MCID: PPL050
MIFTS: 32

Papillary Tumor of the Pineal Region

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Papillary Tumor of the Pineal Region

MalaCards integrated aliases for Papillary Tumor of the Pineal Region:

Name: Papillary Tumor of the Pineal Region 56
Ptpr 56

Characteristics:

Orphanet epidemiological data:

56
papillary tumor of the pineal region
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Orphanet 56 ORPHA251915
UMLS via Orphanet 70 C2985219
ICD10 via Orphanet 34 D44.5

Summaries for Papillary Tumor of the Pineal Region

MalaCards based summary : Papillary Tumor of the Pineal Region, also known as ptpr, is related to neurogenic thoracic outlet syndrome and adult medulloblastoma. An important gene associated with Papillary Tumor of the Pineal Region is VIM (Vimentin), and among its related pathways/superpathways are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Fentanyl and Donepezil have been mentioned in the context of this disorder. Affiliated tissues include pineal, brain and liver, and related phenotype is behavior/neurological.

Wikipedia : 72 Papillary tumors of the pineal region (PTPR) were first described by A. Jouvet et al. in 2003 and were... more...

Related Diseases for Papillary Tumor of the Pineal Region

Diseases related to Papillary Tumor of the Pineal Region via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 206)
id Related Disease Score Top Affiliating Genes
1 neurogenic thoracic outlet syndrome 10.6 SYP VIM
2 adult medulloblastoma 10.5 SYP VIM
3 space motion sickness 10.5 SYP VIM
4 papillary glioneuronal tumor 10.5 SYP VIM
5 biliary dyskinesia 10.5 S100A1 SYP
6 aflatoxins-related hepatocellular carcinoma 10.5 SYP VIM
7 myeloid/lymphoid neoplasm associated with pdgfra rearrangement 10.5 MUC1 VIM
8 spleen angiosarcoma 10.5 MUC1 VIM
9 neurofibroma of the esophagus 10.5 MUC1 S100A1
10 basosquamous carcinoma 10.5 BRAF VIM
11 mannose-binding lectin protein deficiency 10.5 MUC1 VIM
12 vulvar angiokeratoma 10.5 MUC1 VIM
13 noninvasive malignant thymoma 10.5 MUC1 VIM
14 angelman syndrome due to imprinting defect in 15q11-q13 10.5 S100A1 VIM
15 pyoderma 10.5 MUC1 VIM
16 pancreatic intraductal papillary-colloid carcinoma 10.5 MUC1 VIM
17 bladder colloid adenocarcinoma 10.5 MUC1 VIM
18 cellular ependymoma 10.5 MUC1 VIM
19 lice infestation 10.5 MUC1 VIM
20 ganglioglioma 10.5 MUC1 VIM
21 persian gulf syndrome 10.5 MUC1 VIM
22 diffuse meningeal melanocytosis 10.5 MUC1 VIM
23 fallopian tube germ cell cancer 10.5 MUC1 VIM
24 childhood pleomorphic rhabdomyosarcoma 10.5 MUC1 VIM
25 vulvar squamous papilloma 10.5 MUC1 VIM
26 spindle cell liposarcoma 10.5 MUC1 VIM
27 pancreatic serous cystadenoma 10.4 MUC1 VIM
28 subependymal glioma 10.4 MUC1 VIM
29 malignant breast melanoma 10.4 BRAF VIM
30 ovarian mucinous adenocarcinoma 10.4 MUC1 VIM
31 coronary restenosis 10.4 MUC1 VIM
32 extraskeletal mesenchymal chondrosarcoma 10.4 MUC1 VIM
33 pulmonary venoocclusive disease 10.4 MUC1 VIM
34 epithelioid sarcoma 10.4 MUC1 SYP
35 mesenchymoma 10.4 MUC1 VIM
36 small cell neuroendocrine carcinoma 10.4 S100A1 VIM
37 pleural empyema 10.4 MUC1 SYP
38 prostate disease 10.4 MUC1 VIM
39 spastic ataxia 3 10.4 MUC1 VIM
40 epicardium cancer 10.4 MUC1 S100A1
41 tabes dorsalis 10.4 MUC1 SYP
42 dense deposit disease 10.4 MUC1 VIM
43 patent ductus arteriosus and bicuspid aortic valve with hand anomalies 10.4 MUC1 VIM
44 peritoneal serous adenocarcinoma 10.4 MUC1 S100A1
45 chondroblastoma 10.4 MUC1 VIM
46 acidophil adenoma 10.4 SYP VIM
47 kidney benign neoplasm 10.4 MUC1 VIM
48 mitochondrial myopathy with lactic acidosis 10.4 MUC1 VIM
49 microcystic meningioma 10.3 MUC1 VIM
50 chilaiditi syndrome 10.3 MAP2 SYP

Graphical network of the top 20 diseases related to Papillary Tumor of the Pineal Region:



