MCID: PNC019
MIFTS: 46

Pancreatoblastoma malady

Gastrointestinal diseases, Cancer diseases, Endocrine diseases categories

Summaries for Pancreatoblastoma

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63Wikipedia, 32MalaCards
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Wikipedia:63 Pancreatoblastoma is a rare tumor of the pancreas. more...

MalaCards: Pancreatoblastoma is related to pancreatitis and acinar cell carcinoma. 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 pancreas, liver and brain, and related mouse phenotypes are endocrine/exocrine gland and renal/urinary system.

Aliases & Classifications for Pancreatoblastoma

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 48Orphanet, 60UMLS, 56SNOMED-CT, 39NCIt, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

pancreatoblastoma 8 42 48 60


External Ids:

Disease Ontology8 DOID:6823
NCIt39 C4265
MeSH34 C537162
MESH via Orphanet35 C537162
SNOMED-CT56 53618008, 189814006
ICD10 via Orphanet26 C25.1
SNOMED-CT via Orphanet57 53618008
UMLS via Orphanet61 C0334489

Related Diseases for Pancreatoblastoma

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Sources:
17GeneCards, 18GeneDecks
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Diseases related to Pancreatoblastoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 75)
idRelated DiseaseScoreTop Affiliating Genes
1pancreatitis30.6SMAD4
2acinar cell carcinoma30.2CTNNB1, AFP, CHGA
3neuroblastoma29.9SYP
4papillary carcinoma29.9CLDN3, CHGA, SYP, SMAD4
5familial adenomatous polyposis29.9CTNNB1, APC, SMAD4
6adenoma29.9CTNNB1, SMAD4, CHGA, CLDN3, SYP, APC
7adenocarcinoma29.8CHGA, SYP, AFP, APC, CLDN5, CTNNB1
8beckwith-wiedemann syndrome10.3
9neurofibroma10.0SYP
10rhabdoid tumor10.0SYP
11hepatitis10.0AFP
12ependymoma10.0SYP
13parathyroid adenoma10.0CHGA
14neuroendocrine tumor10.0CHGA
15prolactinoma10.0CHGA
16dysembryoplastic neuroepithelial tumor10.0SYP
17hepatitis c10.0AFP
18hypercalcemia10.0CHGA
19ganglioneuroblastoma10.0SYP
20gallbladder cancer10.0APC
21meningioma10.0SYP
22multiple endocrine neoplasia10.0CHGA
23glioblastoma multiforme10.0SMAD4
24germinoma10.0AFP
25sertoli-leydig cell tumor10.0AFP
26sertoli cell tumor10.0SYP
27wilms tumor10.0CTNNB1
28nephroblastoma10.0CTNNB1
29granular cell tumor10.0SYP
30lung cancer10.0SYP, CHGA
31central neurocytoma10.0CHGA, SYP
32melanoma10.0CTNNB1
33endocrine gland cancer10.0SYP, CHGA
34pineocytoma10.0SYP, CHGA
35merkel cell carcinoma10.0CHGA, SYP
36pituitary tumors10.0APC, CHGA
37juvenile polyposis syndrome10.0SMAD4, APC
38large cell carcinoma10.0CHGA, SYP
39seminoma10.0SMAD4, AFP
40pancreatic ductal carcinoma10.0SMAD4, CHGA
41vipoma10.0SMAD4, CHGA
42pancreatic islet cell tumors10.0SYP, CHGA
43oxyphilic adenoma10.0SYP, CLDN7
44gardner syndrome10.0CTNNB1, APC
45medulloblastoma10.0APC, CTNNB1
46peutz-jeghers syndrome10.0CTNNB1, APC
47intestinal neoplasm10.0APC, CTNNB1
48malignant mixed mullerian tumor10.0AFP, CTNNB1
49turcot syndrome10.0APC, CTNNB1
50cholangiocarcinoma10.0SMAD4, AFP

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to pancreatoblastoma

Clinical Features for Pancreatoblastoma

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Drugs & Therapeutics for Pancreatoblastoma

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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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

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Anatomical Context for Pancreatoblastoma

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Sources:
32MalaCards
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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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Sources:
36MGI
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MGI Mouse Phenotypes related to Pancreatoblastoma:

