WHO classification of the tumors of the central nervous system
Encyclopedia
Here it is presented a simplified version of the last WHO
classification of the tumors of the central nervous system
.
For each tumor there are the WHO official name, the ICD-O code (with Arabic numeral, where /0 indicates "benign" tumor, /3 malignant tumor and /1 borderline tumor), and with Roman numeral the WHO Grade (a parameter connected with the "aggressiveness" of the tumor).See the article Grading of the tumors of the central nervous system
.
Who
Who may refer to:* Who , an English-language pronoun* who , a Unix command* Who?, one of the Five Ws in journalism- Art and entertainment :* Who? , a 1958 novel by Algis Budrys...
classification of the tumors of the central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...
.
For each tumor there are the WHO official name, the ICD-O code (with Arabic numeral, where /0 indicates "benign" tumor, /3 malignant tumor and /1 borderline tumor), and with Roman numeral the WHO Grade (a parameter connected with the "aggressiveness" of the tumor).See the article Grading of the tumors of the central nervous system
Grading of the tumors of the central nervous system
The concept of grading of the tumors of the central nervous system, agreeing for such the regulation of the "progressiveness" of these neoplasias , dates back to 1926 and was introduced by P. Bailey and H...
.
1. Tumours of neuroepithelial tissue
- 1.1. Astrocytic tumours
- 1.1.1 Pilocytic astrocytomaPilocytic astrocytomaPilocytic astrocytoma or juvenile pilocytic astrocytoma or cystic cerebellar astrocytoma is a neoplasm of the brain that occurs more often in children and young adults...
(IDC-O 9421/1, WHO grade I)- 1.1.1a Pilomyxoid astrocytoma (IDC-O 9425/3, WHO grade II)
- 1.1.2 Subependymal giant cell cstrocytoma (IDC-O 9384/1, WHO grade I)
- 1.1.3 Pleomorphic xanthoastrocytomaPleomorphic xanthoastrocytomaPleomorphic Xanthoastrocytoma is a neoplasm of the brain that occurs often in children and teenagers. They usually arise supratentorial and superficially from the cerebral hemispheres of the brain and in contact with the leptomeninges, rarely they arise from the spinal cord. They are formed...
(ICD-O 9424/3, WHO grade II) - 1.1.4 Diffuse astrocytoma (IDC-O 9400/3, WHO grade II)
- 1.1.5 Anaplastic astrocytomaAnaplastic astrocytoma-Symptoms:Initial presenting symptoms most commonly are headache, depressed mental status, focal neurological deficits, and/or seizures. The growth rate and mean interval between onset of symptoms and diagnosis is approximately 1.5–2 years but is highly variable, being intermediate between that of...
(IDC-O 9401/3, WHO grade III) - 1.1.6.Glioblastoma (IDC-O 9440/3, WHO grade IV)
- 1.1.6a Giant cell glioblastomaGiant cell glioblastomaThe giant-cell glioblastoma is a histological variant of glioblastoma, presenting a prevalence of bizarre, multinucleated giant cells....
(IDC-O 9441/3, WHO grade IV) - 1.1.6b GliosarcomaGliosarcomaGliosarcoma is a rare type of glioma, a cancer of the brain that comes from glial, or supportive, brain cells, as opposed to the neural brain cells. Gliosarcoma is a malignant cancer, and is defined as a glioblastoma consisting of gliomatous and sarcomatous components.It is estimated that...
(IDC-O 9442/3, WHO grade IV)
- 1.1.6a Giant cell glioblastoma
- 1.1.7 Gliomatosis cerebriGliomatosis cerebriGliomatosis cerebri is a rare primary brain tumor. It is commonly characterized by diffuse infiltration of the brain with neoplastic glial cells that affect various areas of the cerebral lobes...
(IDC-O 9381/3, WHO grade III)
- 1.1.1 Pilocytic astrocytoma
- 1.2. Oligodendroglial tumours
- 1.2.1 OligodendrogliomaOligodendrogliomaOligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults but are also found in children...
(IDC-O 9450/3, WHO grade II) - 1.2.2 Anaplastic oligodendrogliomaOligodendrogliomaOligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults but are also found in children...