Diseases related to Papillary Tumor of the Pineal Region

Symptoms & Phenotypes for Papillary Tumor of the Pineal Region

MGI Mouse Phenotypes related to Papillary Tumor of the Pineal Region:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.1 BRAF ENO2 MAP2 S100A1 SYP VIM

Drugs & Therapeutics for Papillary Tumor of the Pineal Region

Drugs for Papillary Tumor of the Pineal Region (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 47)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
2
Donepezil Approved Phase 3 120014-06-4 3152
3 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
4 Adjuvants, Anesthesia Phase 3
5 Analgesics Phase 3,Phase 2
6 Analgesics, Opioid Phase 3
7 Anesthetics Phase 3
8 Anesthetics, General Phase 3
9 Anesthetics, Intravenous Phase 3
10 Central Nervous System Depressants Phase 3,Phase 2
11 Liver Extracts Phase 3,Phase 1
12 Narcotics Phase 3
13 Peripheral Nervous System Agents Phase 3,Phase 2
14 Cholinergic Agents Phase 3
15 Cholinesterase Inhibitors Phase 3
16 Neurotransmitter Agents Phase 3
17 Nootropic Agents Phase 3,Phase 2
18 Cola Nutraceutical Phase 3,Phase 1
19
Bevacizumab Approved, Investigational Phase 2 216974-75-3
20 Piracetam Approved Phase 2 7491-74-9
21
Pregabalin Approved, Illicit, Investigational Phase 2 148553-50-8 5486971
22 Etiracetam Investigational Phase 2 33996-58-6
23 Angiogenesis Inhibitors Phase 2
24 Angiogenesis Modulating Agents Phase 2
25 Antibodies Phase 2
26 Antibodies, Monoclonal Phase 2
27 Immunoglobulins Phase 2
28 Anti-Anxiety Agents Phase 2
29 Anticonvulsants Phase 2,Phase 1
30 calcium channel blockers Phase 2
31 Calcium, Dietary Phase 2
32 Neuroprotective Agents Phase 2
33 Protective Agents Phase 2
34 Psychotropic Drugs Phase 2
35 Tranquilizing Agents Phase 2
36
Trioxsalen Approved Phase 1 3902-71-4 5585
37
Verteporfin Approved, Investigational Phase 1 129497-78-5
38
Dacarbazine Approved, Investigational Phase 1 4342-03-4 5351166
39
Temozolomide Approved, Investigational Phase 1 85622-93-1 5394
40
Bortezomib Approved, Investigational Phase 1 179324-69-7 387447 93860
41
Entinostat Investigational Phase 1 209783-80-2
42 Dermatologic Agents Phase 1
43 Dihematoporphyrin Ether Phase 1
44 Photosensitizing Agents Phase 1
45 Alkylating Agents Phase 1
46 Histone Deacetylase Inhibitors Phase 1
47 Hormones

Interventional clinical trials:

(show all 14)

id Name Status NCT ID Phase Drugs
1 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
2 Donepezil in Treating Patients Who Have Undergone Radiation Therapy for Brain Tumors Completed NCT00369785 Phase 3 donepezil hydrochloride;Placebo
3 Bevacizumab in Reducing CNS Side Effects in Patients Who Have Undergone Radiation Therapy to the Brain for Primary Brain Tumor, Meningioma, or Head and Neck Cancer Completed NCT00492089 Phase 2 bevacizumab;placebo
4 Levetiracetam or Pregabalin in Treating Seizures in Patients Undergoing Chemotherapy and/or Radiation Therapy For Primary Brain Tumors Completed NCT00629889 Phase 2 levetiracetam;pregabalin
5 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1 verteporfin
6 RO4929097, Temozolomide, and Radiation Therapy in Treating Patients With Newly Diagnosed Malignant Glioma Completed NCT01119599 Phase 1 Gamma-Secretase Inhibitor RO4929097;Temozolomide
7 Bortezomib and Temozolomide in Treating Patients With Brain Tumors or Other Solid Tumors That Have Not Responded to Treatment Completed NCT00544284 Phase 1 bortezomib;temozolomide
8 MS-275 in Treating Patients With Advanced Solid Tumors or Lymphoma Completed NCT00020579 Phase 1 entinostat
9 Study of Tissue Samples From Patients With Glioma or Other Brain Tumors Completed NCT00967200
10 Changes in Semen or Sperm Caused by Temozolomide in Patients With Newly Diagnosed, Progressive, or Recurrent Primary Malignant Brain Tumors Completed NCT00499798
11 Acute Side Effects in Patients Who Are Undergoing Stereotactic Radiosurgery for Brain Tumors or Other Brain Disorders Completed NCT00255671
12 Yoga Therapy in Treating Patients With Malignant Brain Tumors Terminated NCT01234805
13 Gadobutrol Versus Gadopentetate Dimeglumine or Gadobenate Dimeglumine Before DCE-MRI in Diagnosing Patients With Multiple Sclerosis, Grade II-IV Glioma, or Brain Metastases Terminated NCT02967380 Gadobenate Dimeglumine;Gadobutrol
14 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452

