MCID: PNC019
MIFTS: 37

Pancreatoblastoma

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

Aliases & Classifications for Pancreatoblastoma

MalaCards integrated aliases for Pancreatoblastoma:

Name: Pancreatoblastoma 12 77 54 60 45 15 17 74

Characteristics:

Orphanet epidemiological data:

60
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 Ontology 12 DOID:6823
MeSH 45 C537162
NCIt 51 C4265
SNOMED-CT 69 53618008
MESH via Orphanet 46 C537162
ICD10 via Orphanet 35 C25.1
UMLS via Orphanet 75 C0334489
Orphanet 60 ORPHA677
UMLS 74 C0334489

Summaries for Pancreatoblastoma

MalaCards based summary : Pancreatoblastoma is related to acinar cell carcinoma and ectopic cushing syndrome, and has symptoms including abdominal pain, pruritus and icterus. An important gene associated with Pancreatoblastoma is AFP (Alpha Fetoprotein). The drugs Pancrelipase and Gastrointestinal Agents have been mentioned in the context of this disorder. Affiliated tissues include pancreas, lymph node and pituitary, and related phenotypes are vomiting and abdominal pain

Wikipedia : 77 Pancreatoblastoma is a rare type of pancreatic cancer.It occurs mainly in childhood and has a relatively... more...

Related Diseases for Pancreatoblastoma

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

(show top 50) (show all 172)
# Related Disease Score Top Affiliating Genes
1 acinar cell carcinoma 30.5 AFP CHGA SERPINA1
2 ectopic cushing syndrome 30.2 SST SYP
3 beckwith-wiedemann syndrome 10.4
4 hepatitis a 10.4 AFP SERPINA1
5 mucinous cystadenofibroma 10.3 AFP SERPINA1
6 polyembryoma 10.3 AFP SERPINA1
7 teratoma 10.3 AFP SYP
8 malignant sertoli cell tumor 10.3 AFP SYP
9 endometrial small cell carcinoma 10.3 AFP SYP
10 adult teratoma 10.3 AFP CHGA
11 malignant teratoma 10.3 AFP SYP
12 sclerosing hepatic carcinoma 10.3 AFP CHGA
13 male reproductive organ benign neoplasm 10.3 AFP SYP
14 ovarian endodermal sinus tumor 10.3 AFP SERPINA1
15 mediastinal cancer 10.3 AFP SYP
16 seminal vesicle tumor 10.3 AFP SYP
17 pancreatic acinar cell adenocarcinoma 10.3 AFP CHGA
18 primary pigmented nodular adrenocortical disease 10.3 CHGA SYP
19 large cell carcinoma 10.3 CHGA SYP
20 ovarian primitive germ cell tumor 10.3 AFP SERPINA1
21 liver sarcoma 10.3 AFP CHGA
22 pineoblastoma 10.3 AFP SYP
23 ureter small cell carcinoma 10.3 CHGA SYP
24 small cell carcinoma 10.3 CHGA SYP
25 esophageal neuroendocrine tumor 10.3 CHGA SYP
26 pancreatic cancer 10.3
27 urinary bladder small cell neuroendocrine carcinoma 10.3 CHGA SYP
28 ovarian large-cell neuroendocrine carcinoma 10.3 CHGA SYP
29 auditory system cancer 10.3 CHGA SYP
30 peritoneal serous adenocarcinoma 10.3 CHGA SYP
31 cellular ependymoma 10.3 CHGA SYP
32 primary hepatic neuroendocrine carcinoma 10.3 CHGA SYP
33 small cell carcinoma of the bladder 10.3 CHGA SYP
34 pulmonary large cell neuroendocrine carcinoma 10.2 CHGA SYP
35 large cell neuroendocrine carcinoma 10.2 CHGA SYP
36 gallbladder small cell carcinoma 10.2 CHGA SYP
37 atypical follicular adenoma 10.2 CHGA SYP
38 olfactory groove meningioma 10.2 CHGA SYP
39 cauda equina neoplasm 10.2 CHGA SYP
40 bile duct cystadenocarcinoma 10.2 AFP MUC1
41 pancreatic serous cystadenoma 10.2 CHGA SYP
42 oncocytoma 10.2 MUC1 SYP
43 conventional angiosarcoma 10.2 CHGA SYP
44 intestinal pseudo-obstruction 10.2 SST SYP
45 thymus cancer 10.2 MUC1 SYP
46 cavernous hemangioma 10.2 MUC1 SYP
47 hemangioblastoma 10.2 MUC1 SYP
48 hepatoid adenocarcinoma 10.2 AFP MUC1
49 olfactory nerve neoplasm 10.2 CHGA SYP
50 autonomic nervous system benign neoplasm 10.2 CHGA SYP

