MCID: MNN001
MIFTS: 45

Meningeal Melanocytoma

Categories: Neuronal diseases, Rare diseases, Cancer diseases

Aliases & Classifications for Meningeal Melanocytoma

MalaCards integrated aliases for Meningeal Melanocytoma:

Name: Meningeal Melanocytoma 12 59 15 73
Leptomeningeal Melanocytoma 12
Melanocytoma of Meninges 12

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:5900
NCIt 50 C4662
SNOMED-CT 68 128730009 277527003
Orphanet 59 ORPHA252046
UMLS via Orphanet 74 C1266113
UMLS 73 C1266113

Summaries for Meningeal Melanocytoma

MalaCards based summary : Meningeal Melanocytoma, also known as leptomeningeal melanocytoma, is related to localized hypertrophic neuropathy and sclerosing perineurioma, and has symptoms including headache, vomiting and neurologic symptoms. An important gene associated with Meningeal Melanocytoma is MUC1 (Mucin 1, Cell Surface Associated), and among its related pathways/superpathways are Neuroscience and Ca, cAMP and Lipid Signaling. The drugs Donepezil and Fentanyl have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and pituitary, and related phenotypes are integument and pigmentation

Related Diseases for Meningeal Melanocytoma

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

(show top 50) (show all 119)
# Related Disease Score Top Affiliating Genes
1 localized hypertrophic neuropathy 11.0 MUC1 S100B
2 sclerosing perineurioma 11.0 MUC1 S100B
3 reticular perineurioma 11.0 MUC1 S100B
4 non-functioning pituitary adenoma 10.9 MUC1 S100B
5 fibrous meningioma 10.9 MUC1 S100B
6 chordoid meningioma 10.9 MUC1 S100B
7 syringocystadenoma papilliferum 10.9 MUC1 S100B
8 eccrine porocarcinoma 10.9 MUC1 S100B
9 spindle cell rhabdomyosarcoma 10.9 MUC1 S100B
10 aggressive digital papillary adenocarcinoma 10.9 MUC1 S100B
11 myxopapillary ependymoma 10.9 MUC1 S100B
12 pilomatrix carcinoma 10.9 ENO2 MUC1
13 monophasic synovial sarcoma 10.9 MUC1 S100B
14 sinonasal undifferentiated carcinoma 10.9 ENO2 MUC1
15 bednar tumor 10.8 ENO2 S100B
16 chordoid glioma 10.8 MUC1 S100B
17 papillary tumor of the pineal region 10.8 ENO2 MUC1
18 spiradenoma 10.8 MUC1 S100B
19 middle ear adenoma 10.8 ENO2 MUC1
20 microcystic adenoma 10.8 ENO2 MUC1
21 meningitis 10.8
22 middle cerebral artery infarction 10.8 ENO2 S100B
23 syringoma 10.8 MUC1 S100B
24 phakomatosis cesioflammea 10.8 GNA11 GNAQ
25 intraocular melanoma 10.8 GNA11 GNAQ
26 head injury 10.7 ENO2 S100B
27 anaplastic ependymoma 10.7 MUC1 S100B
28 melanotic neuroectodermal tumor 10.7 ENO2 S100B
29 mucinous ovarian cystadenoma 10.7 ENO2 MUC1
30 malignant peritoneal mesothelioma 10.7 CDKN2A MUC1
31 desmoplastic small round cell tumor 10.7 ENO2 MUC1
32 pineocytoma 10.7 ENO2 S100B
33 gastric leiomyosarcoma 10.7 ENO2 YARS
34 mucinous bronchioloalveolar adenocarcinoma 10.7 CDKN2A MUC1
35 endometrial mucinous adenocarcinoma 10.6 CDKN2A MUC1
36 malignant spindle cell melanoma 10.6 NRAS RREB1
37 intracranial cysts 10.6 ENO2 MUC1 S100B
38 epithelioid malignant peripheral nerve sheath tumor 10.6 ENO2 MUC1 S100B
39 ossifying fibromyxoid tumor 10.6 ENO2 MUC1 S100B
40 cholangiolocellular carcinoma 10.6 CDKN2A MUC1
41 malignant giant cell tumor of soft parts 10.6 CD68 S100B
42 cerebral primitive neuroectodermal tumor 10.6 ENO2 MUC1 S100B
43 optic nerve glioma 10.6 ENO2 MUC1 S100B
44 pilomatrixoma 10.6 ENO2 MUC1 S100B
45 ependymoma 10.6 ENO2 MUC1 S100B
46 hemangioblastoma 10.6 ENO2 MUC1 S100B
47 appendix cancer 10.6 MUC1 YARS
48 sarcomatoid mesothelioma 10.5 CDKN2A MUC1
49 syringomyelia 10.5 CWC22 NRAS
50 well-differentiated liposarcoma 10.5 CDKN2A MUC1