Search NIH Clinical Center for Papillary Tumor of the Pineal Region

Genetic Tests for Papillary Tumor of the Pineal Region

Anatomical Context for Papillary Tumor of the Pineal Region

MalaCards organs/tissues related to Papillary Tumor of the Pineal Region:

39
Pineal, Brain, Liver

Publications for Papillary Tumor of the Pineal Region

Articles related to Papillary Tumor of the Pineal Region:

(show all 28)
id Title Authors Year
1
Nuclear CRX and FOXJ1 Expression Differentiates Non-Germ Cell Pineal Region Tumors and Supports the Ependymal Differentiation of Papillary Tumor of the Pineal Region. ( 28719464 )
2017
2
Papillary Tumor of the Pineal Region: A Distinct Molecular Entity. ( 26113311 )
2016
3
Late dissemination via cerebrospinal fluid of papillary tumor of the pineal region: a case report and literature review. ( 27179224 )
2016
4
Cytologic features of papillary tumor of the pineal region: A case report showing tigroid background. ( 27535020 )
2016
5
Increased VEGF levels in one case of papillary tumor of the pineal region with intracranial haemorrhage at presentation: a potential surrogate indicator of tumor angiogenesis and aggressiveness? ( 26745494 )
2016
6
Long-term follow-up of a papillary tumor of the pineal region: addendum to a case report. ( 27568037 )
2016
7
A case of papillary tumor of the pineal region with a long clinical history: molecular characterization and therapeutic consideration with review of the literature. ( 27472995 )
2016
8
Papillary tumor of the pineal region: Histopathological characterization and review of the literature. ( 24556386 )
2015
9
Papillary Tumor of the Pineal Region: MR Signal Intensity Correlated to Histopathology. ( 25688307 )
2015
10
Papillary tumor of the pineal region with extended clinical and radiologic follow-up. ( 26539320 )
2015
11
Papillary tumor of the pineal region presenting with Foster Kennedy sign. ( 26183673 )
2015
12
Outcomes After Surgery and Radiotherapy for Papillary Tumor of the Pineal Region. ( 25749579 )
2015
13
Papillary tumor of the pineal region-report of three cases with literature review. ( 26238893 )
2015
14
Lack of BRAF-V600E Mutation in Papillary Tumor of the Pineal Region. ( 26125673 )
2015
15
Papillary tumor of the pineal region with synchronous suprasellar focus and novel cytogenetic features. ( 26068071 )
2015
16
Papillary tumor of the pineal region: A case report and review of the literature. ( 26622493 )
2015
17
Papillary tumor of the pineal region. Report of two cases and literature review. ( 25440015 )
2014
18
PTEN mutations and activation of the PI3K/Akt/mTOR signaling pathway in papillary tumors of the pineal region. ( 25003235 )
2014
19
Papillary tumor of the pineal region with anaplastic small cell component. ( 23817812 )
2013
20
Bevacizumab is Effective for Recurrent Papillary Tumor of the Pineal Region: First Report. ( 24019784 )
2013
21
Stereotactic radiosurgery of a papillary tumor of the pineal region: case report and review of the literature. ( 23446182 )
2013
22
Early treatment response of a rare papillary tumor of the pineal region after primary proton-beam therapy using the raster-scanning technique at HIT. ( 23235766 )
2012
23
Histopathologic and ultrastructural features and claudin expression in papillary tumors of the pineal region: a multicenter analysis. ( 22588068 )
2012
24
Papillary tumor of the pineal region--a recently described entity: a report of three cases and review of the literature. ( 19642509 )
2009
25
Clinicopathological features from long-term observation of a papillary tumor of the pineal region (PTPR): a case report. ( 19856220 )
2009
26
Papillary tumor of the pineal region: a case report. ( 18987834 )
2008
27
Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. ( 17021405 )
2006
28
Papillary tumor of the pineal region. ( 12657936 )
2003

Variations for Papillary Tumor of the Pineal Region

Expression for Papillary Tumor of the Pineal Region

Search GEO for disease gene expression data for Papillary Tumor of the Pineal Region.

Pathways for Papillary Tumor of the Pineal Region

Pathways related to Papillary Tumor of the Pineal Region according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 11.64 ENO2 MAP2 SYP
2 10.7 MAP2 SYP VIM

GO Terms for Papillary Tumor of the Pineal Region

Cellular components related to Papillary Tumor of the Pineal Region according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 neuron projection GO:0043005 9.02 BRAF MAP2 S100A1 SYP VIM

Biological processes related to Papillary Tumor of the Pineal Region according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cellular response to organic substance GO:0071310 8.62 MAP2 SYP

Molecular functions related to Papillary Tumor of the Pineal Region according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 8.92 BRAF S100A1 SYP VIM

Sources for Papillary Tumor of the Pineal Region

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