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Neoplasm of the nervous system

MedGen UID:: 45046
•Concept ID:: C0027766
•: Neoplastic Process

Synonym:	Nervous system neoplasm
SNOMED CT:	Neoplasm of nervous system (126950007); Tumor of nervous system (126950007)

HPO:	HP:0004375
Monarch Initiative:	MONDO:0021248

Definition

A tumor (abnormal growth of tissue) of the nervous system. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVNeoplasm of the nervous system

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Abnormality of the nervous system
        Abnormal nervous system morphology
        Neoplasm of the nervous system
        Cranial nerve neoplasm
        Abducens nerve neoplasm
        Acoustic neuroma
        Bilateral vestibular schwannoma
        Neurofibromatosis, type 2
        Unilateral vestibular schwannoma
        Facial nerve neoplasm
        Glossopharyngeal nerve neoplasm
        Hypoglossal nerve neoplasm
        Schwannoma of twelfth cranial nerve
        Malignant cranial nerve neoplasm
        Malignant neoplasm of acoustic nerve
        Malignant olfactory nerve neoplasm
        Malignant Optic Nerve Neoplasm
        Oculomotor nerve cancer
        Oculomotor nerve neoplasm
        Olfactory nerve neoplasm
        Olfactory Neurogenic Tumor
        Optic nerve neoplasm
        Bilateral meningioma of optic nerve
        Optic nerve glioma
        Optic nerve sheath meningioma
        Optic Nerve Sheath Solitary Fibrous Tumor
        Spinal accessory nerve neoplasm
        Trigeminal nerve neoplasm
        Trigeminal schwannoma
        Trochlear nerve neoplasm
        Vagus nerve neoplasm
        Vagus nerve paraganglioma
        Vestibulocochlear nerve neoplasm
        Malignant neoplasm of nervous system
        Malignant neoplasm of the central nervous system
        Anaplastic ganglioglioma
        Central Nervous System Carcinoma
        Central Nervous System Embryonal Tumor
        Central Nervous System Histiocytic Sarcoma
        Central nervous system leukemia
        Central Nervous System Melanoma
        Cerebral germinoma
        Choroid plexus carcinoma
        Glioma
        Malignant Central Nervous System Germ Cell Tumor
        Malignant central nervous system mesenchymal, non-meningothelial neoplasm
        Malignant glioma
        Malignant Intracranial Neoplasm
        Malignant tumor of meninges
        Medulloblastoma
        Metastatic Malignant Neoplasm in the Central Nervous System
        Neuroblastic tumor
        Primary central nervous system lymphoma
        Recurrent Primary Malignant Central Nervous System Neoplasm
        Refractory Primary Malignant Central Nervous System Neoplasm
        Retinoblastoma
        Spinal cord cancer
        Metastatic Malignant Neoplasm in the Nervous System
        Peripheral nervous system cancer
        Granular cell cancer
        Malignant peripheral nerve sheath tumor
        Metastatic Paraganglioma
        Peripheral ganglioneuroblastoma
        Peripheral Neuroblastoma
        Peripheral primitive neuroectodermal tumor
        Primitive neuroectodermal tumor
        Central primitive neuroectodermal tumor
        Localized Primitive Neuroectodermal Tumor
        Recurrent Primitive Neuroectodermal Tumor
        Refractory Primitive Neuroectodermal Tumor
        Neoplasm of the central nervous system
        Adult Central Nervous System Neoplasm
        Adult astrocytic tumor
        Adult central nervous system germ cell tumor
        Adult central nervous system primitive neuroectodermal neoplasm
        Adult embryonal tumor with multilayered rosettes, c19mc-altered
        Adult Ependymal Tumor
        Adult Ganglioglioma
        Adult Intracranial Neoplasm
        Adult Meningioma
        Adult Oligodendroglial Tumor
        Adult Spinal Cord Neoplasm
        Benign neoplasm of the central nervous system
        Cerebral hamartoma
        Choroid plexus papilloma
        Hemangioblastoma
        Meningioma
        Multiple central nervous system lipomas
        Neuroepithelial cyst
        Central nervous system cyst
        Arachnoid cyst
        Central Nervous System Colloid Cyst
        Central Nervous System Epidermoid Cyst
        Cerebral cyst
        Choroid plexus cyst
        Colloid cyst
        Endodermal Cyst
        Ependymal cyst
        Intracranial Colloid Cyst
        Intracranial cystic lesion
        Intraspinal Cyst
        Meningeal cyst
        Neurenteric cyst
        Perineural cyst
        Pineal cyst
        Pituitary gland cyst
        Rathke pouch cyst
        Central nervous system hematopoietic neoplasm
        Central Nervous System Histiocytic and Dendritic Cell Neoplasm
        Intracranial Myeloid Sarcoma
        Secondary Central Nervous System Lymphoma
        Childhood Central Nervous System Neoplasm
        Childhood astrocytic tumor
        Childhood Benign Central Nervous System Neoplasm
        Childhood central nervous system germ cell tumor
        Childhood Ependymal Tumor
        Childhood ganglioglioma
        Childhood Intracranial Neoplasm
        Childhood Malignant Central Nervous System Neoplasm
        Childhood Mixed Glioma
        Childhood oligodendroglioma
        Childhood spinal cord tumor
        Pediatric meningioma
        Recurrent Childhood Central Nervous System Neoplasm
        Intracranial Neoplasm
        Benign Intracranial Neoplasm
        Intracranial meningioma
        Metastatic Intracranial Malignant Neoplasm
        Neoplasm of brain
        Posterior Fossa Neoplasm
        Recurrent Intracranial Neoplasm
        Skull base neoplasm
        Visual Pathway Neoplasm
        Intraspinal Neoplasm
        Cauda equina neoplasm
        Epidural spinal canal neoplasm
        Intradural extramedullary spinal canal neoplasm
        Intraspinal meningioma
        Spinal cord tumor
        Neuronal/glioneuronal neoplasm of the central nervous system
        Central neurocytoma
        Cerebellar Glioneuronal and Neuronal Tumors
        Desmoplastic infantile astrocytoma
        Desmoplastic infantile astrocytoma/ganglioglioma
        Desmoplastic infantile ganglioglioma
        Diffuse leptomeningeal glioneuronal tumor
        Dysembryoplastic neuroepithelial tumor
        Dysembryoplastic Neuroepithelial-Like Tumor of the Septum Pellucidum
        Extraventricular neurocytoma
        Gangliocytoma
        Ganglioglioma
        Ganglioneuroma
        Lhermitte-Duclos disease
        Myxoid glioneuronal tumor
        Papillary glioneuronal tumor
        Polymorphous low grade neuroepithelial tumor of the young
        Rosette-forming glioneuronal tumor
        Primary Central Nervous System Neoplasm
        Benign neoplasm of meninges
        Benign neoplasm of spinal cord
        Central nervous system germ cell tumor
        Central Nervous System Hemangioblastoma
        Central nervous system melanocytic neoplasm
        Central nervous system mesenchymal non-meningothelial tumor
        Central Nervous System Paraganglioma
        Central Nervous System SMARCB1/INI1-Deficient Tumor
        Metastatic Primary Malignant Central Nervous System Neoplasm
        Neoplasm of the posterior pituitary
        Primary brain neoplasm
        Recurrent Primary Central Nervous System Neoplasm
        Refractory Primary Central Nervous System Neoplasm
        Sellar Chordoma
        Sellar Neuroblastoma
        Tumor of meninges
        Familial multiple meningioma
        Leptomeningeal Neoplasm
        Meningeal Carcinomatosis
        Meninges hemangiopericytoma
        Primary melanocytic lesion of meninges
        Neoplasm of the peripheral nervous system
        Autonomic nervous system neoplasm
        Paraganglioma
        Benign peripheral nerve granular cell tumor
        Childhood mediastinal neurogenic neoplasm
        Cutaneous Neural Neoplasm
        Benign dermal neurilemmoma
        Cutaneous ganglioneuroma
        Cutaneous granular cell tumor
        Cutaneous Neurofibroma
        Cutaneous Perineurioma
        Dermal Nerve Sheath Myxoma
        Malignant Peripheral Nerve Sheath Tumor of the Skin
        Hemangioma of peripheral nerve
        Intraneural perineurioma
        Lipomatosis of Nerve
        Nerve plexus neoplasm
        Brachial plexus neoplasm
        Lumbar plexus neoplasm
        Nerve sheath neoplasm
        Atypical Neurofibromatous Neoplasm of Uncertain Biologic Potential
        Granular cell tumor
        Hybrid Nerve Sheath Tumor
        Melanotic neurilemmoma
        Neurofibroma
        Neuroma
        Neurothekeoma
        Perineurioma
        Schwannoma
        Solitary Circumscribed Neuroma
        Peripheral nerve schwannoma
        Adrenal Gland Schwannoma
        Bone Schwannoma
        Breast Schwannoma
        Cardiac Schwannoma
        Colorectal Schwannoma
        Esophageal Schwannoma
        Gastric Schwannoma
        Intraocular Schwannoma
        Kidney Schwannoma
        Mediastinal schwannoma
        Neurilemmoma of the pleura
        Paratesticular Schwannoma
        Penile Schwannoma
        Schwannoma of jugular foramen
        Schwannoma of ureter
        Sternal Intraosseous Schwannoma
        Sympathetic neurilemmoma
        Thyroid Gland Schwannoma
        Peripheral neuroepithelioma
        Askin tumor
        Ewing sarcoma
        Nerve root neoplasm
        Nervous system benign neoplasm
        Benign Central Nervous System Mesenchymal, Non-Meningothelial Neoplasm
        Central nervous system chondroma
        Central nervous system hemangioma
        Central nervous system leiomyoma
        Central nervous system lipoma
        Central Nervous System Myofibroblastoma
        Central Nervous System Rhabdomyoma
        Intracranial Fibrous Histiocytoma
        Benign granular cell tumor
        Benign Anal Granular Cell Tumor
        Benign Gastric Granular Cell Tumor
        Colorectal Benign Granular Cell Tumor
        Extrahepatic Bile Duct Benign Granular Cell Tumor
        Gallbladder Benign Granular Cell Tumor
        Oral Cavity Benign Granular Cell Tumor
        Central nervous system mature teratoma
        Adult central nervous system mature teratoma
        Central Nervous System Dermoid Cyst
        Childhood central nervous system mature teratoma
        Pineal region mature teratoma
        Sellar Mature Teratoma
        Non-Metastatic Paraganglioma
        Benign adrenal gland pheochromocytoma
        Benign carotid body paraganglioma
        Neuroepithelial, Perineurial, and Schwann Cell Neoplasm
        Neuroepithelial neoplasm
        Cribriform Neuroepithelial Tumor
        Neurocytoma
        Papillary tumor of the pineal region
        Pinealoma
        Retinal cell neoplasm
        Neurolymphomatosis
        Paraneoplastic neurologic syndrome
        Anti-NMDA receptor encephalitis
        Cancer-associated retinopathy
        Eaton-Lambert syndrome
        Limbic encephalitis
        Myasthenia gravis
        Adult-onset myasthenia gravis
        Juvenile myasthenia gravis
        Neonatal myasthenia gravis
        Transient neonatal myasthenia gravis
        Opsoclonus-myoclonus syndrome
        Paraneoplastic cerebellar degeneration
        Paraneoplastic isolated brainstem encephalitis
        Paraneoplastic polyneuropathy
        Paraneoplastic Subacute Sensory Neuronopathy
        Paraneoplastic sensory ganglionopathy
        Transverse myelitis
        Neuromyelitis optica

Professional guidelines

PubMed

Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.

Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 18 doi: 10.1212/NXI.0000000000001014. PMID: 34006622 Free PMC Article

EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood.

Weller M, van den Bent M, Preusser M, Le Rhun E, Tonn JC, Minniti G, Bendszus M, Balana C, Chinot O, Dirven L, French P, Hegi ME, Jakola AS, Platten M, Roth P, Rudà R, Short S, Smits M, Taphoorn MJB, von Deimling A, Westphal M, Soffietti R, Reifenberger G, Wick W
Nat Rev Clin Oncol 2021 Mar;18(3):170-186. Epub 2020 Dec 8 doi: 10.1038/s41571-020-00447-z. PMID: 33293629 Free PMC Article

Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline.

Melmed S, Casanueva FF, Hoffman AR, Kleinberg DL, Montori VM, Schlechte JA, Wass JA; Endocrine Society
J Clin Endocrinol Metab 2011 Feb;96(2):273-88. doi: 10.1210/jc.2010-1692. PMID: 21296991

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Recent clinical studies

Etiology

High-dose radiation-induced meningiomas.

Salvati M, Cervoni L, Puzzilli F, Bristot R, Delfini R, Gagliardi FM
Surg Neurol 1997 May;47(5):435-41; discussion 441-2. doi: 10.1016/s0090-3019(96)00360-6. PMID: 9131025

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Diagnosis

The importance of escalating molecular diagnostics in patients with low-grade pediatric brain cancer.

Al Assaad M, Gundem G, Liechty B, Sboner A, Medina J, Papaemmanuil E, Sternberg CN, Marks A, Souweidane MM, Greenfield JP, Tran I, Snuderl M, Elemento O, Imielinski M, Pisapia DJ, Mosquera JM
Cold Spring Harb Mol Case Stud 2023 Dec;9(4) Epub 2024 Jan 10 doi: 10.1101/mcs.a006275. PMID: 37652664 Free PMC Article

Genomic profile of two Brazilian choroid plexus tumors by whole-exome sequencing.

Garcia FAO, Evangelista AF, Mançano BM, Moreno DA, Berardinelli GN, de Paula FE, Antoniazzi AP, Júnior CA, Lombardi I, Santana I, Teixeira GR, Costa CE, Reis RM
Cold Spring Harb Mol Case Stud 2023 Feb;9(1) Epub 2023 Mar 24 doi: 10.1101/mcs.a006245. PMID: 36963804 Free PMC Article

Molecular and clonal evolution in recurrent metastatic gliosarcoma.

Anderson KJ, Tan AC, Parkinson J, Back M, Kastelan M, Newey A, Brewer J, Wheeler H, Hudson AL, Amin SB, Johnson KC, Barthel FP, Verhaak RGW, Khasraw M
Cold Spring Harb Mol Case Stud 2020 Feb;6(1) Epub 2020 Feb 3 doi: 10.1101/mcs.a004671. PMID: 31896544 Free PMC Article

Comparative RNA-seq analysis aids in diagnosis of a rare pediatric tumor.

Sanders LM, Rangaswami A, Bjork I, Lam DL, Beale HC, Kephart ET, Durbin A, Learned K, Currie R, Lyle AG, Pfeil J, Shah AT, Lee AG, Leung SG, Behroozfard IH, Breese MR, Peralez J, Hazard FK, Lacayo N, Spunt SL, Haussler D, Salama SR, Sweet-Cordero EA, Vaske OM
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High-dose radiation-induced meningiomas.

Salvati M, Cervoni L, Puzzilli F, Bristot R, Delfini R, Gagliardi FM
Surg Neurol 1997 May;47(5):435-41; discussion 441-2. doi: 10.1016/s0090-3019(96)00360-6. PMID: 9131025

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Therapy

High-dose radiation-induced meningiomas.

Salvati M, Cervoni L, Puzzilli F, Bristot R, Delfini R, Gagliardi FM
Surg Neurol 1997 May;47(5):435-41; discussion 441-2. doi: 10.1016/s0090-3019(96)00360-6. PMID: 9131025

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Prognosis

Comparative RNA-seq analysis aids in diagnosis of a rare pediatric tumor.

DNMT3A co-mutation in an IDH1-mutant glioblastoma.

Fomchenko EI, Erson-Omay EZ, Zhao A, Bindra RS, Huttner A, Fulbright RK, Moliterno J
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BCR-NTRK2 fusion in a low-grade glioma with distinctive morphology and unexpected aggressive behavior.

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Clinical prediction guides

Molecular insights into malignant progression of atypical choroid plexus papilloma.

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Cold Spring Harb Mol Case Stud 2021 Feb;7(1) Epub 2021 Feb 19 doi: 10.1101/mcs.a005272. PMID: 33608379 Free PMC Article

Molecular and clonal evolution in recurrent metastatic gliosarcoma.

DNMT3A co-mutation in an IDH1-mutant glioblastoma.

High-dose radiation-induced meningiomas.

Salvati M, Cervoni L, Puzzilli F, Bristot R, Delfini R, Gagliardi FM
Surg Neurol 1997 May;47(5):435-41; discussion 441-2. doi: 10.1016/s0090-3019(96)00360-6. PMID: 9131025

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Neoplasm of the nervous system

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Genetic Testing Registry

Clinical resources

Practice guidelines

Consumer resources

Reviews

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