(IDC-O 9451/3, WHO grade III)
- 1.2.1 Oligodendroglioma
- 1.3. Oligoastrocytic tumours
- 1.3.1 OligoastrocytomaOligoastrocytomaOligoastrocytomas are a subset of brain tumors that present with an appearance of mixed glial cell origin, astrocytoma and oligodendroglioma. These types of glial cells that become cancerous are involved with insulating and regulating the activity of neuron cells in the central nervous system...
(IDC-O 9382/3, WHO grade II) - 1.3.2 Anaplastic oligoastrocytomaOligoastrocytomaOligoastrocytomas are a subset of brain tumors that present with an appearance of mixed glial cell origin, astrocytoma and oligodendroglioma. These types of glial cells that become cancerous are involved with insulating and regulating the activity of neuron cells in the central nervous system...
(IDC-O 9382/3, WHO grade III)
- 1.3.1 Oligoastrocytoma
- 1.4. Ependymal tumours
- 1.4.1 SubependymomaSubependymomaA subependymoma is a type of brain tumor; specifically, it is a rare form of ependymal tumor.The prognosis for a subependymoma is better than for most ependymal tumors, and is considered a grade I/IV tumor in the World Health Organization classification.They are classically found within the fourth...
(IDC-O 9383/1, WHO grade I) - 1.4.2 Myxopapillary ependymoma (IDC-O 9394/1, WHO grade I)
- 1.4.3 EpendymomaEpendymomaEpendymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymoma is the fourth ventricle...
(IDC-O 9391/3, WHO grade II) - 1.4.4 Anaplastic ependymoma (IDC-O 9392/3, WHO grade III)
- 1.4.1 Subependymoma
- 1.5. Choroid plexus tumours
- 1.5.1 Choroid plexus papillomaChoroid plexus papillomaA Choroid plexus papilloma is a rare, slow-growing, histologically benign intracranial neoplasm or tumor that is commonly located in the ventricular system of the choroid plexus. It may obstruct the cerebrospinal fluid flow, causing increased intracranial pressure.-Pathophysiology:The tumor is...
(ICD-O 9390/0, WHO grade I) - 1.5.2 Atypical choroid plexus papilloma ( ICD-O 9390/1, WHO grade II)
- 1.5.3 Choroid plexus carcinomaChoroid plexus carcinoma-Signs and symptoms:the symptoms of choroid plexus carcinoma are similar to those of other brain tumors,and include:*Persistent or new onset headaches*Macrocephaly or bulging fontanels in infants.*Loss of appetite...
(ICD-O 9390/3, WHO grade III)
- 1.5.1 Choroid plexus papilloma
- 1.6. Other neuroepithelial tumours
- 1.6.1 AstroblastomaAstroblastomaAstroblastoma is a rare, glial tumor derived from the astroblast, a type of cell that closely resembles spongioblastoma and astrocytes. Astroblastoma cells are most likely found in the supratentorial region of the brain that houses the cerebrum, an area responsible for all voluntary movements in...
(IDC-O 9430/3, WHO grade I) - 1.6.2 Chordoid glioma of the third ventricle (IDC-O 9444/1, WHO grade II)
- 1.6.3 Angiocentric glioma (IDC-O 9431/1, WHO grade I)
- 1.6.1 Astroblastoma
- 1.7. Neuronal and mixed neuronal-glial tumours
- 1.7.1 Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos) (IDC-O 9493/0)
- 1.7.2 Desmoplastic infantile astrocytoma/ganglioglioma (IDC-O 9412/1, WHO grade I)
- 1.7.3 Dysembryoplastic neuroepithelial tumourDysembryoplastic neuroepithelial tumourDysembryoplastic neuroepithelial tumour, commonly abbreviated DNT or DNET, is a type of brain tumour.It appears similar to oligodendroglioma, but with visible neurons....
(IDC-O 9413/0, WHO grade I) - 1.7.4 Gangliocytoma (IDC-O 9492/0, WHO grade I)
- 1.7.5 GangliogliomaGangliogliomaGanglioglioma is a tumour that arises from ganglion cells in the central nervous system.The term "gangliocytoma" is sometimes equated with ganglioglioma. However, it is also sometimes equated with ganglioneuroma. In this context, the glial nature of the tumor is de-emphasized...
(IDC-O 9505/1, WHO grade I) - 1.7.6 Anaplastic ganglioglioma (IDC-O 9505/3, WHO grade III)
- 1.7.7 Central neurocytoma (IDC-O 9506/1, WHO grade II)
- 1.7.8 Extraventricular neurocytoma (IDC-O 9506/1, WHO grade II)
- 1.7.9 Cerebellar liponeurocytoma (IDC-O 9506/1, WHO grade II)
- 1.7.10 Papillary glioneuronal tumour (IDC-O 9509/1, WHO grade I)
- 1.7.11 Rosette-forming glioneuronal tumour of the fourth ventricle (IDC-O 9509/1, WHO grade I)
- 1.7.12 ParagangliomaParagangliomaA paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites . About 97% are benign and cured by surgical removal; the remaining 3% are malignant because they are able to produce distant metastases...
(IDC-O 8680/1, WHO grade I)
- 1.8. Tumours of the pineal region
- 1.8.1 PineocytomaPineocytomaPineocytoma, also known as a pinealocytoma, is a benign, slowly-growing tumor of the pineal gland. Unlike the similar condition pineal gland cyst, it is uncommon.-Diagnosis:Pineocytomas are diagnosed from tissue, i.e...
(IDC-O 9361/1, WHO grade I) - 1.8.2 Pineal parenchymal tumour of intermediate differentiation (IDC-O 9362/3, WHO grade II, III)
- 1.8.3 Pineoblastoma (IDC-O 9362/3, WHO grade IV)
- 1.8.4 Papillary tumour of the pineal region (IDC-O 9395/3, WHO grade II, III)
- 1.8.1 Pineocytoma
- 1.9. Embryonal tumours
- 1.9.1 MedulloblastomaMedulloblastomaMedulloblastoma is a highly malignant primary brain tumor that originates in the cerebellum or posterior fossa.Previously, medulloblastomas were thought to represent a subset of primitive neuroectodermal tumor of the posterior fossa...
(IDC-O 9470/3, WHO grade IV)- 1.9.1b Medulloblastoma with extensive nodularity (IDC-O 9471/3, WHO grade IV)
- 1.9.1c Anaplastic medulloblastoma (IDC-O 9474/3, WHO grade IV)
- 1.9.2. CNS Primitive neuroectodermal tumour (IDC-O 9473/3, WHO grade IV)
- 1.9.2a CNS Neuroblastoma (IDC-O 9500/3, WHO grade IV)
- 1.9.3 Atypical teratoid/rhabdoid tumour (IDC-O 9508/3, WHO grade IV)
- 1.9.1 Medulloblastoma
2. Tumours of cranial and paraspinal nerves
- 2.1 SchwannomaSchwannomaA schwannoma is a benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves....
(IDC-O 9560/0, WHO grade I) - 2.2 NeurofibromaNeurofibromaA neurofibroma is a benign nerve sheath tumor in the peripheral nervous system. Usually found in individuals with neurofibromatosis type I , an autosomal dominant genetically-inherited disease, they can result in a range of symptoms from physical disfiguration and pain to cognitive disability...
(IDC-O 9540/0, WHO grade I) - 2.3 Perineurioma (IDC-O 9571/0, 9571/3, WHO grade I, II, III)
- 2.4 Malignant peripheral nerve sheath tumour (MPNST) (IDC-O 9540/3, WHO grade II, III, IV)
3. Tumours of the meninges
- 3.1 Tumours of meningothelial cells
- 3.1.1 MeningiomaMeningiomaThe word meningioma was first used by Harvey Cushing in 1922 to describe a tumor originating from the meninges, the membranous layers surrounding the CNS ....
(IDC-O 9530/0, WHO grade I)- 3.1.1l Atypical meningioma (IDC-O 9539/1, WHO grade II)
- 3.1.1o Anaplastic meningioma (IDC-O 9530/3, WHO grade III)
- 3.1.1 Meningioma
- 3.2 Mesenchymal tumours
- 3.2.1 LipomaLipomaA lipoma is a benign tumor composed of adipose tissue. It is the most common form of soft tissue tumor. Lipomas are soft to the touch, usually movable, and are generally painless. Many lipomas are small but can enlarge to sizes greater than six centimeters. Lipomas are commonly found in adults...
(IDC-O 8850/0) - 3.2.2 AngiolipomaAngiolipomaAngiolipoma is a subcutaneous nodule with vascular structure, having all other features of a typical lipoma. They are commonly painful....
(IDC-O 8861/0) - 3.2.3 HibernomaHibernomaA hibernoma is a benign tumour of brown fat, i.e. adipose tissue , and thus a type of lipoma.They arise when brown fat continue to grow.-Histology:...
(IDC-O 8880/0) - 3.2.4 LiposarcomaLiposarcomaLiposarcoma is a malignant tumor that arises in fat cells in deep soft tissue, such as that inside the thigh or in the retroperitoneum.They are typically large bulky tumors which tend to have multiple smaller satellites extending beyond the main confines of the tumor.Liposarcomas, like all...
(IDC-O 8850/3) - 3.2.5 Solitary fibrous tumour (IDC-O 8815/0)
- 3.2.6 FibrosarcomaFibrosarcomaFibrosarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells.Usually in males ages 30 to 40. Originates in fibrous tissues of the bone. Invades long or flat bones such as femur, tibia,...
(IDC-O 8810/3) - 3.2.7 Malignant fibrous histiocytomaMalignant fibrous histiocytomaPleomorphic undifferentiated sarcoma , also undifferentiated pleomorphic sarcoma and previously malignant fibrous histiocytoma , is a type of soft tissue sarcoma....
(IDC-O 8830/3) - 3.2.8 LeiomyomaLeiomyomaA leiomyoma is a benign smooth muscle neoplasm that is not premalignant. They can occur in any organ, but the most common forms occur in the uterus, small bowel and the esophagus.- Etymology:* Greek:** λεῖος leios "smooth"...
(IDC-O 8890/0) - 3.2.9 LeiomyosarcomaLeiomyosarcomaLeiomyosarcoma , aka LMS, is a malignant cancer of smooth muscle....
(IDC-O 8890/3) - 3.2.10 RhabdomyomaRhabdomyomaA rhabdomyoma is a benign tumor of striated muscle. Rhabdomyomas may be either "cardiac" or "extracardiac" . Extracardiac forms of rhabdomyoma are subclassified into three distinct types: Adult type, Fetal type, and Genital type.Cardiac rhabdomyomas are the most common primary tumor of the heart...
(IDC-O 8900/0) - 3.2.11 RhabdomyosarcomaRhabdomyosarcomaA rhabdomyosarcoma is a type of cancer, specifically a sarcoma , in which the cancer cells are thought to arise from skeletal muscle progenitors. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location...
(IDC-O 8900/3) - 3.2.12 ChondromaChondromaA chondroma is a benign cartilaginous tumor, which is encapsulated with a lobular growing pattern.Tumor cells resemble normal cells and produce the cartilaginous matrix ....
(IDC-O 9220/0) - 3.2.13 ChondrosarcomaChondrosarcomaChondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. Chondrosarcoma is a member of a category of "soft tissue" malignancies known as sarcomas. About 30% of skeletal system cancers are chondrosarcomas...
(IDC-O 9220/3) - 3.2.14 OsteomaOsteomaAn osteoma is a new piece of bone usually growing on another piece of bone, typically the skull. It is a benign tumor.When the bone tumor grows on other bone it is known as "homoplastic osteoma"; when it grows on other tissue it is called "heteroplastic osteoma".Osteoma represents the most common...
(IDC-O 9180/0) - 3.2.15 OsteosarcomaOsteosarcomaOsteosarcoma is an aggressive cancerous neoplasm arising from primitive transformed cells of mesenchymal origin that exhibit osteoblastic differentiation and produce malignant osteoid...
(IDC-O 9180/3) - 3.2.16 OsteochondromaOsteochondromaOsteochondroma is a type of benign tumor that consists of cartilage and bone. It is a benign capped-cartilage outgrowth, connected to bone by a stalk.It is the most frequently observed neoplasm of the skeleton....
(IDC-O 9210/0) - 3.2.17 Haemangioma (IDC-O 9120/0)
- 3.2.18 Epithelioid hemangioendotheliomaEpithelioid hemangioendotheliomaEpithelioid hemangioendothelioma is a rare tumor, first characterized by Sharon Weiss, M.D., that both clinically and histologically is intermediate between angiosarcoma and hemangioma. It is a vascular cancer in the lining of the blood vessels in the liver and lungs so rare that only 0.01 percent...
(IDC-O 9133/1) - 3.2.19 Haemangiopericytoma (IDC-O 9150/1, WHO grade II)
- 3.2.20 Anaplastic haemangiopericytoma (IDC-O 9150/3, WHO grade III)
- 3.2.21 AngiosarcomaAngiosarcomaAngiosarcoma is a malignant neoplasm of endothelial-type cells that line vessel walls. This may be in reference to blood or lymphatic vessels ....
(IDC-O 9120/3) - 3.2.22 Kaposi Sarcoma (IDC-O 9140/3)
- 3.2.23 Ewing SarcomaEwing sarcomaEwing sarcoma is a malignant round-cell tumour. It is a rare disease in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, the ribs and clavicle....
- PNET (IDC-O 9364/3)
- 3.2.1 Lipoma
- 3.3 Primary melanocytic lesions
- 3.3.1 Diffuse melanocytosis (IDC-O 8728/0)
- 3.3.2 Melanocytoma (IDC-O 8728/1)
- 3.3.3 Malignant melanoma (IDC-O 8720/3)
- 3.3.4 Meningeal melanomatosis (IDC-O 8728/3)
- 3.4 Other neoplasms related to the meninges
- 3.4.1 Haemangioblastoma (IDC-O 9161/1, WHO grade I)
4. Tumors of the haematopoietic system
- 4.1 Malignant LymphomasPrimary central nervous system lymphomaA primary central nervous system lymphoma , also known as microglioma and primary brain lymphoma, is a primary intracranial tumor appearing mostly in patients with severe immunosuppression . PCNSLs represent around 20% of all cases of lymphomas in HIV infections...
(IDC-O 9590/3) - 4.2 Plasmocytoma (IDC-O 9731/3)
- 4.3 Granulocytic sarcomaGranulocytic sarcomaA myeloid sarcoma , is a solid tumor composed of immature white blood cells called myeloblasts...
(IDC-O 9930/3)
5. Germ cell tumours
- 5.1 GerminomaGerminomaA germinoma is a type of germ cell tumor which is not differentiated upon examination. It may be benign or malignant.-Classification:The term germinoma most often has referred to a tumor in the brain that has a histology identical to two other tumors: dysgerminoma in the ovary and seminoma in the...
(IDC-O 9064/3) - 5.2 Embryonal carcinomaEmbryonal carcinomaEmbryonal carcinoma is a relatively uncommon type of germ cell tumour that occurs in the ovaries and testes.-Ovarian embryonal carcinoma:In the ovary, embryonal carcinoma is quite rare, amounting to approximately three percent of ovarian germ cell tumours. The median age at diagnosis is 15 years...
(IDC-O 9070/3) - 5.3 Yolk sac tumour (IDC-O 9071/3)
- 5.4 ChoriocarcinomaChoriocarcinomaChoriocarcinoma is a malignant, trophoblastic and aggressive cancer, usually of the placenta. It is characterized by early hematogenous spread to the lungs...
(IDC-O 9100/3) - 5.5 TeratomaTeratomaA teratoma is an encapsulated tumor with tissue or organ components resembling normal derivatives of all three germ layers. There are rare occasions when not all three germ layers are identifiable...
(IDC-O 9080/1) - 5.6 Mixed germ cell tumours (IDC-O 9085/3)
6. Tumours of the sellar region
- 6.1 CraniopharyngiomaCraniopharyngiomaCraniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue, that occurs most commonly in children but also in men and women in their 50s and 60s....
(IDC-O 9350/1, WHO grade I) - 6.2 Granular cell tumour (IDC-O 9582/0, WHO grade I)
- 6.3 PituicytomaPituicytomaPituicytoma is a rare brain tumor. It grows at the base of the brain from the pituitary gland. This tumor is thought to be derived from the parenchymal cells of the posterior lobe of the pituitary gland, the so-called pituicytes. Some researchers believe that they arise from the folliculostellate...
(IDC-O 9432/1, WHO grade I) - 6.4 Spindle cell oncocytoma of the adenohypophysis (IDC-O 8991/0, WHO grade I)
External links
- AFIP Course Syllabus - Astrocytoma WHO Grading Lecture Handout