MCID: PNC019
MIFTS: 36

Pancreatoblastoma

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

Aliases & Classifications for Pancreatoblastoma

MalaCards integrated aliases for Pancreatoblastoma:

Name: Pancreatoblastoma 12 76 53 59 44 15 73

Characteristics:

Orphanet epidemiological data:

59
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 44 C537162
NCIt 50 C4265
SNOMED-CT 68 53618008
Orphanet 59 ORPHA677
MESH via Orphanet 45 C537162
UMLS via Orphanet 74 C0334489
ICD10 via Orphanet 34 C25.1
UMLS 73 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 liver, and related phenotypes are vomiting and abdominal pain

Wikipedia : 76 Pancreatoblastoma is a rare type of pancreatic... more...

Related Diseases for Pancreatoblastoma

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

(show top 50) (show all 175)
# Related Disease Score Top Affiliating Genes
1 acinar cell carcinoma 30.4 AFP CHGA SERPINA1
2 ectopic cushing syndrome 30.1 SST SYP
3 adenoma of the pancreas 29.9 CHGA SST SYP
4 beckwith-wiedemann syndrome 10.3
5 mucinous cystadenofibroma 10.2 AFP SERPINA1
6 polyembryoma 10.2 AFP SERPINA1
7 teratoma 10.2 AFP SYP
8 malignant sertoli cell tumor 10.2 AFP SYP
9 endometrial small cell carcinoma 10.2 AFP SYP
10 adult teratoma 10.2 AFP CHGA
11 malignant teratoma 10.2 AFP SYP
12 sclerosing hepatic carcinoma 10.2 AFP CHGA
13 male reproductive organ benign neoplasm 10.2 AFP SYP
14 ovarian endodermal sinus tumor 10.2 AFP SERPINA1
15 mediastinal cancer 10.2 AFP SYP
16 seminal vesicle tumor 10.2 AFP SYP
17 pancreatic acinar cell adenocarcinoma 10.2 AFP CHGA
18 primary pigmented nodular adrenocortical disease 10.2 CHGA SYP
19 large cell carcinoma 10.2 CHGA SYP
20 liver sarcoma 10.2 AFP CHGA
21 ovarian primitive germ cell tumor 10.2 AFP SERPINA1
22 pineoblastoma 10.2 AFP SYP
23 ureter small cell carcinoma 10.2 CHGA SYP
24 small cell carcinoma 10.2 CHGA SYP
25 esophageal neuroendocrine tumor 10.2 CHGA SYP
26 urinary bladder small cell neuroendocrine carcinoma 10.2 CHGA SYP
27 ovarian large-cell neuroendocrine carcinoma 10.2 CHGA SYP
28 pneumothorax 10.2 MUC1 SERPINA1
29 auditory system cancer 10.2 CHGA SYP
30 peritoneal serous adenocarcinoma 10.2 CHGA SYP
31 cellular ependymoma 10.2 CHGA SYP
32 gallbladder small cell carcinoma 10.2 CHGA SYP
33 primary hepatic neuroendocrine carcinoma 10.2 CHGA SYP
34 small cell carcinoma of the bladder 10.2 CHGA SYP
35 pulmonary large cell neuroendocrine carcinoma 10.2 CHGA SYP
36 large cell neuroendocrine 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 oncocytoma 10.2 MUC1 SYP
42 pancreatic serous cystadenoma 10.2 CHGA SYP
43 intestinal pseudo-obstruction 10.2 SST SYP
44 thymus cancer 10.2 MUC1 SYP
45 conventional angiosarcoma 10.2 CHGA SYP
46 cavernous hemangioma 10.2 MUC1 SYP
47 hemangioblastoma 10.2 MUC1 SYP
48 neurofibroma 10.2 MUC1 SYP
49 hepatoid adenocarcinoma 10.2 AFP MUC1
50 olfactory nerve 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:

59 32 (show all 9)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002013
2 abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002027
3 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
4 jaundice 59 32 occasional (7.5%) Occasional (29-5%) HP:0000952
5 diarrhea 59 32 frequent (33%) Frequent (79-30%) HP:0002014
6 abdominal distention 59 32 frequent (33%) Frequent (79-30%) HP:0003270
7 abnormality of the lymph nodes 59 32 occasional (7.5%) Occasional (29-5%) HP:0002733
8 pancreatic calcification 59 32 frequent (33%) Frequent (79-30%) HP:0005213
9 elevated maternal serum alpha-fetoprotein 59 32 frequent (33%) Frequent (79-30%) HP:0005984

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:

41
Pancreas, Lymph Node, Liver, Lung, Colon, Pituitary, Thymus

Publications for Pancreatoblastoma

Articles related to Pancreatoblastoma:

