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
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Hypothesis
Medulloblastoma6
- These discoveries challenge many current mouse-centric models of normal human cerebellar development and models regarding the pathogenesis of several neurodevelopmental phenotypes affecting the cerebellum, including Dandy-Walker malformation and medulloblastoma. (nih.gov)
- 1. [A clinical and pathological study of cerebellar medulloblastoma in adult patients]. (nih.gov)
- 16. MATH-1 production by an adult medulloblastoma suggestive of a cerebellar external granule cell precursor origin. (nih.gov)
- Background: The cyclin-dependent kinase inhibitor p27 Kip1 functions during normal cerebellar development and has demonstrated tumor suppressor functions in mouse models of medulloblastoma. (prinsesmaximacentrum.nl)
- Radiation Induced Multiple Skin Neoplasms Following Craniospinal Irradiation for Medulloblastoma. (amjcaserep.com)
- Following craniospinal irradiation in children with medulloblastoma, secondary neoplasms are among the most serious long-term sequelae that include leukemias and solid tumors of the urinary or digestive tracts, thyroid, skin, and central nervous system. (amjcaserep.com)
Tumor3
- The term Yo proteins refers to a family of proteins highly expressed in the cytoplasm of cerebellar Purkinje cells and in the tumor cells (usually gynecologic or breast) of patients with anti-Yo-positive paraneoplastic cerebellar degeneration. (medscape.com)
- Analysis of cerebellar tumor incidence in compound mutant mice carrying the activated Smoothened (SmoA1) allele that were heterozygous or nullizygous for p27 Kip1 revealed that p27 Kip1 loss did not alter the frequency of tumor initiation. (prinsesmaximacentrum.nl)
- 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)
Associated with paraneoplastic cerebellar d2
- Neoplasms associated with paraneoplastic cerebellar degeneration (PCD) are adult onset and more prevalent in females. (medscape.com)
- Serum antibodies to the Yo antigen are usually associated with paraneoplastic cerebellar degeneration arising in female patients with gynecological or breast malignancy and are rarely associated with other tumors. (ox.ac.uk)
Malignant2
- A familial cancer predisposition syndrome associated with a variety of malignant and benign neoplasms, most frequently retinal, cerebellar, and spinal hemangioblastoma, renal cell carcinoma (RCC), and pheochromocytoma/paraganglioma. (orpha.net)
- Neoplasms: benign tumors and malignant neoplasia of the brain. (symptomsus.com)
Retinal1
- I risk of developing future urothelial neoplasms or squamous cell carcinoma, cerebellar haemangioblastoma, retinal angioma, renal and liver, toxicity, which impairs quality of life is one parameter the mean. (familytreecounseling.com)
Tumors1
- Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA . (nih.gov)
Pathology2
- Depending on the nature of the underlying pathology, bradykinesia and other signs may be accompanied by cerebellar ataxia, dementia praecox, pyramidal syndrome, and other neurological symptoms. (symptomsus.com)
- CT of the chest revealed a 3.4-cm anterior mediastinal soft tissue mass, with biopsy and pathology results consistent with seminoma ( figure, G-H ). There was no evidence of testicular neoplasm on ultrasound. (neurology.org)
Ataxia5
- Autoantibody screening in subacute cerebellar ataxia. (ox.ac.uk)
- Cardinal manifestations of cerebellar dysfunction include dysmetria, GAIT ATAXIA, and MUSCLE HYPOTONIA. (rush.edu)
- In 40% of patients, cerebellar ataxia is found, in 2 - corticobulbar disorders. (symptomsus.com)
- The main manifestation of olivopontocerebellar degeneration is progressive cerebellar ataxia. (symptomsus.com)
- Homozygous null mice develop ataxia, impaired blance, and tremors with age and show altered cerebellar phospholipid composition and anemia. (jax.org)
Obstruction of the fourth ventricle1
- This axial T2 magnetic resonance image shows a cerebellar vermian midline mass with contrast enhancement and obstruction of the fourth ventricle. (medscape.com)
Diseases3
- Cerebellar Diseases" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (rush.edu)
- This graph shows the total number of publications written about "Cerebellar Diseases" by people in this website by year, and whether "Cerebellar Diseases" was a major or minor topic of these publications. (rush.edu)
- Below are the most recent publications written about "Cerebellar Diseases" by people in Profiles. (rush.edu)
Cerebral2
- 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)
- Introduction -- Where to start -- Nonneoplastic flags -- Cerebral parenchymal lesions: I. Metastatic neoplasms -- Cerebral parenchymal lesions: II. (edu.au)
Progressive cerebellar1
- Fragile X premutation carriers: characteristic MR imaging findings of adult male patients with progressive cerebellar and cognitive dysfunction. (rush.edu)
Spinal2
- Neurogenic stuttering typically appears following some sort of injury or disease to the central nervous system i.e. the brain and spinal cord, including cortex, subcortex, cerebellar, and even the neural pathway regions. (stutteringhelp.org)
- A) Parasagittal T1 postcontrast images of the cervical spine demonstrate homogenous enhancement of the spinal cord from the craniocervical junction that extends through the midthoracic spine and also several ill-defined mass-like areas of enhancement in both cerebellar hemispheres involving both cortex and white matter. (neurology.org)
Cerebellum2
Posterior fossa2
- Neoplasms of the posterior fossa. (medlineplus.gov)
- Clinical and radiographic predictors of neurological outcome following posterior fossa decompression for spontaneous cerebellar hemorrhage. (rush.edu)
Vermis1
- The majority of medulloblastomas arise from the inferior cerebellar vermis, from which they extend into and typically fill the fourth ventricle. (medscape.com)
Degeneration12
- Paraneoplastic cerebellar degeneration in a woman with ovarian cancer. (medscape.com)
- DIAGNOSIS: Stage IIIC endometrioid adenocarcinoma of the ovary with paraneoplastic cerebellar degeneration. (medscape.com)
- [ 14 ] The development of paraneoplastic cerebellar degeneration is quite rapid and patients are severely disabled in days to weeks. (medscape.com)
- Mild memory and cognitive deficits as well as affective symptoms can occur in about 20% of patients with paraneoplastic cerebellar degeneration. (medscape.com)
- Most patients have occult malignancy (60-70%), so patients are less likely to develop symptoms of paraneoplastic cerebellar degeneration if they have a known history of malignancy. (medscape.com)
- History, examination, and diagnostic testing help to differentiate paraneoplastic cerebellar degeneration from other conditions that are statistically more likely to occur than paraneoplastic cerebellar degeneration. (medscape.com)
- Early diagnosis of paraneoplastic cerebellar degeneration can lead to early diagnosis and treatment of the occult malignancy. (medscape.com)
- The hallmark of paraneoplastic cerebellar degeneration (PCD) is cerebellar dysfunction. (medscape.com)
- These target antigens are also known as cerebellar degeneration related proteins (CDR). (medscape.com)
- Female guinea pigs had intraventricular injections of either IgG from a patient with paraneoplastic cerebellar degeneration (PCD) and anti-Purkinje cell antibodies (anti-Yo IgG) or control IgG. (nih.gov)
- Anti-Yo antibodies and cerebellar degeneration in a man with adenocarcinoma of the esophagus. (ox.ac.uk)
- We report a male patient who presented with paraneoplastic cerebellar degeneration and anti-Yo antibodies following removal of an esophageal adenocarcinoma. (ox.ac.uk)
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)
Syndrome1
- This is known as cerebellar cognitive affective syndrome. (medscape.com)
Granule2
- To define the mechanisms linking proliferation and apoptotic threshold, we examined the effect of conditionally deleting Bcl2l1, the gene that codes the antiapoptotic protein BCL-xL, in cerebellar granule neuron progenitors (CGNPs), and of co-deleting Bcl2l1 homologs, antiapoptotic Mcl-1, or pro-apoptotic Bax. (nih.gov)
- We found that cerebella in conditional Bcl2l1-deleted (Bcl-xLcKO) mice were severely hypoplastic due to the increased apoptosis of CGNPs and their differentiated progeny, the cerebellar granule neurons (CGNs). (nih.gov)
Clinical1
- Clinical or pathologic evidence of cerebellar involvement was not seen in any of the animals. (nih.gov)
Brainstem2
- The neoplasm can also invade adjacent brainstem structures, including the cardiorespiratory centers of the fourth ventricular floor. (medscape.com)
- The indications are neoplasms of the poste- rior cranial fossa and/or the cerebellopontine angle, like VIII cranial nerve schwannomas, meningiomas, epidermoid cysts, and metasta- ses, with or without internal auditory canal involvement with/with- out serviceable hearing, symptomatic neurovascular conflicts, or brainstem implant placement. (unibo.it)
Proteins1
- Antiapoptotic Bcl-2 family proteins BCL-xL and MCL-1 integrate neural progenitor survival and proliferation during postnatal cerebellar neurogenesis. (nih.gov)
Antibodies1
- In a retrospective study of 280 sera from patients presenting with cerebellar signs, seven of whom had proved positive for the typical paraneoplastic serum antibodies that were requested by the clinicians, raised concentrations of antibodies to voltage-gated calcium channels or to glutamic acid decarboxylase were detected in a further seven sera. (ox.ac.uk)
Disorders1
- This group of hereditary pathologies is characterized by cerebellar disorders. (symptomsus.com)
Onset1
- The age of onset of VHL-associated neoplasms for three affected siblings was in the third decade of life and in the fourth decade for the fourth sibling. (nih.gov)
Patients1
- Patients with VHL disease have a propensity to develop neoplasms of several different organ sites. (nih.gov)
Prognosis1
- A cerebellar neoplasm of adults with favorable prognosis. (nih.gov)
Mice3
- We know very little about human cerebellar development, in stark contrast to the wealth of knowledge from decades of research on cerebellar developmental biology of model organisms, especially mice. (nih.gov)
- Recent studies show that multiple aspects of human cerebellar development significantly differ from mice and even rhesus macaques, a nonhuman primate. (nih.gov)
- Despite the overall reduction in cerebellar growth, SHH-dependent proliferation was prolonged in Bcl-xLcKO mice, as more CGNPs remained proliferative in the second postnatal week. (nih.gov)
Involvement1
- Cerebellar involvement in motor and non-motor sequence learning was examined with serial reaction time tasks (SRT). (tau.ac.il)
Lesions1
- Medulloblastomas appear as hyperdense, noncalcified lesions of the fourth ventricle or cerebellar hemisphere on computed tomography (CT) scans. (medscape.com)
Development1
- The development of cerebellar tonsillar herniation may be accompanied by neck pain. (medscape.com)