36
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

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Sources:
50PubMed
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Articles related to Pancreatoblastoma:

(show top 50)    (show all 112)
idTitleAuthorsYear
1
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. (23991735)
2013
2
Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma. (23741195)
2013
3
Infantile Extrapancreatic Pancreatoblastoma: A Report on a Rare Infantile Abdominal Mass. (24072247)
2013
4
Spontaneous rupture of an advanced pancreatoblastoma: aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers. (23583162)
2013
5
Management of pancreatoblastoma in children and young adults. (22525406)
2012
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
Simultaneous occurrence of pancreatoblastoma and neuroblastoma in a newborn with beckwith-wiedemann syndrome. (20495482)
2010
9
Pancreatoblastoma: two case reports from a medical center in Taiwan. (20057322)
2010
10
Pancreaticoduodenectomy for pancreatoblastoma: a case report and literature review. (20165952)
2010
11
Pancreatoblastoma in an adult patient. A case report. (19287117)
2009
12
Imprint cytology of pancreatoblastoma: a case report and review of the literature. (19229998)
2009
13
Pancreatoblastoma in adults: a review of the literature. (19077457)
2009
14
A peculiar variant of pancreatoblastoma in an adult. (18362850)
2008
15
Long-term survival of pancreatoblastoma in children. (18090935)
2007
16
Adrenocorticotropin-secreting pancreatoblastoma. (17642425)
2007
17
Pancreatoblastoma. (17228135)
2007
18
Pancreatoblastoma in an adult: case report and review of the literature. (16769539)
2006
19
Pancreatoblastoma in a teenage patient. (16728924)
2006
20
A case of pancreatoblastoma prenatally diagnosed as intraperitoneal cyst. (16896817)
2006
21
Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature. (16080945)
2005
22
Fine-needle aspiration cytology of pancreatoblastoma in a young woman: report of a case and review of the literature. (16138370)
2005
23
Septal enhancement within pancreatoblastoma of the pancreatic head presenting with progressive anaemia. (15205844)
2004
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
Adult pancreatoblastoma. A case report. (11859308)
2002
27
Pancreatoblastoma. (11770246)
2001
28
Prolonged survival after resection of pancreatoblastoma and synchronous liver metastases in an adult. (11677959)
2001
29
Pancreatoblastoma: ultrastructural and image DNA cytometric analysis. (11244266)
2001
30
Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients. (11466723)
2001
31
Alpha-fetoprotein variants in a case of pancreatoblastoma. (11026522)
2000
32
Sonography and CT of pancreatoblastoma in children. (10845499)
2000
33
Pancreatoblastoma: imaging findings in 10 patients and review of the literature. (10671596)
2000
34
Pancreatoblastoma. Three original cases and review of the literature. (10937753)
2000
35
Pancreatoblastoma: optically clear nuclei in squamoid corpuscles are rich in biotin. (9796720)
1998
36
Pancreatoblastoma. (9396538)
1997
37
Pancreatoblastoma in children. Case report and review of the literature. (9007475)
1996
38
Adult pancreatoblastoma: a case report and review of the literature. (8792711)
1996
39
Pancreatoblastoma: case report and review of treatment in the literature. (8699999)
1996
40
Pancreatoblastoma in an older adult. (7605173)
1995
41
CT, MRI, and color Doppler ultrasound correlation of pancreatoblastoma: a case report. (8469598)
1993
42
Pancreatoblastoma with marked elevation of serum alpha-fetoprotein. An autopsy case report with immunocytochemical study. (1689089)
1990
43
Fine needle aspiration cytology of pancreatoblastoma with immunocytochemical and ultrastructural studies. (2220242)
1990
44
Use of enzyme histochemistry in the diagnosis of pancreatoblastoma. (2553578)
1989
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
Pancreatoblastoma in an adult. (3013120)
1986
48
Pancreatoblastoma. A histochemical and ultrastructural analysis. (6141001)
1984
49
Diagnosis of human tumors. Case 2: Pancreatoblastoma mimicking fibrolamellar hepatocarcinoma. (6326359)
1983
50
Morphogenesis of pancreatoblastoma, infantile carcinoma of the pancreas: report of two cases. (188539)
1977