Graphical network of the top 20 diseases related to Pancreatoblastoma:



Diseases related to Pancreatoblastoma

Symptoms & Phenotypes for Pancreatoblastoma

Human phenotypes related to Pancreatoblastoma:

60 33 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 vomiting 60 33 frequent (33%) Frequent (79-30%) HP:0002013
2 abdominal pain 60 33 frequent (33%) Frequent (79-30%) HP:0002027
3 weight loss 60 33 frequent (33%) Frequent (79-30%) HP:0001824
4 diarrhea 60 33 frequent (33%) Frequent (79-30%) HP:0002014
5 abdominal distention 60 33 frequent (33%) Frequent (79-30%) HP:0003270
6 pancreatic calcification 60 33 frequent (33%) Frequent (79-30%) HP:0005213
7 elevated maternal serum alpha-fetoprotein 60 33 frequent (33%) Frequent (79-30%) HP:0005984
8 jaundice 60 33 occasional (7.5%) Occasional (29-5%) HP:0000952
9 abnormality of the lymph nodes 60 33 occasional (7.5%) Occasional (29-5%) HP:0002733

UMLS symptoms related to Pancreatoblastoma:


abdominal pain, pruritus, icterus

Drugs & Therapeutics for Pancreatoblastoma

Drugs for Pancreatoblastoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Pancrelipase Approved, Investigational 53608-75-6
2 Gastrointestinal Agents
3 pancreatin

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Incidence of POPF in the Resection of the Left Pancreas With RFAT Recruiting NCT03570502

Search NIH Clinical Center for Pancreatoblastoma

Cochrane evidence based reviews: pancreatoblastoma

Genetic Tests for Pancreatoblastoma

Anatomical Context for Pancreatoblastoma

MalaCards organs/tissues related to Pancreatoblastoma:

42
Pancreas, Lymph Node, Pituitary, Colon, Brain, Kidney, Thymus

Publications for Pancreatoblastoma

Articles related to Pancreatoblastoma:

(show top 50) (show all 136)
# Title Authors Year
1
Pancreatoblastoma: an Atypical Presentation and a Literature Review. ( 28194556 )
2018
2
Integrated Molecular Characterization of the Lethal Pediatric Cancer Pancreatoblastoma. ( 29233928 )
2018
3
Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant. ( 29235221 )
2018
4
Pancreatoblastoma: an unusual diagnosis in an adult patient. ( 29285688 )
2018
5
Identification and characterization of a novel adenomatous polyposis coli mutation in adult pancreatoblastoma. ( 29535845 )
2018
6
Adult pancreatoblastoma: A case report and clinicopathological review of the literature. ( 29753278 )
2018
7
Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases. ( 30174723 )
2018
8
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. ( 28082787 )
2017
9
Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report. ( 28538503 )
2017
10
Pancreatoblastoma With Metastatic Retroperitoneal Lymph Node and PET/CT. ( 28872552 )
2017
11
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. ( 25110242 )
2017
12
Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. ( 27046982 )
2016
13
Fine needle aspiration cytology diagnosis of a pancreatoblastoma in an infant: case report with a summary of prior published cases. ( 27038102 )
2016
14
Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report. ( 27134883 )
2016
15
Multi-Visceral Transplantation in a 21-Year-Old Man with Prior Pancreatoblastoma. ( 27721967 )
2016
16
Adult pancreatoblastoma - Case report and review of literature. ( 27761191 )
2016
17
Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt. ( 26881602 )
2015
18
Pancreatoblastoma in an adult. ( 25673604 )
2015
19
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. ( 26056794 )
2015
20
Adult pancreatoblastoma: A case report and review of the literature. ( 26137059 )
2015
21
Clinicopathological review of pancreatoblastoma in adults. ( 26312218 )
2015
22
Multifocal pancreatoblastoma with pseudocyst in a 2-year-old boy. ( 26591960 )
2015
23
Infantile extrapancreatic pancreatoblastoma: a report on a rare infantile abdominal mass. ( 24072247 )
2014
24
Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. ( 23042010 )
2013
25
Spontaneous rupture of an advanced pancreatoblastoma: aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers. ( 23583162 )
2013
26
Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma. ( 23741195 )
2013
27
CT-guided radiofrequency ablation following high-dose chemotherapy of a liver-metastasizing pancreatoblastoma with tumor thrombus in the portal vein. ( 23828484 )
2013
28
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. ( 23991735 )
2013
29
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. ( 24081396 )
2013
30
Congenital pancreatoblastoma associated with β-catenin mutation. ( 21953767 )
2012
31
Congenital pancreatoblastoma: report of an atypical case and review of the literature. ( 22278199 )
2012
32
Management of pancreatoblastoma in children and young adults. ( 22525406 )
2012
33
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. ( 22572137 )
2012
34
Clinical analysis of childhood pancreatoblastoma arising from the tail of the pancreas. ( 22584784 )
2012
35
Exocrine Pancreatic Neoplasms of Nonductal Origin: Acinar Cell Carcinoma, Pancreatoblastoma, and Solid-Pseudopapillary Neoplasm. ( 26837489 )
2011
36
Synchronous ectopic pancreatoblastoma in a child: a case report. ( 21655073 )
2011
37
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). ( 21696948 )
2011
38
Central pancreatectomy for pancreatoblastoma in a 16-year-old girl. ( 21843703 )
2011
39
Simultaneous occurrence of pancreatoblastoma and neuroblastoma in a newborn with beckwith-wiedemann syndrome. ( 20495482 )
2010
40
Pancreatoblastoma: two case reports from a medical center in Taiwan. ( 20057322 )
2010
41
Pancreaticoduodenectomy for pancreatoblastoma: a case report and literature review. ( 20165952 )
2010
42
Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. ( 20632017 )
2010
43
Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma. ( 20658635 )
2010
44
Corticotropin-releasing hormone-secreting pancreatoblastoma in an adult patient. ( 20664482 )
2010
45
Imaging findings of pancreatoblastoma in 4 children including a case of ectopic pancreatoblastoma. ( 20686762 )
2010
46
Pancreatoblastoma in adults: a review of the literature. ( 19077457 )
2009
47
Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. ( 19194274 )
2009
48
Imprint cytology of pancreatoblastoma: a case report and review of the literature. ( 19229998 )
2009
49
Fluorescence in situ hybridization analysis of pancreatoblastoma. ( 19238024 )
2009
50
Pancreatoblastoma in an adult patient. A case report. ( 19287117 )
2009

Variations for Pancreatoblastoma

Expression for Pancreatoblastoma

Search GEO for disease gene expression data for Pancreatoblastoma.

Pathways for Pancreatoblastoma

GO Terms for Pancreatoblastoma

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.35 AFP CHGA MUC1 SERPINA1 SST
2 extracellular region GO:0005576 9.1 AFP CHGA MUC1 NTS SERPINA1 SST

Sources for Pancreatoblastoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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