MCID: PNC019
MIFTS: 35

Pancreatoblastoma

Categories: Rare diseases, Gastrointestinal diseases, Cancer diseases, Endocrine 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 189814006 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, icterus and pruritus. An important gene associated with Pancreatoblastoma is AFP (Alpha Fetoprotein), and among its related pathways/superpathways is Glucocorticoid receptor regulatory network. Affiliated tissues include pancreas, lymph node and liver, and related phenotypes are weight loss and vomiting

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 142)
# Related Disease Score Top Affiliating Genes
1 acinar cell carcinoma 30.3 AFP CHGA SERPINA1
2 ectopic cushing syndrome 30.2 SST SYP
3 adenoma of the pancreas 29.6 CHGA SST SYP
4 mucinous cystadenofibroma 10.7 AFP SERPINA1
5 polyembryoma 10.7 AFP SERPINA1
6 malignant sertoli cell tumor 10.7 AFP SYP
7 ovarian endodermal sinus tumor 10.6 AFP SERPINA1
8 malignant teratoma 10.6 AFP SYP
9 endometrial small cell carcinoma 10.6 AFP SYP
10 adult teratoma 10.6 AFP CHGA
11 mediastinal cancer 10.6 AFP SYP
12 seminal vesicle tumor 10.6 AFP SYP
13 sclerosing hepatic carcinoma 10.6 AFP CHGA
14 ovarian primitive germ cell tumor 10.6 AFP SERPINA1
15 cystadenofibroma 10.6 AFP SERPINA1
16 pancreatic acinar cell adenocarcinoma 10.6 AFP CHGA
17 pineoblastoma 10.6 AFP SYP
18 liver sarcoma 10.6 AFP CHGA
19 esophageal neuroendocrine tumor 10.5 CHGA SYP
20 ureter small cell carcinoma 10.5 CHGA SYP
21 urinary bladder small cell neuroendocrine carcinoma 10.5 CHGA SYP
22 ovarian large-cell neuroendocrine carcinoma 10.5 CHGA SYP
23 auditory system cancer 10.5 CHGA SYP
24 peritoneal serous adenocarcinoma 10.5 CHGA SYP
25 cellular ependymoma 10.5 CHGA SYP
26 gallbladder small cell carcinoma 10.5 CHGA SYP
27 primary hepatic neuroendocrine carcinoma 10.5 CHGA SYP
28 small cell carcinoma of the bladder 10.5 CHGA SYP
29 pulmonary large cell neuroendocrine carcinoma 10.5 CHGA SYP
30 atypical follicular adenoma 10.5 CHGA SYP
31 chordoid meningioma 10.5 CHGA SYP
32 olfactory nerve neoplasm 10.5 CHGA SYP
33 cauda equina neoplasm 10.5 CHGA SYP
34 pancreatic serous cystadenoma 10.5 CHGA SYP
35 conventional angiosarcoma 10.5 CHGA SYP
36 nodular ganglioneuroblastoma 10.5 CHGA SYP
37 pulmonary sclerosing hemangioma 10.5 CHGA SYP
38 pineocytoma 10.5 CHGA SYP
39 respiratory system benign neoplasm 10.5 CHGA SYP
40 gastric squamous cell carcinoma 10.5 CHGA SYP
41 gastric small cell carcinoma 10.5 CHGA SYP
42 subependymoma 10.5 CHGA SYP
43 pulmonary blastoma 10.5 CHGA SYP
44 ureter, cancer of 10.5 CHGA SYP
45 tanycytic ependymoma 10.5 CHGA SYP
46 papillary ependymoma 10.5 CHGA SYP
47 malignant glandular tumor of peripheral nerve sheath 10.5 SST SYP
48 non-functioning pancreatic endocrine tumor 10.5 CHGA SYP
49 esophageal varix 10.4 AFP SST
50 diffuse pulmonary fibrosis 10.4 CHGA SERPINA1

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

UMLS symptoms related to Pancreatoblastoma:


abdominal pain, icterus, pruritus

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

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance GR00327-A 8.8 AFP KRT14 KRT17

Drugs & Therapeutics for Pancreatoblastoma

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, Brain

Publications for Pancreatoblastoma

Articles related to Pancreatoblastoma:

(show top 50) (show all 133)
# 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 With Metastatic Retroperitoneal Lymph Node and PET/CT. ( 28872552 )
2017
6
Pancreatoblastoma: an Atypical Presentation and a Literature Review. ( 28194556 )
2017
7
Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report. ( 28538503 )
2017
8
Pancreatoblastoma: an unusual diagnosis in an adult patient. ( 29285688 )
2017
9
Pancreatoblastoma Masquerading as a Retroperitoneal Germ Cell Tumor. ( 28082787 )
2017
10
Pancreatoblastoma masquerading as hepatoblastoma: A diagnostic dilemma. ( 27046982 )
2016
11
Pancreatoblastoma an Unusual Occurrence of a Tumour in Paediatric Age Group: A Case Report. ( 27134883 )
2016
12
Multi-Visceral Transplantation in a 21-Year-Old Man with Prior Pancreatoblastoma. ( 27721967 )
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
Adult pancreatoblastoma - Case report and review of literature. ( 27761191 )
2016
15
Adult pancreatoblastoma: A case report and review of the literature. ( 26137059 )
2015
16
Response of Relapsed Pancreatoblastoma to a Combination of Vinorelbine and Oral Cyclophosphamide. ( 26056794 )
2015
17
Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt. ( 26881602 )
2015
18
Clinicopathological review of pancreatoblastoma in adults. ( 26312218 )
2015
19
Multifocal pancreatoblastoma with pseudocyst in a 2-year-old boy. ( 26591960 )
2015
20
Pancreatoblastoma in an adult. ( 25673604 )
2015
21
Pancreatoblastoma, a Rare Childhood Tumor: A Case Report. ( 25110242 )
2014
22
Spontaneous rupture of an advanced pancreatoblastoma: aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers. ( 23583162 )
2013
23
Pancreatoblastoma: a rare, adult pancreatic tumor with many faces. ( 23991735 )
2013
24
Infantile Extrapancreatic Pancreatoblastoma: A Report on a Rare Infantile Abdominal Mass. ( 24072247 )
2013
25
Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma. ( 23741195 )
2013
26
The diagnosis and surgical treatment of pancreatoblastoma in adults: a case series and review of the literature. ( 24081396 )
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
Use of gemcitabine with multi-stage surgical resection as successful second-line treatment of metastatic pancreatoblastoma. ( 23042010 )
2013
29
Congenital pancreatoblastoma: report of an atypical case and review of the literature. ( 22278199 )
2012
30
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology. ( 22572137 )
2012
31
Clinical analysis of childhood pancreatoblastoma arising from the tail of the pancreas. ( 22584784 )
2012
32
Congenital pancreatoblastoma associated with I^-catenin mutation. ( 21953767 )
2012
33
Management of pancreatoblastoma in children and young adults. ( 22525406 )
2012
34
Central pancreatectomy for pancreatoblastoma in a 16-year-old girl. ( 21843703 )
2011
35
Pancreatoblastoma: a report from the European cooperative study group for paediatric rare tumours (EXPeRT). ( 21696948 )
2011
36
Synchronous ectopic pancreatoblastoma in a child: a case report. ( 21655073 )
2011
37
Pancreaticoduodenectomy for pancreatoblastoma: a case report and literature review. ( 20165952 )
2010
38
Simultaneous occurrence of pancreatoblastoma and neuroblastoma in a newborn with beckwith-wiedemann syndrome. ( 20495482 )
2010
39
Pancreatoblastoma: two case reports from a medical center in Taiwan. ( 20057322 )
2010
40
Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy. ( 20632017 )
2010
41
Corticotropin-releasing hormone-secreting pancreatoblastoma in an adult patient. ( 20664482 )
2010
42
Imaging findings of pancreatoblastoma in 4 children including a case of ectopic pancreatoblastoma. ( 20686762 )
2010
43
Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma. ( 20658635 )
2010
44
Pancreatoblastoma in an adult patient. A case report. ( 19287117 )
2009
45
Imprint cytology of pancreatoblastoma: a case report and review of the literature. ( 19229998 )
2009
46
Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. ( 19194274 )
2009
47
Fluorescence in situ hybridization analysis of pancreatoblastoma. ( 19238024 )
2009
48
Pancreatoblastoma in adults: a review of the literature. ( 19077457 )
2009
49
Revisiting metastatic adult pancreatoblastoma. A case and review of the literature. ( 18981556 )
2008
50
Childhood pancreatoblastoma: clinical features and immunohistochemistry analysis. ( 18308466 )
2008

Variations for Pancreatoblastoma

Expression for Pancreatoblastoma

Search GEO for disease gene expression data for Pancreatoblastoma.

Pathways for Pancreatoblastoma

Pathways related to Pancreatoblastoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.71 AFP KRT14 KRT17

GO Terms for Pancreatoblastoma

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
Content
Loading form....