Genetic Variations for Pancreatoblastoma

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Expression for genes affiliated with Pancreatoblastoma

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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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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Sources:
53Reactome, 37NCBI BioSystems Database, 4Cell Signaling Technology, 29KEGG, 12EMD Millipore, 52R&D Systems, 51QIAGEN
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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

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Sources:
44Novoseek, 28IUPHAR, 2BitterDB, 49PharmGKB, 11DrugBank, 59Tocris Bioscience, 24HMDB
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Compounds related to Pancreatoblastoma according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1smcc4410.2SYP, CHGA
2111in-pentetreotide4410.2SYP, CHGA
3s 1004410.2CHGA, SYP
4lithium chloride4410.1CTNNB1, CHGA
5methylazoxymethanol acetate4410.0APC, CTNNB1
61-hydroxyanthraquinone4410.0APC, CTNNB1
7samp4410.0CTNNB1, APC
81,2-dimethylhydrazine4410.0APC, CTNNB1
9azoxymethane4410.0CTNNB1, APC
102-amino-3-methylimidazo(4,5-f)quinoline4410.0CTNNB1, APC
11fatty acid449.9APC, CTNNB1
12b 723449.9AFP, SYP, CHGA
13exisulind449.9APC, CTNNB1
14silver449.9AFP, SYP, CHGA
15l-amino acid449.9CHGA, SYP
16sodium bisulfite449.9CTNNB1, APC
17steroid449.9AFP, SYP, SMAD4
18phip449.9CTNNB1, APC
19gold449.8CHGA, SYP, AFP
20haloperidol44 28 2 49 1113.8CTNNB1, SYP, CHGA
21dexamethasone44 49 28 1112.7CTNNB1, AFP
22bicalutamide44 59 1111.7CHGA, CTNNB1
23epinephrine44 11 2411.7CHGA, AFP
24lithium44 49 11 2412.7SYP, CTNNB1, APC
25tgf beta1449.7SMAD4, AFP, CTNNB1
26sucrose44 11 2411.7CTNNB1, SYP, CHGA
27crcs449.7SMAD4, CTNNB1, APC
28acth449.6CHGA, SYP, APC
29dopamine44 28 11 2412.6CHGA, CTNNB1, APC
30chloramphenicol44 2 1111.6AFP, CHGA, SMAD4
31gnrh449.5SMAD4, CHGA, CTNNB1
32etoposide44 49 59 1112.5CHGA, AFP
33agar449.5CTNNB1, AFP, SMAD4
34cisplatin44 49 59 1112.4CTNNB1, AFP, SYP, CHGA
35hydrogen44 2410.4APC, AFP, SYP, CHGA
36vitamin d449.4CHGA, AFP, CTNNB1, APC
37glutamine449.3SYP, AFP, CTNNB1, APC
38glycogen44 2410.1CHGA, SYP, AFP, CTNNB1, APC
39progesterone44 59 28 11 2413.1APC, CTNNB1, AFP, SYP, CHGA
40cholesterol44 28 11 2411.9AFP, SYP, CHGA, CLDN3
41paraffin448.7APC, CTNNB1, AFP, SYP, CHGA, SMAD4
42testosterone44 59 11 2411.7APC, CTNNB1, AFP, SYP, CHGA, SMAD4
43serine448.6APC, CTNNB1, AFP, SYP, CHGA, SMAD4
44retinoic acid44 249.6KRT15, CHGA, SYP, AFP, CTNNB1, APC
45vegf448.3CTNNB1, AFP, SYP, CHGA, CLDN3, SMAD4
46tyrosine448.2APC, CTNNB1, AFP, SYP, CHGA, KRT15
47oligonucleotide447.9SMAD4, APC, CTNNB1, AFP, CHGA, CLDN5
48calcium44 49 11 2410.8CLDN3, CLDN5, CHGA, SYP, AFP, APC

GO Terms for genes affiliated with Pancreatoblastoma

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16Gene Ontology
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Cellular components related to Pancreatoblastoma according to GeneCards/GeneDecks:

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

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

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
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet