MCID: PNC019
MIFTS: 32

Pancreatoblastoma malady

Categories: Rare diseases, Gastrointestinal diseases, Cancer diseases, Endocrine diseases

Aliases & Classifications for Pancreatoblastoma

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Sources:
11Disease Ontology, 13DISEASES, 31ICD10 via Orphanet, 39MeSH, 40MESH via Orphanet, 45NCIt, 48NIH Rare Diseases, 54Orphanet, 62SNOMED-CT, 68UMLS, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Pancreatoblastoma:

Name: Pancreatoblastoma 11 48 54 39 13 68

Characteristics:

Orphanet epidemiological data:

54
pancreatoblastoma:
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),<1/1000000 (Worldwide); Age of onset: Adult,Childhood; Age of death: adult,early childhood,late childhood

Classifications:



External Ids:

Disease Ontology11 DOID:6823
MeSH39 C537162
NCIt45 C4265
Orphanet54 ORPHA677
SNOMED-CT62 189814006, 53618008
MESH via Orphanet40 C537162
UMLS via Orphanet69 C0334489
ICD10 via Orphanet31 C25.1

Summaries for Pancreatoblastoma

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Wikipedia:71 Pancreatoblastoma is a rare type of pancreatic cancer. more...

MalaCards based summary: Pancreatoblastoma is related to rete testis adenoma and pontine hemorrhage, and has symptoms including abdominal pain, icterus and pruritus. An important gene associated with Pancreatoblastoma is AFP (Alpha Fetoprotein), and among its related pathways is Glucocorticoid receptor regulatory network. Affiliated tissues include pancreas, liver and brain, and related mouse phenotype Increased shRNA abundance.

Related Diseases for Pancreatoblastoma

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

(show top 50)    (show all 144)
idRelated DiseaseScoreTop Affiliating Genes
1rete testis adenoma10.3AFP, SERPINA1
2pontine hemorrhage10.3AFP, SERPINA1
3infiltrating renal pelvis transitional cell carcinoma10.3AFP, SYP
4proliferative glomerulonephritis10.3AFP, SYP
5ovarian germ cell teratoma10.3AFP, SYP
6vipoma10.3AFP, CHGA
7kidney disease10.3AFP, SYP
8rectum leiomyoma10.3AFP, SYP
9malignant granular cell myoblastoma10.3AFP, CHGA
10nipple neoplasm10.3AFP, SYP
11uterine corpus serous adenocarcinoma10.2AFP, CHGA
12medullomyoblastoma10.2AFP, SYP
13pneumothorax10.2AFP, SYP
14retroperitoneal hemangiopericytoma10.2AFP, SERPINA1
15pigmented villonodular synovitis10.2AFP, CHGA
16peroneal nerve paralysis10.2CHGA, SYP
17thymus small cell carcinoma10.2CHGA, SYP
18brain stem ependymoma10.2CHGA, SYP
19non specific chronic endometritis10.2CHGA, SYP
20cellular ependymoma10.2AFP, SERPINA1
21prolactin producing pituitary tumor10.2CHGA, SYP
22epithelioid malignant peripheral nerve sheath tumor10.2CHGA, SYP
23cartilage cancer10.2CHGA, SYP
24arthus reaction10.2AFP, SYP
25villous adenocarcinoma10.2CHGA, SYP
26jejunal neuroendocrine tumor10.2CHGA, SYP
27renal pelvis adenocarcinoma10.2CHGA, SYP
28spasmodic dysphonia10.2CHGA, SYP
29barre-lieou syndrome10.2CHGA, SYP
30microinvasive cervical squamous cell carcinoma10.2CHGA, SYP
31perineural angioma10.2CHGA, SYP
32gastric duplication cysts10.2CHGA, SYP
33diffuse cutaneous mastocytosis10.2CHGA, SYP
34hair follicle neoplasm10.2AFP, SERPINA1
35main bronchus cancer10.2CHGA, SYP
36gliofibroma10.2CHGA, SYP
37cavernous hemangioma of face10.2CHGA, SYP
38superior vena cava syndrome10.2CHGA, SYP
39exudative glomerulonephritis10.2CHGA, SYP
40fragile x-associated tremor/ataxia syndrome10.2CHGA, SYP
41hepatorenal syndrome10.2CHGA, SYP
42acalculous cholecystitis10.2CHGA, SYP
43postgastrectomy syndrome10.2SST, SYP
44piussan lenaerts mathieu syndrome10.2CHGA, SYP
45brain stem astrocytic neoplasm10.2CHGA, SYP
46anal squamous cell carcinoma10.2CHGA, SYP
47sclerosing hemangioma10.2AFP, CHGA
48pemphigus foliaceus10.2SST, SYP
49uterine corpus myxoid leiomyosarcoma10.2CHGA, SYP
50calcific tendinitis10.2CHGA, SYP

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to pancreatoblastoma

Symptoms & Phenotypes for Pancreatoblastoma

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Human phenotypes related to Pancreatoblastoma:

 54 64 (show all 6)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 weight loss64 54 Frequent (79-30%) HP:0001824
2 vomiting64 54 Frequent (79-30%) HP:0002013
3 diarrhea64 54 Frequent (79-30%) HP:0002014
4 abdominal pain64 54 Frequent (79-30%) HP:0002027
5 elevated maternal serum alpha-fetoprotein64 54 Frequent (79-30%) HP:0005984
6 jaundice64 54 Occasional (29-5%) HP:0000952

UMLS symptoms related to Pancreatoblastoma:


abdominal pain, icterus, pruritus

GenomeRNAi Phenotypes related to Pancreatoblastoma according to GeneCards Suite gene sharing:

26
idDescriptionGenomeRNAi Source AccessionScoreTop Affiliating Genes
1GR00327-A8.8AFP, KRT14, KRT17

Drugs & Therapeutics for Pancreatoblastoma

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Pancreatoblastoma


Cochrane evidence based reviews: pancreatoblastoma

Genetic Tests for Pancreatoblastoma

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Anatomical Context for Pancreatoblastoma

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MalaCards organs/tissues related to Pancreatoblastoma:

36
Pancreas, Liver, Brain

Publications for Pancreatoblastoma

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Articles related to Pancreatoblastoma:

(show top 50)    (show all 125)
idTitleAuthorsYear
1
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. (28082787)
2017
2
Fine needle aspiration cytology diagnosis of a pancreatoblastoma in an infant: case report with a summary of prior published cases. (27038102)
2016
3
Multi-Visceral Transplantation in a 21-Year-Old Man with Prior Pancreatoblastoma. (27721967)
2016
4
Adult pancreatoblastoma - Case report and review of literature. (27761191)
2016
5
Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report. (27134883)
2016
6
Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. (27046982)
2016
7
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. (26056794)
2015
8
Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt. (26881602)
2015
9
Adult pancreatoblastoma: A case report and review of the literature. (26137059)
2015
10
Pancreatoblastoma in an adult. (25673604)
2015
11
Clinicopathological review of pancreatoblastoma in adults. (26312218)
2015
12
Multifocal pancreatoblastoma with pseudocyst in a 2-year-old boy. (26591960)
2015
13
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. (25110242)
2014
14
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. (23991735)
2013
15
Infantile Extrapancreatic Pancreatoblastoma: A Report on a Rare Infantile Abdominal Mass. (24072247)
2013
16
Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. (23042010)
2013
17
Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma. (23741195)
2013
18
CT-guided radiofrequency ablation following high-dose chemotherapy of a liver-metastasizing pancreatoblastoma with tumor thrombus in the portal vein. (23828484)
2013
19
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. (24081396)
2013
20
Spontaneous rupture of an advanced pancreatoblastoma: aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers. (23583162)
2013
21
Congenital pancreatoblastoma: report of an atypical case and review of the literature. (22278199)
2012
22
Clinical analysis of childhood pancreatoblastoma arising from the tail of the pancreas. (22584784)
2012
23
Management of pancreatoblastoma in children and young adults. (22525406)
2012
24
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. (22572137)
2012
25
Congenital pancreatoblastoma associated with I^-catenin mutation. (21953767)
2012
26
Central pancreatectomy for pancreatoblastoma in a 16-year-old girl. (21843703)
2011
27
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). (21696948)
2011
28
Synchronous ectopic pancreatoblastoma in a child: a case report. (21655073)
2011
29
Corticotropin-releasing hormone-secreting pancreatoblastoma in an adult patient. (20664482)
2010
30
Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma. (20658635)
2010
31
Simultaneous occurrence of pancreatoblastoma and neuroblastoma in a newborn with beckwith-wiedemann syndrome. (20495482)
2010
32
Imaging findings of pancreatoblastoma in 4 children including a case of ectopic pancreatoblastoma. (20686762)
2010
33
Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. (20632017)
2010
34
Pancreaticoduodenectomy for pancreatoblastoma: a case report and literature review. (20165952)
2010
35
Pancreatoblastoma: two case reports from a medical center in Taiwan. (20057322)
2010
36
Pancreatoblastoma in an adult patient. A case report. (19287117)
2009
37
Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. (19194274)
2009
38
Pancreatoblastoma in adults: a review of the literature. (19077457)
2009
39
Imprint cytology of pancreatoblastoma: a case report and review of the literature. (19229998)
2009
40
Fluorescence in situ hybridization analysis of pancreatoblastoma. (19238024)
2009
41
Childhood pancreatoblastoma: clinical features and immunohistochemistry analysis. (18308466)
2008
42
A peculiar variant of pancreatoblastoma in an adult. (18362850)
2008
43
Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. (18981556)
2008
44
Pancreatoblastoma. (17228135)
2007
45
Diagnosis and treatment of pancreatoblastoma in China. (17198189)
2007
46
Adrenocorticotropin-secreting pancreatoblastoma. (17642425)
2007
47
Pancreatoblastoma metastases to the brain. Case illustration. (17330549)
2007
48
Long-term survival of pancreatoblastoma in children. (18090935)
2007
49
Pancreatoblastoma in a teenage patient. (16728924)
2006
50
A case of pancreatoblastoma prenatally diagnosed as intraperitoneal cyst. (16896817)
2006

Variations for Pancreatoblastoma

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Expression for genes affiliated with Pancreatoblastoma

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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 Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
18.8AFP, KRT14, KRT17

GO Terms for genes affiliated with Pancreatoblastoma

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Cellular components related to Pancreatoblastoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cell peripheryGO:00719449.1KRT14, KRT17

Sources for Pancreatoblastoma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet