Cerebellar Neoplasms
Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study. (1/828)
PURPOSE: From 1986 to 1992, "eight-drugs-in-one-day" (8-in-1) chemotherapy both before and after radiation therapy (XRT) (54 Gy tumor/36 Gy neuraxis) was compared with vincristine, lomustine (CCNU), and prednisone (VCP) after XRT in children with untreated, high-stage medulloblastoma (MB). PATIENTS AND METHODS: Two hundred three eligible patients with an institutional diagnosis of MB were stratified by local invasion and metastatic stage (Chang T/M) and randomized to therapy. Median time at risk from study entry was 7.0 years. RESULTS: Survival and progression-free survival (PFS) +/- SE at 7 years were 55%+/-5% and 54%+/-5%, respectively. VCP was superior to 8-in-1 chemotherapy, with 5-year PFS rates of 63%+/-5% versus 45%+/-5%, respectively (P = .006). Upon central neuropathology review, 188 patients were confirmed as having MB and were the subjects for analyses of prognostic factors. Children aged 1.5 to younger than 3 years had inferior 5-year estimates of PFS, compared with children 3 years old or older (P = .0014; 32%+/-10% v 58%+/-4%, respectively). For MB patients 3 years of age or older, the prognostic effect of tumor spread (MO v M1 v M2+) on PFS was powerful (P = .0006); 5-year PFS rates were 70%+/-5%, 57%+/-10%, and 40%+/-8%, respectively. PFS distributions at 5 years for patients with M0 tumors with less than 1.5 cm2 of residual tumor, versus > or = 1.5 cm2 of residual tumor by scan, were significantly different (P = .023; 78%+/-6% v 54%+/-11%, respectively). CONCLUSION: VCP plus XRT is a superior adjuvant combination compared with 8-in-1 chemotherapy plus XRT. For patients with M0 tumors, residual tumor bulk (not extent of resection) is a predictor for PFS. Patients with M0 tumors, > or = 3 years with < or = 1.5 cm2 residual tumor, had a 78%+/-6% 5-year PFS rate. Children younger than 3 years old who received a reduced XRT dosage had the lowest survival rate. (+info)Hemangioblastoma mimicking tentorial meningioma: preoperative embolization of the meningeal arterial blood supply--case report. (2/828)
A 72-year-old male presented with a primary hemangioblastoma of the posterior fossa with unusual dural attachment and meningeal arterial blood supply from the external carotid artery and marginal tentorial artery. Preoperative embolization facilitated complete resection of the tumor with no resultant neurological deficit. Hemangioblastoma must be included in the differential diagnosis of tumors with dural involvement. Preoperative embolization is very useful in such tumors. (+info)Activation of the CD95 (APO-1/Fas) pathway in drug- and gamma-irradiation-induced apoptosis of brain tumor cells. (3/828)
Chemotherapeutic agents and gamma-irradiation used in the treatment of brain tumors, the most common solid tumors of childhood, have been shown to act primarily by inducing apoptosis. Here, we report that activation of the CD95 pathway was involved in drug- and gamma-irradiation-induced apoptosis of medulloblastoma and glioblastoma cells. Upon treatment CD95 ligand (CD95-L) was induced that stimulated the CD95 pathway by crosslinking CD95 via an autocrine/paracrine loop. Blocking CD95-L/receptor interaction using F(ab')2 anti-CD95 antibody fragments strongly reduced apoptosis. Apoptosis depended on activation of caspases (interleukin 1beta-converting enzyme/Ced-3 like proteases) as it was almost completely abrograted by the broad range caspase inhibitor benzyloxycarbonyl-Val-Ala-Asp-fluoromethyl ketone. Apoptosis was mediated by cleavage of the receptor proximal caspase FLICE/MACH (caspase-8) and the downstream caspase CPP32 (caspase-3, Apopain) resulting in cleavage of the prototype caspase substrate PARP. Moreover, CD95 was upregulated in wild-type p53 cells thereby increasing responsiveness towards CD95 triggering. Since activation of the CD95 system upon treatment was also found in primary medulloblastoma cells ex vivo, these findings may have implications to define chemosensitivity and to develop novel therapeutic strategies in the management of malignant brain tumors. (+info)Cicatricial fibromatosis mimics metastatic medulloblastoma. (4/828)
Cicatricial fibromatoses usually occur in the anterior abdominal wall or in the extremities, but rarely in the scalp or the soft tissues of the neck. We report a case of desmoid fibromatosis that developed in a 15-year-old boy 8 months after surgery for cerebellar medulloblastoma. (+info)Unexpected stomach uptake of technetium-99m-MDP. (5/828)
Two pediatric cases are described in which the results of each patient's bone scan demonstrated abnormal stomach uptake. There have been a number of reports in the literature describing stomach uptake of bone agents, however, it is an uncommon finding. (+info)Clinical features and outcomes in patients with non-acoustic cerebellopontine angle tumours. (6/828)
OBJECTIVES: Non-acoustic tumours of the cerebellopontine angle differ from vestibular schwannomas in their prevalence, clinical features, operative management, and surgical outcome. These features were studied in patients presenting to the regional neuro-otological unit. METHODS: A retrospective analysis of clinical notes identified 42 patients with non-acoustic tumours of the cerebellopontine angle. Data were extracted regarding presenting clinical features, histopathological data after surgical resection, surgical morbidity and mortality, and clinical outcome (mean 32 months follow up). RESULTS: The study group comprised 25 meningiomas (60%), 12 epidermoid cysts/cholesteatomata (28%), and five other tumours. In patients with meningiomas, symptoms differed considerably from patients presenting with vestibular schwannomas. Cerebellar signs were present in 52% and hearing loss in only 68%. Twenty per cent of patients had hydrocephalus at the time of diagnosis. After surgical resection, normal facial nerve function was preserved in 75% of cases. In the epidermoid group, fifth, seventh, and eighth nerve deficits were present in 42%, 33%, and 66% respectively. There were no new postoperative facial palsies. There were two recurrences (17%) requiring reoperation. Overall, there were two perioperative deaths from pneumonia and meningitis. CONCLUSIONS: Patients with non-acoustic lesions of the cerebellopontine angle often present with different symptoms and signs from those found in patients with schwannomas. Hearing loss is less prevalent and cerebellar signs and facial paresis are more common as presenting features. Hydrocephalus is often present in patients presenting with cerebellopontine angle meningiomas. Non-acoustic tumours can usually be resected with facial nerve preservation. (+info)Medullomyoblastoma: A case report. (7/828)
Medullomyoblastoma is a rare tumour seen in childhood. We report a medullomyoblastoma occurring in the cerebellar vermis of a 4 year old boy. The light microscopic features, immunohistochemistry and histogenesis are described. (+info)Choroid plexus papilloma of cerebellopontine angle with extension to foramen magnum. (8/828)
A case of choroid plexus papilloma resembling meningioma of cerebellopontine (CP) angle with its extension to foramen magnum is presented. Occurrence of this tumour in CP angle is very rare. Its extension towards foramen magnum is further rare. It was a real diagnostic enigma preoperatively as the tumour was resembling meningioma upto some extent on radiological study. Retromastoid craniectomy with microsurgical excision of tumour and its extension was achieved in toto. Tumour was attached to few rootlets of lower cranial nerves which were preserved. Attachment of the tumour with lower cranial nerves again caused diagnostic confusion with neurofibroma intraoperatively. (+info)Cerebellar neoplasms refer to abnormal growths or tumors that develop in the cerebellum, which is the part of the brain responsible for coordinating muscle movements and maintaining balance. These tumors can be benign (non-cancerous) or malignant (cancerous), and they can arise from various types of cells within the cerebellum.
The most common type of cerebellar neoplasm is a medulloblastoma, which arises from primitive nerve cells in the cerebellum. Other types of cerebellar neoplasms include astrocytomas, ependymomas, and brain stem gliomas. Symptoms of cerebellar neoplasms may include headaches, vomiting, unsteady gait, coordination problems, and visual disturbances. Treatment options depend on the type, size, and location of the tumor, as well as the patient's overall health and age. Treatment may involve surgery, radiation therapy, chemotherapy, or a combination of these approaches.
List of MeSH codes (C04)
Cerebellar vermis
List of MeSH codes (C10)
List of diseases (P)
Neuronal lineage marker
List of diseases (C)
List of diseases (T)
List of diseases (L)
Diffuse leptomeningeal glioneuronal tumor
ZNF238
Epstein-Barr virus-associated lymphoproliferative diseases
List of diseases (B)
Intention tremor
Astroblastoma
Lipofuscin
Autoimmune autonomic ganglionopathy
Medulloblastoma
Erdheim-Chester disease
List of MeSH codes (C23)
Cerebritis
WHO classification of tumours of the central nervous system
List of cancer types
Cowden syndrome
List of diseases (S)
Meningioma
List of diseases (A)
Diffuse large B-cell lymphoma associated with chronic inflammation
Gluten-sensitive enteropathy-associated conditions
Pineal gland
Index of oncology articles
Cerebellar Neoplasms | GMR | Genetics and Molecular Research | The Original by FUNPEC-RP
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Molecular/Genetics
List of MeSH codes (C04) - Wikipedia
00134003 | PEIR Digital Library
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HuGE Navigator|Genopedia|PHGKB
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Cerebellum9
- GAD65 is involved in the synthesis, packaging, and release of GABA, whereas the other three play important roles in the induction of long-term depression (LTD). Thus, the auto-antibodies toward these synaptic molecules likely impair fundamental synaptic machineries involved in unique functions of the cerebellum, potentially leading to the development of cerebellar ataxias (CAs). (springer.com)
- Autoimmunity affects the cerebellum, leading to the manifestations of the cerebellar ataxias (CAs), termed immune-mediated cerebellar ataxia (IMCAs). (springer.com)
- The cerebellar vermis (from Latin vermis, "worm") is located in the medial, cortico-nuclear zone of the cerebellum, which is in the posterior fossa of the cranium. (wikipedia.org)
- The cerebellum develops in a rostro-caudal manner, with rostral regions in the midline giving rise to the vermis, and caudal regions developing into the cerebellar hemispheres. (wikipedia.org)
- Postnatally, proliferation and organization of the cellular components of the cerebellum continues, with completion of the foliation pattern by 7 months of life and final migration, proliferation, and arborization of cerebellar neurons by 20 months. (wikipedia.org)
- Anomalies of the cerebellar vermis are diagnosed in this manner and include phenotypes consistent with Dandy-Walker malformation, rhombencephalosynapsis, displaying no vermis with fusion of the cerebellar hemispheres, pontocerebellar hypoplasia, or stunted growth of the cerebellum, and neoplasms. (wikipedia.org)
- They have important implications for our understanding of the cognitive sequelae of early cerebellar damage as well as the involvement of the cerebellum in task switching. (tau.ac.il)
- Cerebellar glioblastoma multiforme (GBM) is extremely rare in the cerebellum at any age but especially in children. (openneuroimagingjournal.com)
- In the reported bovine, after necropsy, this neoplasm was found in cerebellum, region of cerebellar vermis. (ufsc.br)
Vermis7
- The majority of medulloblastomas arise from the inferior cerebellar vermis, from which they extend into and typically fill the fourth ventricle. (medscape.com)
- The vermis is intimately associated with all regions of the cerebellar cortex, which can be divided into three functional parts, each having distinct connections with the brain and spinal cord. (wikipedia.org)
- This region comprises the vermis and intermediate parts of the cerebellar hemispheres. (wikipedia.org)
- Medulloblastomas most commonly occur in the cerebellar vermis. (uab.edu)
- Imaging studies depict the typical location in the cerebellar vermis, with filling and enlargement of the fourth ventricle. (uab.edu)
- About one half arise in the optic pathways or hypothalamus, and one third arise in the cerebellar hemispheres or vermis. (uab.edu)
- We report a case of a 10-year-old girl who suffered a cerebellar haemorrhage and subsequent CMS following surgical treatment of a ruptured arteriovenous malformation (AVM) in the cerebellar vermis. (regionh.dk)
Tumor6
- [ 5 ] Other, rarer cystic lesions, such as solid pseudopapillary epithelial neoplasm and cystic pancreatic neuroendocrine tumor (cPNET), tend to harbor features that suggest a specific diagnosis, usually leading to surgical removal. (medscape.com)
- Cerebral meningioma is the most frequently reported primary brain tumor of cats and accounts for almost 10% of all nonhematopoietic neoplasms. (vin.com)
- Boor, PJ & Schoene, WC 1975, ' Fetal Cerebellar Tissue Associated with a Primitive Neuro-epithelial Tumor in an Ovarian Teratoma ', Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques , vol. 2, no. 2, pp. 139-141. (utmb.edu)
- there was a negative correlation with cerebellar hemisphere tumor location. (psu.edu)
- The V600E point mutation is also observed in other low-grade gliomas and glioneuronal neoplasms, including approximately two-thirds of pleomorphic xanthoastrocytomas, and lower percentages of ganglioglioma, desmoplastic infantile ganglioglioma, dysembrioplastic neuroepithelial tumor, and papillary craniopharyngioma. (blogspot.com)
- She was submitted for posterior fossa craniotomy and microsurgical resection of cerebellar tumor and then to 18 Gy adjuvant radiotherapy to the tumor bed and 23 Gy to the neuroaxis. (surgicalneurologyint.com)
Lymphoma2
- The histologic type of the associated neoplasm is usually carcinoma or lymphoma. (childrensmercy.org)
- 1 B cell neoplasms other than multiple myeloma including non-Hodgkin's lymphomas, and acute and chronic leukaemias might also exhibit lytic bone lesions, hypercalcaemia, and monoclonal gammopathy via the particular actions of interleukin (IL-1), IL-6, or tumour necrosis factor-α secreted by the neoplastic B cell clone, but not reported previously secondary to a primary cerebral lymphoma. (bmj.com)
Carcinoma1
- Paraneoplastic cerebellar syndrome and sensory ganglionopathy with papillary thyroid carcinoma. (ox.ac.uk)
Tumors6
- Medulloblastomas are primarily childhood tumors, where they account for one third of all posterior fossa neoplasms. (uab.edu)
- Pituitary gland neoplasms and tumors arising from cranial nerves are considered secondary brain tumors. (vin.com)
- Neoplasms of the brain and spinal cord derived from glial cells which vary from histologically benign forms to highly anaplastic and malignant tumors. (embl.de)
- The clinical features of patients with cerebellar GBM are similar to those of other aggressive fast growing infratentorial tumors. (openneuroimagingjournal.com)
- Cerebellar hemispheric tumors of older children and adults show similar imaging features, except that contrast enhancement is more variable than in young children. (medscape.com)
- Medulloblastoma is an embryonal neoplasm and accounts for 1% of all adult intracranial tumors. (surgicalneurologyint.com)
Paraneoplastic6
- Paraneoplastic Cerebellar Degeneration" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (childrensmercy.org)
- This graph shows the total number of publications written about "Paraneoplastic Cerebellar Degeneration" by people in this website by year, and whether "Paraneoplastic Cerebellar Degeneration" was a major or minor topic of these publications. (childrensmercy.org)
- Below are the most recent publications written about "Paraneoplastic Cerebellar Degeneration" by people in Profiles. (childrensmercy.org)
- Paraneoplastic Neurological Syndromes (PNSs) are rare syndromes caused by or associated with an underlying neoplasm. (athenslab.gr)
- The following table summarizes the anti-neural antibodies, the associated paraneoplastic neurological syndromes, and the most common underlying neoplasms. (athenslab.gr)
- Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to a neoplasm. (medscape.com)
Malignant2
- Malignant neoplasms show a greater degree of anaplasia and have the properties of invasion and metastasis, compared to benign neoplasms . (lookformedical.com)
- Glioblastoma Multiforme is a highly malignant central nervous system neoplasm. (openneuroimagingjournal.com)
Hemispheres1
- A smaller proportion of medulloblastomas occur in one of the cerebellar hemispheres of patients who are typically older (adolescents or young adults), a subset in which the desmoplastic/nodular variant predominates. (medscape.com)
Posterior2
- Neoplasms of the posterior fossa. (medlineplus.gov)
- Cerebellar mutism syndrome (CMS) is a well-known complication of posterior fossa (PF) tumour surgery. (regionh.dk)
Medulloblastoma1
- Magnetic resonance image showing a lateral cerebellar mass with a "grapelike" lobular appearance characteristic of the extensively nodular medulloblastoma. (medscape.com)
Syndrome1
- Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. (psu.edu)
Benign2
- 1. A benign, slowly growing, cerebellar neoplasm composed of capillary-forming endothelial cells. (wordinfo.info)
- Objective and Importance: Aneurysmal bone cysts (ABC) are benign bone neoplasms which typically involve the spine and long bones. (johnshopkins.edu)
Astrocytoma1
- Astrocytoma, childhood cerebellar or cerebral. (lookfordiagnosis.com)
Cystic neoplasms3
- Pancreatic cystic neoplasms represent a small yet increasingly detected entity of pancreatic abnormalities. (medscape.com)
- Approximately 70% of pancreatic cystic neoplasms are discovered incidentally. (medscape.com)
- 1 2 3 Lack of a solid component and complete suppression on FLAIR differentiates them from cystic neoplasms. (hkmj.org)
Cortex2
- It sends fibers to deep cerebellar nuclei that, in turn, project to both the cerebral cortex and the brain stem, thus providing modulation of descending motor systems. (wikipedia.org)
- Pathologically the cerebellar cortex and subcortical nuclei demonstrate diffuse degenerative changes. (childrensmercy.org)
Midline3
- This axial T2 magnetic resonance image shows a cerebellar vermian midline mass with contrast enhancement and obstruction of the fourth ventricle. (medscape.com)
- Aberrant constitutive activation of BRAF tends to be seen in cerebellar and midline pilocytic astrocyomas whereas the activating point mutation at BRAF V600E is more likely to be seen in cerebral examples. (blogspot.com)
- Brain magnetic resonance imaging (MRI) showed an intra-axial left hemisphere cerebellar lesion causing midline shift tonsilar herniation. (surgicalneurologyint.com)
Fourth ventricle1
- Medulloblastomas appear as hyperdense, noncalcified lesions of the fourth ventricle or cerebellar hemisphere on computed tomography (CT) scans. (medscape.com)
Cysts1
- The most frequently encountered neoplastic pancreatic cysts include intraductal papillary mucinous neoplasm (IPMN), serous cystadenoma (SCA), mucinous cystic neoplasm with ovarian stroma (MCN), and solid pseudopapillary epithelial neoplasm. (medscape.com)
Mucinous1
- Note the smooth external contour typical of a mucinous cystic neoplasm. (medscape.com)
Brainstem1
- The neoplasm can also invade adjacent brainstem structures, including the cardiorespiratory centers of the fourth ventricular floor. (medscape.com)
Lesions2
Computed Tomography1
- Computed tomography, magnetic resonance imaging and angiogram showed an expansile bone neoplasm involving the left petrous temporal bone. (johnshopkins.edu)
Lesion1
- Brain magnetic resonance imaging (MRI) showed an intra-axial left hemisphere cerebellar lesion. (surgicalneurologyint.com)
Immunohistochemistry1
- My feeling is that if you have a histomorphologically classic pure astrocytic neoplasm, there is no need for p53 immunohistochemistry. (blogspot.com)
Commonly2
- Despite their immune diversity, the majority of IMCAs is commonly associated with auto-antibodies against cerebellar autoantigens. (springer.com)
- What neoplasm is commonly associated with this inherited disorder? (cap.org)
Intracranial1
- Neurofibromatosis 1 is an autosomally dominated inherited genetic condition that predisposes those involved to the development of intracranial neoplasms. (medlink.com)
Postoperative1
- Conclusion: The postoperative course was uneventful, and the imaging studies demonstrated total removal of the neoplasm. (johnshopkins.edu)
Cerebral1
- They occur in characteristic locations alongside penetrating vessels in the mesencephalothalamic area, cerebral white matter, and cerebellar dentate nuclei. (hkmj.org)
Pilocytic1
- Pilocytic astrocytomas are slowly growing neoplasms of children and young adults which usually arise around the third and fourth ventricles. (uab.edu)
Vestibular1
- Difficulties with balance or gait suggest cerebellar or vestibular involvement. (vin.com)
Older children and adults1
- In older children and adults they may occur laterally in the cerebellar hemisphere. (uab.edu)
Imaging1
- Imaging demonstrated minimal enhancement and hemorrhage of a cerebellar mass. (openneuroimagingjournal.com)
Bone3
- We present a rare case of an ABC in the temporal bone with significant cerebellar compression. (johnshopkins.edu)
- This diagnosis should be considered in the differential of bone neoplasms in this region. (johnshopkins.edu)
- Overview of Myeloproliferative Neoplasms Myeloproliferative neoplasms are clonal proliferations of bone marrow stem cells, which can manifest as an increased number of platelets, red blood cells (RBCs), or white blood cells (WBCs). (msdmanuals.com)
Mutation1
- Nodular follicular thyroid neoplasm with a somatic NRAS mutation. (ocu-radiology.jp)