(show top 50) (show all 136)
# Title Authors Year
1
Integrated Molecular Characterization of the Lethal Pediatric Cancer Pancreatoblastoma. ( 29233928 )
2018
2
Adult pancreatoblastoma: A case report and clinicopathological review of the literature. ( 29753278 )
2018
3
Identification and characterization of a novel <i>adenomatous polyposis coli</i> mutation in adult pancreatoblastoma. ( 29535845 )
2018
4
Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant. ( 29235221 )
2018
5
Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases. ( 30174723 )
2018
6
Pancreatoblastoma With Metastatic Retroperitoneal Lymph Node and PET/CT. ( 28872552 )
2017
7
Pancreatoblastoma: an Atypical Presentation and a Literature Review. ( 28194556 )
2017
8
Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report. ( 28538503 )
2017
9
Pancreatoblastoma: an unusual diagnosis in an adult patient. ( 29285688 )
2017
10
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. ( 28082787 )
2017
11
Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. ( 27046982 )
2016
12
Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report. ( 27134883 )
2016
13
Multi-Visceral Transplantation in a 21-Year-Old Man with Prior Pancreatoblastoma. ( 27721967 )
2016
14
Fine needle aspiration cytology diagnosis of a pancreatoblastoma in an infant: case report with a summary of prior published cases. ( 27038102 )
2016
15
Adult pancreatoblastoma - Case report and review of literature. ( 27761191 )
2016
16
Adult pancreatoblastoma: A case report and review of the literature. ( 26137059 )
2015
17
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. ( 26056794 )
2015
18
Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt. ( 26881602 )
2015
19
Clinicopathological review of pancreatoblastoma in adults. ( 26312218 )
2015
20
Multifocal pancreatoblastoma with pseudocyst in a 2-year-old boy. ( 26591960 )
2015
21
Pancreatoblastoma in an adult. ( 25673604 )
2015
22
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. ( 25110242 )
2014
23
Spontaneous rupture of an advanced pancreatoblastoma: aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers. ( 23583162 )
2013
24
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. ( 23991735 )
2013
25
Infantile Extrapancreatic Pancreatoblastoma: A Report on a Rare Infantile Abdominal Mass. ( 24072247 )
2013
26
Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma. ( 23741195 )
2013
27
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. ( 24081396 )
2013
28
CT-guided radiofrequency ablation following high-dose chemotherapy of a liver-metastasizing pancreatoblastoma with tumor thrombus in the portal vein. ( 23828484 )
2013
29
Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. ( 23042010 )
2013
30
Congenital pancreatoblastoma: report of an atypical case and review of the literature. ( 22278199 )
2012
31
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. ( 22572137 )
2012
32
Clinical analysis of childhood pancreatoblastoma arising from the tail of the pancreas. ( 22584784 )
2012
33
Congenital pancreatoblastoma associated with I^-catenin mutation. ( 21953767 )
2012
34
Management of pancreatoblastoma in children and young adults. ( 22525406 )
2012
35
Central pancreatectomy for pancreatoblastoma in a 16-year-old girl. ( 21843703 )
2011
36
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). ( 21696948 )
2011
37
Synchronous ectopic pancreatoblastoma in a child: a case report. ( 21655073 )
2011
38
Exocrine Pancreatic Neoplasms of Nonductal Origin: Acinar Cell Carcinoma, Pancreatoblastoma, and Solid-Pseudopapillary Neoplasm. ( 26837489 )
2011
39
Pancreaticoduodenectomy for pancreatoblastoma: a case report and literature review. ( 20165952 )
2010
40
Simultaneous occurrence of pancreatoblastoma and neuroblastoma in a newborn with beckwith-wiedemann syndrome. ( 20495482 )
2010
41
Pancreatoblastoma: two case reports from a medical center in Taiwan. ( 20057322 )
2010
42
Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. ( 20632017 )
2010
43
Corticotropin-releasing hormone-secreting pancreatoblastoma in an adult patient. ( 20664482 )
2010
44
Imaging findings of pancreatoblastoma in 4 children including a case of ectopic pancreatoblastoma. ( 20686762 )
2010
45
Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma. ( 20658635 )
2010
46
Pancreatoblastoma in an adult patient. A case report. ( 19287117 )
2009
47
Imprint cytology of pancreatoblastoma: a case report and review of the literature. ( 19229998 )
2009
48
Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. ( 19194274 )
2009
49
Fluorescence in situ hybridization analysis of pancreatoblastoma. ( 19238024 )
2009
50
Pancreatoblastoma in adults: a review of the literature. ( 19077457 )
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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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