoblastoma in an elderly patient: a case report and review of the literature. (14567752)
2003
24
Pancreatoblastoma. A case report with special emphasis on squamoid corpuscles with optically clear nuclei rich in biotin. (12920766)
2003
25
Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas : frequent alterations in the APC/beta-catenin pathway and chromosome 11p. (11696422)
2001
26
Alpha-fetoprotein variants in a case of pancreatoblastoma. (11026522)
2000
27
Imaging features of pancreatoblastoma: a case report. (10593288)
1999
28
Pancreatoblastoma in children. Case report and review of the literature. (9007475)
1996
29
Pancreatoblastoma: a histochemical and immunohistochemical analysis. (8944621)
1996
30
Pancreatoblastoma: case report and review of treatment in the literature. (8699999)
1996
31
&quot;Chemotherapy for unresectable pancreatoblastoma&quot;. (8614383)
1996
32
CT and US findings of pancreatoblastoma. (8626892)
1996
33
Cytogenetic and flow cytometric analysis of a pancreatoblastoma. (7889500)
1995
34
Adenocarcinoma of the pancreas in childhood (pancreatoblastoma): report of a case with good response to chemotherapy. (8804461)
1995
35
Pancreatoblastoma. A clinicopathologic study and review of the literature. (7503360)
1995
36
Pancreatoblastoma: a case report. (7954063)
1994
37
Pancreatoblastoma and solid and cystic papillary tumor: two tumors related to pancreatic ontogeny. (7809509)
1994
38
Pancreatoblastoma in an adult: its separation from acinar cell carcinoma. (8032529)
1994
39
CT, MRI, and color Doppler ultrasound correlation of pancreatoblastoma: a case report. (8469598)
1993
40
Pancreatoblastoma--histopathological and ultrastructural analysis of two cases. (1524733)
1992
41
Pancreatoblastoma: response to chemotherapy. (1708850)
1991
42
Use of enzyme histochemistry in the diagnosis of pancreatoblastoma. (2553578)
1989
43
Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association. (2845376)
1988
44
Immunocytochemical markers of uncommon pancreatic tumors. Acinar cell carcinoma, pancreatoblastoma, and solid cystic (papillary-cystic) tumor. (3542187)
1987
45
Immunohistochemical study of pancreatoblastoma. (2449037)
1987
46
Pancreatoblastoma in Japan, with differential diagnosis from papillary cystic tumor (ductuloacinar adenoma) of the pancreas. (3554891)
1987
47
Radiotherapy for locally recurrent infantile pancreatic carcinoma (pancreatoblastoma). (3115557)
1987
48
Alpha-fetoprotein-producing pancreatoblastoma. A case report. (2420440)
1986
49
Pancreatoblastoma in a neonate with Wiedemann-Beckwith syndrome. (3792392)
1986
50
Pancreatoblastoma. A histochemical and ultrastructural analysis. (6141001)
1984

Genetic Variations for Pancreatoblastoma

Expression for genes affiliated with Pancreatoblastoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
54Reactome, 38NCBI BioSystems Database, 4Cell Signaling Technology, 30KEGG, 12EMD Millipore, 53R&D Systems, 52QIAGEN
See all sources

Pathways related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show all 18)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
9.6CTNNB1, APC
29.6CTNNB1, APC
39.3AFP, CTNNB1, APC
4
Hide members
9.2APC, CTNNB1, SMAD4
5
Hide members
9.2APC, CTNNB1, SMAD4
69.2SMAD4, CTNNB1, APC
7
Hide members
9.2SMAD4, CTNNB1, APC
89.2SMAD4, CTNNB1, APC
98.9SMAD4, AFP, CTNNB1, APC
108.6CTNNB1, CLDN5, CLDN3
118.5CLDN5, CLDN3, CLDN7
128.5CLDN7, CLDN3, CLDN5
13
Hide members
8.1CTNNB1, CLDN5, CLDN3, CLDN7
14
Cell adhesion Integrin-mediated cell adhesion and migration
Hide members
8.1CTNNB1, CLDN5, CLDN3, CLDN7
158.1CTNNB1, CLDN5, CLDN3, CLDN7
16
Hide members
8.1CLDN7, CLDN3, CLDN5, CTNNB1
17
Hide members
8.1CTNNB1, CLDN5, CLDN3, CLDN7
188.1CLDN7, CLDN3, CLDN5, CTNNB1

Compounds for genes affiliated with Pancreatoblastoma

Sources:
45Novoseek, 29IUPHAR, 2BitterDB, 50PharmGKB, 11DrugBank, 60Tocris Bioscience, 24HMDB
See all sources