Graphical network of the top 20 diseases related to Meningeal Melanocytoma:



Diseases related to Meningeal Melanocytoma

Symptoms & Phenotypes for Meningeal Melanocytoma

UMLS symptoms related to Meningeal Melanocytoma:


headache, vomiting, neurologic symptoms

MGI Mouse Phenotypes related to Meningeal Melanocytoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.5 CDKN2A GNA11 GNAQ MLANA MME NRAS
2 pigmentation MP:0001186 9.02 CDKN2A GNA11 GNAQ MLANA NRAS

Drugs & Therapeutics for Meningeal Melanocytoma

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

(show all 46)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Donepezil Approved Phase 3 120014-06-4 3152
2
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
3 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
4 Analgesics Phase 3
5 Central Nervous System Depressants Phase 3
6 Adjuvants, Anesthesia Phase 3
7 Cholinergic Agents Phase 3
8 Cholinesterase Inhibitors Phase 3
9 Narcotics Phase 3
10 Neurotransmitter Agents Phase 3
11 Analgesics, Opioid Phase 3
12 Anesthetics Phase 3
13 Nootropic Agents Phase 3
14 Anesthetics, General Phase 3
15 Anesthetics, Intravenous Phase 3
16 Peripheral Nervous System Agents Phase 3
17 Liver Extracts Phase 3
18 Cola Nutraceutical Phase 3
19
Bevacizumab Approved, Investigational Phase 2 216974-75-3
20 Angiogenesis Inhibitors Phase 2
21 Angiogenesis Modulating Agents Phase 2
22 Antibodies Phase 2
23 Immunoglobulins Phase 2
24 Antibodies, Monoclonal Phase 2
25
Verteporfin Approved, Investigational Phase 1 129497-78-5
26
Trioxsalen Approved Phase 1 3902-71-4 5585
27
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
28
Everolimus Approved Phase 1 159351-69-6 6442177
29
Sirolimus Approved, Investigational Phase 1 53123-88-9 5284616 6436030 46835353
30 Etanidazole Investigational Phase 1 22668-01-5
31 Dermatologic Agents Phase 1
32 Dihematoporphyrin Ether Phase 1
33 Photosensitizing Agents Phase 1
34 Mitogens Phase 1
35 Dialysis Solutions Phase 1
36 Anti-Bacterial Agents Phase 1
37 Antibiotics, Antitubercular Phase 1
38 Immunosuppressive Agents Phase 1
39 Anti-Infective Agents Phase 1
40 Antifungal Agents Phase 1
41 Fluorides Phase 1
42 Protective Agents Phase 1
43 Cariostatic Agents Phase 1
44
Temozolomide Approved, Investigational 85622-93-1 5394
45 Alkylating Agents
46 Antineoplastic Agents, Alkylating

Interventional clinical trials:


# 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 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1 verteporfin
5 A Feasibility, Dose-Escalation Study Using Intracerebral Microdialysis to Assess the Neuropharmacodynamics of Temsirolimus in Patients With Primary or Metastatic Brain Tumors Completed NCT00784914 Phase 1 temsirolimus
6 Positron Emission Tomography Using Fluorine F 18 EF5 to Find Oxygen in Tumor Cells of Patients Who Are Undergoing Surgery or Biopsy for Newly Diagnosed Brain Tumors Terminated NCT00110032 Phase 1 EF5
7 Study of Tissue Samples From Patients With Glioma or Other Brain Tumors Completed NCT00967200
8 Changes in Semen or Sperm Caused by Temozolomide in Patients With Newly Diagnosed, Progressive, or Recurrent Primary Malignant Brain Tumors Completed NCT00499798
9 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452 Not Applicable

Search NIH Clinical Center for Meningeal Melanocytoma

Genetic Tests for Meningeal Melanocytoma

Anatomical Context for Meningeal Melanocytoma

MalaCards organs/tissues related to Meningeal Melanocytoma:

41
Brain, Liver, Pituitary, Temporal Lobe, Spinal Cord, Pineal

Publications for Meningeal Melanocytoma

Articles related to Meningeal Melanocytoma:

(show top 50) (show all 91)
# Title Authors Year
1
Spinal meningeal melanocytoma in a 5-year-old child: a case report and review of literature. ( 29780233 )
2018
2
Primary meningeal melanocytoma in sellar region, simulating a nonfunctioning pituitary adenoma; a case report and literature review. ( 29382618 )
2018
3
Intracranial meningeal melanocytoma diagnosed using an interdisciplinary approach: a case report and review of the literature. ( 29941032 )
2018
4
Diagnostic Clue of Meningeal Melanocytoma: Case Report and Review of Literature. ( 28120582 )
2017
5
Supratentorial intermediate grade meningeal melanocytoma with intratumoral bleed in the background of neurocutaneous melanosis: Report of an unusual case and review of literature. ( 28413547 )
2017
6
Orbital meningeal melanocytoma: Histological, immunohistochemical and molecular characterization of a case and review of the literature. ( 27499153 )
2016
7
Squash cytopathology of primary meningeal melanocytoma. ( 26755006 )
2016
8
Primary meningeal melanocytoma mimicking a nonfunctioning pituitary adenoma. ( 26857865 )
2016
9
Cervical Spinal Meningeal Melanocytoma Presenting as Intracranial Superficial Siderosis. ( 26770862 )
2015
10
Primary Meningeal Melanocytoma in the Left Temporal Lobe Associated with Nevus Ota: a Case Report and Review of the Literature. ( 25862111 )
2015
11
Cerebellar meningeal melanocytoma associated with nevus of Ota: An extremely rare case. ( 26259856 )
2015
12
Intermediate-grade meningeal melanocytoma associated with nevus of Ota: a case report and review of the literature. ( 25933209 )
2015
13
Meningeal melanocytoma of the middle cranial fossa (the Meckel's cave). ( 25947998 )
2015
14
A case report on intermediate-grade malignant meningeal melanocytoma with elevated cell proliferation index. ( 26617913 )
2015
15
The GNAQ in the haystack: intramedullary meningeal melanocytoma of intermediate grade at T9-10 in a 58-year-old woman. ( 26544768 )
2015
16
Primary meningeal melanocytoma of the sellar region: review of the literature and differential diagnosis with special reference to angiographical features. ( 25583147 )
2015
17
Meningeal melanocytoma: case report and literature review. ( 24791361 )
2014
18
Primary ventral foramen magnum meningeal melanocytoma. ( 24823756 )
2014
19
BRAF V600E mutation in two distinct meningeal melanocytomas associated with a nevus of Ota. ( 24493731 )
2014
20
Spinal meningeal melanocytoma with benign histology showing leptomeningeal spread: case report. ( 23690716 )
2013
21
MR imaging findings of the intraspinal meningeal melanocytoma: correlation with histopathologic findings. ( 22442042 )
2012
22
Multifocal spinal meningeal melanocytoma: an illustrated case review. ( 23208918 )
2012
23
Unusual tonsillar herniation in meningeal melanocytoma: a case report. ( 23408465 )
2012
24
Primary meningeal melanocytoma of the anterior cranial fossa: a case report and review of the literature. ( 22759717 )
2012
25
Primary meningeal melanocytoma located in foramen magnum: a case report and review of the literatures. ( 22770412 )
2012
26
Multifocal meningeal melanocytoma of the conus medullaris. ( 21887493 )
2011
27
Malignant transformation of intracranial meningeal melanocytoma. Case report and review of the literature. ( 21062362 )
2011
28
Spinal meningeal melanocytoma in the S-1 nerve root sheath with paraspinal extension mimicking schwannoma. ( 21492734 )
2011
29
Intracranial meningeal melanocytoma associated with nevus of Ota. ( 21924617 )
2011
30
Spinal meningeal melanocytoma. ( 22347335 )
2011
31
Meningeal melanocytoma of the cerebellopontine angle as the unusual cause of superficial siderosis. ( 21207224 )
2011
32
Spinal meningeal melanocytoma: A rare meningeal tumor. ( 21264144 )
2010
33
Meningeal melanocytoma extruded from the skull of a neonate--case report. ( 20339277 )
2010
34
Intracranial intermediate-grade meningeal melanocytoma with increased cellular proliferative index: an illustrative case associated with a nevus of Ota. ( 19449182 )
2009
35
Multifocal meningeal melanocytoma: a new pathological entity or the result of leptomeningeal seeding? ( 19361258 )
2009
36
Intraoperative smear cytology of meningeal melanocytoma of the posterior fossa. ( 18093219 )
2009
37
Intermediate grade meningeal melanocytoma of cervical spine. ( 19139906 )
2009
38
Primary meningeal melanocytoma. ( 18769099 )
2008
39
Cerebellopontine angle meningeal melanocytoma: a rare tumor in an uncommon location. Case report. ( 17564187 )
2007
40
Malignant transformation of spinal meningeal melanocytoma. Case report and review of the literature. ( 17542513 )
2007
41
Supratentorial meningeal melanocytoma mimicking a convexity meningioma. ( 16391879 )
2006
42
Coexisting intracranial meningeal melanocytoma, dermoid tumor, and Dandy-Walker cyst in a patient with neurocutaneous melanosis. Case report. ( 16572661 )
2006
43
Case report: Intracranial meningeal melanocytoma: a cause of high signal on T1- and low signal on T2-weighted MRI. ( 16488214 )
2006
44
Meningeal melanocytoma. ( 16491422 )
2006
45
Meningeal melanocytoma of the posterior fossa: case report and literature review. ( 15734524 )
2005
46
Meningeal melanocytoma associated with ipsilateral nevus of Ota presenting as intracerebral hemorrhage: case report. ( 15918956 )
2005
47
Transition from meningeal melanocytoma to primary cerebral melanoma. Case report. ( 15352613 )
2004
48
Spinal meningeal melanocytoma. Report of two cases and review of the literature. ( 15029918 )
2004
49
Malignant transformation of meningeal melanocytoma: a case report. ( 14604228 )
2003
50
Aggressive management of orbital meningeal melanocytoma. ( 14663571 )
2003

Variations for Meningeal Melanocytoma

Expression for Meningeal Melanocytoma

Search GEO for disease gene expression data for Meningeal Melanocytoma.

Pathways for Meningeal Melanocytoma

GO Terms for Meningeal Melanocytoma

Cellular components related to Meningeal Melanocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.23 ENO2 GNA11 GNAQ GPR180 MME MUC1

Biological processes related to Meningeal Melanocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 action potential GO:0001508 9.46 GNA11 GNAQ
2 G-protein coupled acetylcholine receptor signaling pathway GO:0007213 9.43 GNA11 GNAQ
3 phospholipase C-activating dopamine receptor signaling pathway GO:0060158 9.4 GNA11 GNAQ
4 replicative senescence GO:0090399 9.37 CDKN2A MME
5 Ras protein signal transduction GO:0007265 9.33 CDKN2A NRAS RREB1
6 developmental pigmentation GO:0048066 9.32 GNA11 GNAQ
7 phototransduction, visible light GO:0007603 9.26 GNA11 GNAQ
8 entrainment of circadian clock GO:0009649 8.96 GNA11 GNAQ
9 regulation of melanocyte differentiation GO:0045634 8.62 GNA11 GNAQ

Molecular functions related to Meningeal Melanocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 G-protein beta/gamma-subunit complex binding GO:0031683 9.16 GNA11 GNAQ
2 guanyl nucleotide binding GO:0019001 8.96 GNA11 GNAQ
3 type 2A serotonin receptor binding GO:0031826 8.62 GNA11 GNAQ

Sources for Meningeal Melanocytoma

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....