CPNET
MCID: PRM027
MIFTS: 60

Primitive Neuroectodermal Tumor (CPNET) malady

Cancer diseases, Neuronal diseases categories
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Summaries for Primitive Neuroectodermal Tumor

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MalaCards: Primitive Neuroectodermal Tumor, also known as localized primitive neuroectodermal tumor, is related to peripheral primitive neuroectodermal tumor and sarcoma. An important gene associated with Primitive Neuroectodermal Tumor is MYCN (v-myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog), and among its related pathways are Notch Pathway and Nuclear signaling by ERBB4. The compounds hmba and eosin have been mentioned in the context of this disorder. Affiliated tissues include kidney, bone and uterus, and related mouse phenotypes are digestive/alimentary and no phenotypic analysis.

Aliases & Classifications for Primitive Neuroectodermal Tumor

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8Disease Ontology, 62UMLS, 35MeSH, 58SNOMED-CT, 40NCIt
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Neuronal diseases


Aliases & Descriptions:

primitive neuroectodermal tumor 8
localized primitive neuroectodermal tumor 8 62
extraosseous ewings sarcoma-primitive neuroepithelial tumor 62
primitive neuroectodermal tumour 8
neuroectodermal tumor, primitive 62
cns pnet 8
cpnet 8


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Disease Ontology8 DOID:168
MeSH35 D018242
SNOMED-CT58 39781001, 189925001
NCIt40 C5398

Related Diseases for Primitive Neuroectodermal Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Primitive Neuroectodermal Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 208)
idRelated DiseaseScoreTop Affiliating Genes
1peripheral primitive neuroectodermal tumor31.8EWSR1, PTPRC, VIM, MYCN, S100B
2sarcoma31.7EWSR1, PTPRC, TP53, VIM, MYCN, S100B
3cerebral primitive neuroectodermal tumor31.4MYC, MYCN
4medulloblastoma31.3S100B, RASSF1, SYP, IFNB1, MYC, MYCN
5neuroepithelioma31.2EWSR1, MYCN, MYC
6rhabdoid tumor31.1SYP, VIM, PTPRC
7neuroblastoma31.1TP53, MYCN, MYC, SYP
8pineoblastoma31.0GFAP, SYP
9childhood medulloblastoma30.9MYCN, MYC
10olfactory neuroblastoma30.7SYP, S100B
11retinoblastoma30.7GFAP, PTPRC, TP53, MYCN, MYC, SYP
12ganglioglioma30.7GFAP, SYP, S100B
13embryonal rhabdomyosarcoma30.6EWSR1, PTPRC, MYCN
14leukemia30.6EWSR1, PTPRC, TP53, MYCN, MYC, IFNB1
15ependymoma30.6GFAP, VIM, SYP, RASSF1, S100B
16gastrointestinal stromal tumor30.6S100B, VIM
17gliosarcoma30.5GFAP
18fibrous histiocytoma30.5EWSR1
19rhabdomyosarcoma30.5EWSR1, PTPRC, TP53, VIM, MYCN, S100B
20synovial sarcoma30.5S100B, RASSF1, MYCN, VIM
21oligodendroglioma30.4SYP, TP53, GFAP
22paraganglioma30.4GFAP, SYP, S100B
23astrocytoma30.4GFAP, TP53, VIM, IFNB1, SYP, RASSF1
24neurofibroma30.3VIM, SYP, S100B
25ganglioneuroma30.3GFAP, MYCN, SYP, RASSF1, S100B
26malignant glioma30.3GFAP, TP53, IFNB1, SYP
27meningioma30.3GFAP, VIM, SYP, S100B
28wilms tumor30.2RASSF1, MYCN, VIM, PTPRC, EWSR1
29supratentorial primitive neuroectodermal tumor11.1
30cerebritis10.8
31teratoma10.7
32atypical teratoid rhabdoid tumor10.6
33cervicitis10.6
34germ cell tumors10.6
35neuronitis10.5
36intracranial primitive neuroectodermal tumor10.5
37extraosseous ewing's sarcoma10.5
38prostatitis10.5
39thyroiditis10.5
40localized peripheral primitive neuroectodermal tumor10.5
41supratentorial primitive neuroectodermal tumors, childhood10.5
42cauda equina syndrome10.4
43trilateral retinoblastoma10.4
44unilateral retinoblastoma10.4
45cystic teratoma10.4
46mediastinitis10.4
47pancreatitis10.4
48paraplegia10.4
49desmoplastic small round cell tumor10.4
50nonseminomatous germ cell tumor10.4

Graphical network of the top 20 diseases related to Primitive Neuroectodermal Tumor:



Diseases related to primitive neuroectodermal tumor

Symptoms for Primitive Neuroectodermal Tumor

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Drugs & Therapeutics for Primitive Neuroectodermal Tumor

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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

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Genetic Tests for Primitive Neuroectodermal Tumor

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Anatomical Context for Primitive Neuroectodermal Tumor

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33MalaCards
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MalaCards organs/tissues related to Primitive Neuroectodermal Tumor:

33
Kidney, Bone, Uterus, Brain, Breast, Ovary, Cervix, Adrenal gland, Spinal cord, Pancreas, Liver, Prostate, Testis, Lung, Thyroid, Bone marrow, Thymus, Heart, Pineal, Colon, B lymphoblasts, T cells, Myeloid, Lymph node, Testes, Cortex, Cerebellum, Retina, Skin

Animal Models for Primitive Neuroectodermal Tumor or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Primitive Neuroectodermal Tumor:

37 (show all 16)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053818.5GFAP, PTPRC, TP53, MYCN, MYC
2MP:00030128.3PTPRC, TP53, MYCN, MYC, IFNB1, SYP
3MP:00053898.2MYC, MYCN, VIM, TP53, EWSR1
4MP:00053908.2EWSR1, PTPRC, TP53, MYCN, MYC, IFNB1
5MP:00053698.1EWSR1, GFAP, TP53, VIM, MYCN, MYC
6MP:00020068.0EWSR1, PTPRC, TP53, MYC, IFNB1, RASSF1
7MP:00053857.6GFAP, TP53, VIM, MYCN, MYC, RASSF1
8MP:00053867.5GFAP, TP53, VIM, MYCN, MYC, S100B
9MP:00053787.5EWSR1, GFAP, PTPRC, TP53, VIM, MYCN
10MP:00053977.4EWSR1, PTPRC, TP53, VIM, MYCN, MYC
11MP:00036317.2GFAP, PTPRC, TP53, VIM, MYCN, MYC
12MP:00053847.0EWSR1, GFAP, PTPRC, TP53, VIM, MYCN
13MP:00107686.9EWSR1, GFAP, PTPRC, TP53, VIM, MYCN
14MP:00053766.8GFAP, PTPRC, TP53, VIM, MYCN, MYC
15MP:00053876.8EWSR1, GFAP, PTPRC, TP53, VIM, MYC
16MP:00028736.6S100B, EWSR1, GFAP, PTPRC, TP53, MYCN

Publications for Primitive Neuroectodermal Tumor

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52PubMed
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Articles related to Primitive Neuroectodermal Tumor:

(show top 50)    (show all 637)
idTitleAuthorsYear
1
Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid. (23888220)
2013
2
FDG PET/CT appearance of portal vein tumor thrombus in the gastric primitive neuroectodermal tumor: uncommon primary tumor site with rare finding. (23242047)
2013
3
Rare pulmonary primitive neuroectodermal tumor metastasizing to the right atrium: a case report. (22392199)
2012
4
Low-grade bone lesions in survivors of childhood medulloblastoma/primitive neuroectodermal tumor. (22054800)
2012
5
Primitive neuroectodermal tumor/Ewing's sarcoma of the urinary bladder: a case report and its molecular diagnosis. (21063743)
2011
6
Primary primitive neuroectodermal tumor of the urinary bladder: a case report and literature review. (20852971)
2011
7
Paravaginal peripheral primitive neuroectodermal tumor: A rare tumor. (22557788)
2011
8
Intraspinal primitive neuroectodermal tumor in a man with neurofibromatosis type 1: Case report and review of the literature. (22140640)
2011
9
Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements. (20348883)
2010
10
Inferior vena cava thrombus in renal primitive neuroectodermal tumor is not rare. (20690331)
2010
11
Primary vulvar Ewing's sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: a case report. (19656640)
2010
12
Uterine primitive neuroectodermal tumor: a case report. (18523790)
2009
13
Dumbbell-shaped peripheral primitive neuroectodermal tumor of the spine--case report and review of the literature. (19050994)
2009
14
Uncommon pleural effusion: pleuropulmonary metastasis from primitive neuroectodermal tumor. (19618040)
2009
15
Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases. (18452568)
2009
16
Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation. (19390556)
2009
17
Primary primitive neuroectodermal tumor of the orbit. (19700881)
2009
18
A phase II study of preradiotherapy chemotherapy followed by hyperfractionated radiotherapy for newly diagnosed high-risk medulloblastoma/primitive neuroectodermal tumor: a report from the Children's Oncology Group (CCG 9931). (19356859)
2009
19
Primary primitive neuroectodermal tumor of the uterus: a case report. (17823807)
2008
20
Extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of the posterior mediastinum with t(11;22)(q24;q12). (19267114)
2008
21
Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. (17366107)
2007
22
Primitive neuroectodermal tumor arising in a testicular teratoma with retroperitoneal metastasis: report of an interesting case with review of literature. (17991577)
2007
23
Neonatal macrocephaly: cerebral primitive neuroectodermal tumor or neuroblastoma as an infrequent cause--a case report and review of the literature. (17909991)
2007
24
A phase II study of the farnesyl transferase inhibitor, tipifarnib, in children with recurrent or progressive high-grade glioma, medulloblastoma/primitive neuroectodermal tumor, or brainstem glioma: a Children's Oncology Group study. (17932894)
2007
25
Renal primitive neuroectodermal tumor in childhood with intracardiac extension. (16904470)
2006
26
Quantitative mRNA expression analysis of neurotrophin-receptor TrkC and oncogene c-MYC from formalin-fixed, paraffin-embedded primitive neuroectodermal tumor samples. (17080715)
2006
27
Huge primitive neuroectodermal tumor of the pancreas: report of a case and review of the literature. (17009412)
2006
28
Untreated pediatric primitive neuroectodermal tumor in vivo: quantitation of taurine with MR spectroscopy. (16118174)
2005
29
A primitive neuroectodermal tumor on the face: case report. (16033149)
2005
30
Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging. (15131035)
2004
31
Second malignant neoplasms in childhood acute lymphoblastic leukemia: primitive neuroectodermal tumor of the chest wall with germline p53 mutation as a second malignant neoplasm. (15114597)
2004
32
Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. (12594313)
2003
33
Multiple systemic metastases of posterior fossa - primitive neuroectodermal tumor (PF-PNET) in adult: case report. (12715029)
2003
34
Primitive neuroectodermal tumor of the cervix: a clinicopathologic and immunohistochemical study of two cases. (12376920)
2002
35
Extraaxial primitive neuroectodermal tumor mimicking a vestibular schwannoma: diagnostic and therapeutic difficulties. Report of two cases. (11302660)
2001
36
Role of MAP kinase pathways in primitive neuroectodermal tumors. (11724348)
2001
37
Intense p53 staining is a valuable prognostic indicator for poor prognosis in medulloblastoma/central nervous system primitive neuroectodermal tumors. (11451203)
2001
38
Primitive neuroectodermal tumor of the lung. (11231490)
2001
39
Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. (10623711)
2000
40
Primary primitive neuroectodermal tumor of the cauda equina. (10987262)
2000
41
Bone morphogenetic proteins-2 and -4 attenuate apoptosis in a cerebellar primitive neuroectodermal tumor cell line. (10336254)
1999
42
Documentation of EWS gene rearrangements by fluorescence in-situ hybridization (FISH) in frozen sections of Ewing's sarcoma-peripheral primitive neuroectodermal tumor. (10078922)
1999
43
The Ewing tumor family of peripheral primitive neuroectodermal tumors expresses human gastrin-releasing peptide. (9622091)
1998
44
Centrosome amplification as a possible mechanism for numerical chromosome aberrations in cerebral primitive neuroectodermal tumors with TP53 mutations. (10072601)
1998
45
Primitive neuroectodermal tumor of the cervix uteri. A case report. (9065588)
1997
46
Renal primitive neuroectodermal tumor: a morphologic, cytogenetic, and molecular analysis with the establishment of two cultured cell lines. (9559290)
1997
47
TGF beta 1 and TGF beta 2 are potential growth regulators for medulloblastomas, primitive neuroectodermal tumors, and ependymomas: evidence in support of an autocrine hypothesis. (8200640)
1994
48
Secretogranin II expression in Ewing's sarcomas and primitive neuroectodermal tumors. (1342962)
1992
49
Immunohistochemistry of primitive neuroectodermal tumors in infants with special emphasis on cytokeratin expression. (1723829)
1991
50
Overexpression of the pseudoautosomal gene MIC2 in Ewing's sarcoma and peripheral primitive neuroectodermal tumor. (1695726)
1990

Variations for Primitive Neuroectodermal Tumor

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Expression for genes affiliated with Primitive Neuroectodermal Tumor

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Primitive Neuroectodermal Tumor

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Pathways for genes affiliated with Primitive Neuroectodermal Tumor

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50PathCards, 61Tocris Bioscience, 55Reactome, 38NCBI BioSystems Database, 30KEGG, 53QIAGEN, 60Thomson Reuters, 5Cell Signaling Technology
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Compounds for genes affiliated with Primitive Neuroectodermal Tumor

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45Novoseek, 11DrugBank, 29IUPHAR, 24HMDB, 3BitterDB, 51PharmGKB, 61Tocris Bioscience
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Compounds related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 132)
idCompoundScoreTop Affiliating Genes
1hmba459.5MYCN, TP53, MYC
2eosin459.3S100B, VIM, GFAP
3mucicarmine459.2S100B, SYP, VIM
4agarose459.1S100B, PTPRC, MYC, MYCN
5s 100459.0S100B, SYP, VIM, GFAP
6reticulin459.0VIM, S100B, SYP, GFAP
7catecholamine458.9VIM, S100B, SYP, MYCN
8ganglioside458.9VIM, GFAP, SYP, PTPRC
9b 723458.8S100B, SYP, VIM, PTPRC
10acth458.8GFAP, S100B, PTPRC, SYP, IFNB1
11temozolomide45 119.8TP53, VIM, IFNB1
12adriamycin458.8MYC, VIM, TP53, MYCN
13dopamine45 29 24 1111.7S100B, GFAP, SYP, IFNB1, MYCN
14choline45 24 1110.6GFAP, VIM, SYP, S100B
15hematoxylin458.5S100B, GFAP, VIM, SYP, PTPRC
16formaldehyde45 249.5SYP, S100B, PTPRC, GFAP, VIM
17chloramphenicol45 3 51 1111.5VIM, MYCN, MYC, IFNB1, S100B
18agar458.5RASSF1, VIM, MYC, MYCN, GFAP
19vincristine45 51 1110.4MYC, IFNB1, VIM, MYCN, TP53
20glutamine458.4VIM, S100B, SYP, GFAP, EWSR1
21cytarabine45 51 1110.4VIM, GFAP, TP53, MYC, IFNB1
22leucine458.4GFAP, S100B, SYP, MYC, MYCN, VIM
23retinoid458.4VIM, MYCN, TP53, IFNB1, MYC
24iron45 249.3GFAP, S100B, IFNB1, MYCN, VIM
25doxorubicin45 51 1110.3MYC, MYCN, VIM, TP53, IFNB1
26actinomycin d458.3IFNB1, TP53, VIM, MYCN, MYC
27thymidine45 249.3TP53, GFAP, MYC, IFNB1, S100B
28bromodeoxyuridine458.3MYC, S100B, GFAP, VIM, IFNB1, MYCN
29dbc-amp458.2GFAP, VIM, MYCN, S100B, SYP, IFNB1
30glycogen45 249.2SYP, VIM, MYC, GFAP, PTPRC, S100B
31threonine458.1IFNB1, S100B, RASSF1, GFAP, PTPRC, MYC
325-aza-2deoxycytidine458.1VIM, RASSF1, IFNB1, MYC, TP53
33creatinine458.1SYP, GFAP, S100B, IFNB1, VIM
34paclitaxel45 51 1110.0MYC, IFNB1, S100B, GFAP, VIM, TP53
35cisplatin45 51 61 1111.0MYCN, MYC, GFAP, SYP, IFNB1, TP53
36genistein45 29 61 3 24 1112.9IFNB1, VIM, MYC, MYCN, GFAP, PTPRC
37tyrosine457.8S100B, SYP, IFNB1, MYC, PTPRC, GFAP
38methionine457.7RASSF1, S100B, GFAP, PTPRC, VIM, IFNB1
39alanine457.7MYC, IFNB1, RASSF1, S100B, GFAP, VIM
40etoposide45 51 61 1110.7MYC, S100B, VIM, TP53, MYCN, SYP
41dexamethasone45 51 29 1110.7PTPRC, GFAP, MYC, SYP, VIM, IFNB1
42cycloheximide457.7GFAP, IFNB1, MYC, MYCN, PTPRC, TP53
43progesterone45 29 61 24 1111.5PTPRC, SYP, IFNB1, VIM, TP53, S100B
44oligonucleotide457.4EWSR1, RASSF1, IFNB1, MYC, TP53, VIM
45vegf457.4MYCN, S100B, SYP, IFNB1, MYC, VIM
46cysteine457.3VIM, PTPRC, IFNB1, SYP, S100B, GFAP
47paraffin457.1VIM, TP53, EWSR1, GFAP, PTPRC, S100B
48estrogen456.6IFNB1, PTPRC, S100B, RASSF1, SYP, MYC
49retinoic acid45 247.4MYC, IFNB1, RASSF1, SYP, MYCN, VIM
50serine455.9S100B, TP53, PTPRC, GFAP, EWSR1, VIM

GO Terms for genes affiliated with Primitive Neuroectodermal Tumor

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16Gene Ontology
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Cellular components related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein complexGO:0432349.3SYP, MYC, TP53

Biological processes related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show all 16)
idNameGO IDScoreTop Affiliating Genes
1regulation of neuronal synaptic plasticityGO:04816810.0S100B, SYP
2negative regulation of reactive oxygen species metabolic processGO:200037810.0MYCN, TP53
3long-term synaptic potentiationGO:0602919.9S100B, GFAP
4negative regulation of fibroblast proliferationGO:0481479.8TP53, MYC
5response to gamma radiationGO:0103329.8MYC, TP53
6cellular response to drugGO:0356909.7MYC, TP53
7Bergmann glial cell differentiationGO:0600209.6VIM, GFAP
8astrocyte developmentGO:0140029.6VIM, GFAP
9intermediate filament organizationGO:0451099.6VIM, GFAP
10positive regulation of mesenchymal cell proliferationGO:0020539.6MYC, MYCN
11B cell proliferationGO:0421009.6PTPRC, IFNB1
12cellular response to UVGO:0346449.6MYC, TP53
13negative regulation of neuron projection developmentGO:0109779.3GFAP, VIM
14cell cycle arrestGO:0070509.3TP53, MYC, RASSF1
15cellular response to DNA damage stimulusGO:0069749.3RASSF1, MYC, TP53
16positive regulation of transcription from RNA polymerase II promoterGO:0459449.0IFNB1, MYC, MYCN, TP53

Molecular functions related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1identical protein bindingGO:0428027.1EWSR1, TP53, VIM, SYP, RASSF1, S100B
2protein bindingGO:0055156.7EWSR1, PTPRC, TP53, VIM, MYCN, MYC

Products for genes affiliated with Primitive Neuroectodermal Tumor

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Sources for Primitive Neuroectodermal Tumor

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4CDC
13ExPASy
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24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
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40NCIt
41NDF-RT
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48OMIM via Orphanet
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53QIAGEN
59SNOMED-CT via Orphanet
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