Compounds related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1smcc4510.2SYP, CHGA
2111in-pentetreotide4510.2SYP, CHGA
3s 1004510.2CHGA, SYP
4lithium chloride4510.1CTNNB1, CHGA
5methylazoxymethanol acetate4510.0APC, CTNNB1
61-hydroxyanthraquinone4510.0APC, CTNNB1
7samp4510.0CTNNB1, APC
81,2-dimethylhydrazine4510.0APC, CTNNB1
9azoxymethane4510.0CTNNB1, APC
102-amino-3-methylimidazo(4,5-f)quinoline4510.0CTNNB1, APC
11fatty acid459.9APC, CTNNB1
12b 723459.9AFP, SYP, CHGA
13exisulind459.9APC, CTNNB1
14silver459.9AFP, SYP, CHGA
15l-amino acid459.9CHGA, SYP
16sodium bisulfite459.9CTNNB1, APC
17steroid459.9AFP, SYP, SMAD4
18phip459.9CTNNB1, APC
19gold459.8CHGA, SYP, AFP
20haloperidol45 29 2 50 1113.8CTNNB1, SYP, CHGA
21dexamethasone45 50 29 1112.7CTNNB1, AFP
22bicalutamide45 60 1111.7CHGA, CTNNB1
23epinephrine45 11 2411.7CHGA, AFP
24lithium45 50 11 2412.7SYP, CTNNB1, APC
25tgf beta1459.7SMAD4, AFP, CTNNB1
26sucrose45 11 2411.7CTNNB1, SYP, CHGA
27crcs459.7SMAD4, CTNNB1, APC
28acth459.6CHGA, SYP, APC
29dopamine45 29 11 2412.6CHGA, CTNNB1, APC
30chloramphenicol45 2 1111.6AFP, CHGA, SMAD4
31gnrh459.5SMAD4, CHGA, CTNNB1
32etoposide45 50 60 1112.5CHGA, AFP
33agar459.5CTNNB1, AFP, SMAD4
34cisplatin45 50 60 1112.4CTNNB1, AFP, SYP, CHGA
35hydrogen45 2410.4APC, AFP, SYP, CHGA
36vitamin d459.4CHGA, AFP, CTNNB1, APC
37glutamine459.3SYP, AFP, CTNNB1, APC
38glycogen45 2410.1CHGA, SYP, AFP, CTNNB1, APC
39progesterone45 60 29 11 2413.1APC, CTNNB1, AFP, SYP, CHGA
40cholesterol45 29 11 2411.9AFP, SYP, CHGA, CLDN3
41paraffin458.7APC, CTNNB1, AFP, SYP, CHGA, SMAD4
42testosterone45 60 11 2411.7APC, CTNNB1, AFP, SYP, CHGA, SMAD4
43serine458.6APC, CTNNB1, AFP, SYP, CHGA, SMAD4
44retinoic acid45 249.6KRT15, CHGA, SYP, AFP, CTNNB1, APC
45vegf458.3CTNNB1, AFP, SYP, CHGA, CLDN3, SMAD4
46tyrosine458.2APC, CTNNB1, AFP, SYP, CHGA, KRT15
47oligonucleotide457.9SMAD4, APC, CTNNB1, AFP, CHGA, CLDN5
48calcium45 50 11 2410.8CLDN3, CLDN5, CHGA, SYP, AFP, APC

GO Terms for genes affiliated with Pancreatoblastoma

Sources:
16Gene Ontology
See all sources

Cellular components related to Pancreatoblastoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell-cell adherens junctionGO:0059139.6CTNNB1, APC
2beta-catenin destruction complexGO:0308779.3CTNNB1, APC
3lateral plasma membraneGO:0163289.0APC, CTNNB1, CLDN7
4tight junctionGO:0059238.1CLDN7, CLDN3, CLDN5, APC

Biological processes related to Pancreatoblastoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1SMAD protein signal transductionGO:0603959.8SMAD4, AFP
2gastrulation with mouth forming secondGO:0017029.7CTNNB1, SMAD4
3thymus developmentGO:0485389.6CTNNB1, APC
4proximal/distal pattern formationGO:0099549.5CTNNB1, APC
5T cell differentiation in thymusGO:0330779.3CTNNB1, APC
6calcium-independent cell-cell adhesionGO:0163388.6CLDN7, CLDN3, CLDN5

Molecular functions related to Pancreatoblastoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cadherin bindingGO:0452969.6CTNNB1, APC
2I-SMAD bindingGO:0704119.6SMAD4, CTNNB1
3R-SMAD bindingGO:0704129.4SMAD4, CTNNB1
4structural molecule activityGO:0051988.1CTNNB1, CLDN5, CLDN3, CLDN7
5identical protein bindingGO:0428027.9SMAD4, CLDN7, CLDN3, CLDN5, SYP

Products for genes affiliated with Pancreatoblastoma

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  • Proteins
  • Lysates
  • Antibodies

Sources for Pancreatoblastoma

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet