Hemangioblastoma
Cerebellar Neoplasms
von Hippel-Lindau Disease
Spinal Cord Neoplasms
Optic Nerve Neoplasms
Cauda Equina
Vestibulocochlear Nerve Diseases
Von Hippel-Lindau Tumor Suppressor Protein
Peripheral Nervous System Neoplasms
Cerebral Ventricle Neoplasms
Ependymoma
Syringomyelia
Meningioma
Neoplasms, Multiple Primary
Spinal Cord Diseases
Magnetic Resonance Imaging
Hemangioblastoma mimicking tentorial meningioma: preoperative embolization of the meningeal arterial blood supply--case report. (1/145)
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)Third International Meeting on von Hippel-Lindau disease. (2/145)
Five years after the identification of the von Hippel-Lindau (VHL) gene, physicians, scientists and concerned VHL family members met to review the current state of knowledge on the diagnosis and treatment of VHL and to summarize the latest information on the biochemistry of the VHL protein (pVHL). The NIH and University of Pennsylvania groups reported the detection of germ-line mutations in 100% (93 of 93) of VHL families studied. Several studies determined the frequency of VHL germ-line mutations in individuals with a single manifestation of VHL without a family history of VHL. National groups to improve the diagnosis and treatment of individuals with VHL disease have been established in Great Britain, Denmark, France, Holland, Italy, Japan, Poland, and the United States. Evidence for the existence of genes that modify the expression of VHL was presented. The VHL protein appears to have several distinct functions: (a) down-regulation of hypoxia-inducible mRNAs; (b) proper assembly of the extracellular fibronectin matrix; (c) regulation of exit from the cell cycle; and (d) regulation of expression of carbonic anhydrases 9 and 12. (+info)Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria. (3/145)
OBJECTIVE: "Cerebellar mutis" and subsequent dysarthria (MSD) is a documented complication of posterior fossa surgery in children. In this prospective study the following risk factors for MSD were assessed: type, size and site of the tumour; hydrocephalus at presentation and after surgery, cerebellar incision site, postoperative infection, and cerebellar swelling. METHODS: In a consecutive series of 42 children with a cerebellar tumour, speech and neuroradiological studies (CT and MRI) were systematically analysed preoperatively and postoperatively. Speech was assessed using the Mayo Clinic lists and the severity of dysarthria using the Michigan rating scale. RESULTS: Twelve children (29%) developed MSD postoperatively. The type of tumour, midline localisation, and vermal incision were significant single independent risk factors. In addition, an interdependency of possible risk factors (tumour>5 cm, medulloblastoma) was found. CONCLUSION: MSD often occurs after paediatric cerebellar tumour removal and is most likely after removal of a medulloblastoma with a maximum lesion diameter>5 cm. (+info)The impact of molecular genetic analysis of the VHL gene in patients with haemangioblastomas of the central nervous system. (4/145)
OBJECTIVES: Haemangioblastoma of the CNS occurs as a sporadic entity and as a manifestation of the autosomal dominant von Hippel-Lindau disease with the major additional components retinal angioma, renal cancer, and pheochromocytoma. Genetic testing for germline mutations predisposing to von Hippel-Lindau disease has been available since identification of the VHL tumour suppressor gene. The impact of this testing was evaluated in patients with haemangioblastomas seen in this centre. METHODS: A register and database of patients with symptomatic haemangioblastomas for the last 15 years was evaluated. The VHL gene was analysed by the SSCP method for all exons and Southern blotting for mutations and deletions of the gene. RESULTS: 141 patients with haemangioblastoma of the CNS were registered. In 81 patients (57%) there was a disease predisposing germline mutation including eight novel mutations. Population related calculation of patients from the administrative district of Freiburg disclosed VHL germline mutations in 22% of the patients with haemangioblastoma. Analysis of mutation carriers for clinical information suggestive of the syndrome showed (1) a positive family history of a brain tumour in 50%, (2) a history for the patient of extracranial manifestations in 36% (retinal angioma 30%, pheochromocytoma 6%), and (3) 19% presenting with multiple brain tumours when first admitted. By genetic testing of haemangioblastoma patients without any indications of von Hippel-Lindau disease mutation carriers were identified in 14%. Sensitivity of VHL germline testing was 86%. CONCLUSIONS: DNA analysis for VHL germline mutations is clearly superior to clinical information in the diagnosis of von Hippel-Lindau disease. Although the percentage of von Hippel-Lindau disease associated haemangioblastoma decreases after the fourth decade of life and is infrequent in patients without other symptomatic lesions and a negative family history, it is recommended that every patient with CNS haemangioblastoma should be screened for von Hippel-Lindau disease germline mutations. This provides the key information and enables screening for extraneurological tumours of the patients and investigations of the patient's family to ameliorate management of von Hippel-Lindau disease. (+info)Spinal cord vascular disease: characterization with fast three-dimensional contrast-enhanced MR angiography. (5/145)
BACKGROUND AND PURPOSE: Noninvasive characterization of spinal vascular lesions is essential for guiding clinical management, and several MR angiographic techniques have been applied in the past with variable results. The purpose of our study was to assess the potential of a dynamic 3D contrast-enhanced MR angiographic sequence to characterize spinal vascular lesions and to identify their arterial feeders and venous drainage. METHODS: A contrast-enhanced gradient-echo 3D pulse sequence providing angiographic information within 24 seconds was applied prospectively in 12 consecutive patients with a presumed spinal vascular lesion. The images were evaluated for visibility of the arterial feeder, and the results were compared with those of conventional angiography performed the next day. RESULTS: The MR angiographic findings proved that the lesions were correctly characterized as spinal arteriovenous malformations (AVMs) (n = 6), spinal dural arteriovenous fistulas (AVFs) (n = 3), a hemangioblastoma (n = 1), a teratoma (n = 1), and a vertebral hemangioma (n = 1). The arterial feeder was visible in all six AVMs and in the hemangioblastoma, corresponding to conventional angiographic findings. In two of three spinal dural AVFs, an enlarged draining medullary vein was seen within the neural foramen, providing correct localization. The third fistula could not be seen owing to reduced image quality from motion artifacts. CONCLUSION: Fast 3D contrast-enhanced MR angiography is a noninvasive technique with high accuracy in the characterization of spinal vascular disease. Visibility of the arterial pedicles corresponds well with that of digital subtraction angiography, facilitating the management of these patients. (+info)Hemangioblastomas with blood supply from the dural arteries--two case reports. (6/145)
Hemangioblastomas are benign vascular tumors that often occur in the cerebellum, and are located near the pia mater. The blood supply is usually received through the pia mater, and rarely through the external carotid artery. The present cases of hemangioblastoma received blood supply from the external carotid artery (occipital artery) and a branch of the internal carotid artery (carotico-tympanic artery or artery of Bernasconi Cassinari) through the dural branches. The dural arteries were not the main feeders in either case, but preoperative embolization of the occipital artery contributed to minimum bleeding during the operation in one case. Incomplete resection of hemangioblastoma is related to multicentricity of the tumors, small mural nodules, or brain stem involvement. Angiography is valuable for demonstrating arterial supply to small or multiple mural nodules. Conventional angiography is necessary for investigation of the external carotid artery branches. (+info)Hemangioblastomas of the retina: impact of von Hippel-Lindau disease. (7/145)
PURPOSE: To assess the prevalence of von Hippel-Lindau (VHL) disease and prognosis of vision in patients with retinal hemangioblastomas (HBs). METHODS: Thirty-six consecutive patients with retinal HBs were treated at Helsinki University Hospital between 1974 and 1998. Detailed neurologic, ophthalmologic, and radiologic examinations; pedigree; mutation analyses; and collection of all relevant clinical, imaging, operative, and autopsy data were performed to identify VHL. RESULTS: The median follow-up time was 10 years. No patient was lost to follow-up. There were three patient groups: 1) 11 patients with clinically definite VHL; 2) 10 patients with clinically suspected VHL with more than one retinal HB (5/10) or visceral cysts (5/10), but with no family history, no detected germ-line mutations, and no VHL-related neoplasms; and 3) 15 patients without VHL with a single retinal HB but no other data suggestive of VHL. In the 11 patients with definite VHL, retinal HBs were detected at a median age of 27 years versus 40 years in the 15 non-VHL patients, and 21 of the 22 eyes were affected. Two VHL patients were totally blind at the end of follow-up compared with one legally blind patient with suspected VHL, but none of the non-VHL patients was blind. The clinical appearance of HBs did not differ among the patient groups. CONCLUSIONS: The prevalence of VHL among patients with retinal HBs was 30% to 58% (11-21 of 36). Visual prognosis was more favorable in non-VHL than VHL patients. All patients with retinal HB should undergo thorough VHL exclusion. (+info)Haemangioblastoma of a cervical sensory nerve root in Von Hippel-Lindau syndrome. (8/145)
Spinal haemangioblastomas are rare, accounting for only about 7% of all central nervous system cases. The case of a 40-year-old woman with a haemangioblastoma arising solely from a cervical sensory nerve root is presented. At operation via a cervical laminectomy, it was possible to resect the tumour en masse with the sensory ramus, by extending the laminectomy through the exit foramen for C6. Haemangioblastomas are commonly intramedullary, and have only been reported in this location on one previous occasion. The patient has Von Hippel-Lindau syndrome and a history of multiple solid tumours. The possible role of the Von Hippel-Lindau tumour suppressor gene in the pathogenesis of these neoplasms is discussed. (+info)Hemangioblastoma is a rare, benign (non-cancerous) tumor that develops from the blood vessels in the central nervous system, most commonly found in the brain and spinal cord. These tumors can be associated with von Hippel-Lindau disease, an inherited disorder that predisposes affected individuals to develop various types of tumors and cysts throughout their bodies. Hemangioblastomas are typically slow-growing but can cause symptoms due to pressure on surrounding tissues or by causing the formation of cysts or fluid-filled sacs near the tumor. Symptoms may include headaches, dizziness, balance problems, weakness, numbness, or vision changes depending on the location and size of the tumor. Treatment options usually involve surgical removal of the tumor, radiation therapy, or observation with regular imaging follow-ups.
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.
Von Hippel-Lindau (VHL) disease is a rare genetic disorder characterized by the development of tumors and cysts in various parts of the body. It is caused by mutations in the VHL gene, which leads to the abnormal growth of blood vessels, resulting in the formation of these tumors.
The tumors associated with VHL disease can develop in several organs, including the eyes (in the form of retinal hemangioblastomas), the brain and spinal cord (in the form of cerebellar hemangioblastomas and spinal cord hemangioblastomas), the adrenal glands (in the form of pheochromocytomas or paragangliomas), the kidneys (in the form of clear cell renal cell carcinomas), and the pancreas (in the form of serous cystadenomas or neuroendocrine tumors).
Individuals with VHL disease are at risk for developing multiple tumors over their lifetime, and the severity of the disease can vary widely from person to person. The diagnosis of VHL disease is typically made through genetic testing, family history, and imaging studies to detect the presence of tumors. Treatment may involve surgical removal of the tumors, radiation therapy, or other interventions depending on the location and size of the tumors. Regular monitoring and follow-up are essential for individuals with VHL disease to manage their condition effectively.
Spinal cord neoplasms refer to abnormal growths or tumors within the spinal cord. These can be benign (non-cancerous) or malignant (cancerous). They originate from the cells within the spinal cord itself (primary tumors), or they may spread to the spinal cord from other parts of the body (metastatic tumors). Spinal cord neoplasms can cause various symptoms depending on their location and size, including back pain, neurological deficits, and even paralysis. Treatment options include surgery, radiation therapy, and chemotherapy.
Optic nerve neoplasms refer to abnormal growths or tumors that develop within or near the optic nerve. These tumors can be benign (non-cancerous) or malignant (cancerous).
Benign optic nerve neoplasms include optic nerve meningiomas and schwannomas, which originate from the sheaths surrounding the optic nerve. They usually grow slowly and may not cause significant vision loss, but they can lead to compression of the optic nerve, resulting in visual field defects or optic disc swelling (papilledema).
Malignant optic nerve neoplasms are rare but more aggressive. The most common type is optic nerve glioma, which arises from the glial cells within the optic nerve. These tumors can quickly damage the optic nerve and cause severe vision loss.
It's important to note that any optic nerve neoplasm requires prompt medical evaluation and treatment, as they can potentially lead to significant visual impairment or even blindness if left untreated.
Retinal neoplasms are abnormal growths or tumors that develop in the retina, which is the light-sensitive tissue located at the back of the eye. These neoplasms can be benign or malignant and can have varying effects on vision depending on their size, location, and type.
Retinal neoplasms can be classified into two main categories: primary and secondary. Primary retinal neoplasms originate from the retina or its surrounding tissues, while secondary retinal neoplasms spread to the retina from other parts of the body.
The most common type of primary retinal neoplasm is a retinoblastoma, which is a malignant tumor that typically affects children under the age of five. Other types of primary retinal neoplasms include capillary hemangioma, cavernous hemangioma, and combined hamartoma of the retina and RPE (retinal pigment epithelium).
Secondary retinal neoplasms are usually metastatic tumors that spread to the eye from other parts of the body, such as the lung, breast, or skin. These tumors can cause vision loss, eye pain, or floaters, and may require treatment with radiation therapy, chemotherapy, or surgery.
It is important to note that retinal neoplasms are relatively rare, and any symptoms or changes in vision should be evaluated by an ophthalmologist as soon as possible to rule out other potential causes and develop an appropriate treatment plan.
The Cauda Equina refers to a bundle of nerves at the lower end of the spinal cord within the vertebral column. It originates from the lumbar (L1-L5) and sacral (S1-S5) regions and looks like a horse's tail, hence the name "Cauda Equina" in Latin. These nerves are responsible for providing motor and sensory innervation to the lower extremities, bladder, bowel, and sexual organs. Any damage or compression to this region can lead to serious neurological deficits, such as bowel and bladder incontinence, sexual dysfunction, and lower limb weakness or paralysis.
Neurosurgical procedures are operations that are performed on the brain, spinal cord, and peripheral nerves. These procedures are typically carried out by neurosurgeons, who are medical doctors with specialized training in the diagnosis and treatment of disorders of the nervous system. Neurosurgical procedures can be used to treat a wide range of conditions, including traumatic injuries, tumors, aneurysms, vascular malformations, infections, degenerative diseases, and congenital abnormalities.
Some common types of neurosurgical procedures include:
* Craniotomy: A procedure in which a bone flap is temporarily removed from the skull to gain access to the brain. This type of procedure may be performed to remove a tumor, repair a blood vessel, or relieve pressure on the brain.
* Spinal fusion: A procedure in which two or more vertebrae in the spine are fused together using bone grafts and metal hardware. This is often done to stabilize the spine and alleviate pain caused by degenerative conditions or spinal deformities.
* Microvascular decompression: A procedure in which a blood vessel that is causing pressure on a nerve is repositioned or removed. This type of procedure is often used to treat trigeminal neuralgia, a condition that causes severe facial pain.
* Deep brain stimulation: A procedure in which electrodes are implanted in specific areas of the brain and connected to a battery-operated device called a neurostimulator. The neurostimulator sends electrical impulses to the brain to help alleviate symptoms of movement disorders such as Parkinson's disease or dystonia.
* Stereotactic radiosurgery: A non-invasive procedure that uses focused beams of radiation to treat tumors, vascular malformations, and other abnormalities in the brain or spine. This type of procedure is often used for patients who are not good candidates for traditional surgery due to age, health status, or location of the lesion.
Neurosurgical procedures can be complex and require a high degree of skill and expertise. Patients considering neurosurgical treatment should consult with a qualified neurosurgeon to discuss their options and determine the best course of action for their individual situation.
The vestibulocochlear nerve, also known as the 8th cranial nerve, is responsible for transmitting sound and balance information from the inner ear to the brain. Vestibulocochlear nerve diseases refer to conditions that affect this nerve and can result in hearing loss, vertigo, and balance problems.
These diseases can be caused by various factors, including genetics, infection, trauma, tumors, or degeneration. Some examples of vestibulocochlear nerve diseases include:
1. Vestibular neuritis: an inner ear infection that causes severe vertigo, nausea, and balance problems.
2. Labyrinthitis: an inner ear infection that affects both the vestibular and cochlear nerves, causing vertigo, hearing loss, and tinnitus.
3. Acoustic neuroma: a benign tumor that grows on the vestibulocochlear nerve, causing hearing loss, tinnitus, and balance problems.
4. Meniere's disease: a inner ear disorder that causes vertigo, hearing loss, tinnitus, and a feeling of fullness in the ear.
5. Ototoxicity: damage to the inner ear caused by certain medications or chemicals that can result in hearing loss and balance problems.
6. Vestibular migraine: a type of migraine that is associated with vertigo, dizziness, and balance problems.
Treatment for vestibulocochlear nerve diseases varies depending on the specific condition and its severity. It may include medication, physical therapy, surgery, or a combination of these approaches.
The Von Hippel-Lindau (VHL) tumor suppressor protein is a crucial component in the regulation of cellular growth and division, specifically through its role in oxygen sensing and the ubiquitination of hypoxia-inducible factors (HIFs). The VHL protein forms part of an E3 ubiquitin ligase complex that targets HIFs for degradation under normoxic conditions. In the absence of functional VHL protein or in hypoxic environments, HIFs accumulate and induce the transcription of genes involved in angiogenesis, cell proliferation, and metabolism.
Mutations in the VHL gene can lead to the development of Von Hippel-Lindau syndrome, a rare inherited disorder characterized by the growth of tumors and cysts in various organs, including the central nervous system, retina, kidneys, adrenal glands, and pancreas. These tumors often arise from the overactivation of HIF-mediated signaling pathways due to the absence or dysfunction of VHL protein.
Cyst fluid refers to the fluid accumulated within a cyst, which is a closed sac-like or capsular structure, typically filled with liquid or semi-solid material. Cysts can develop in various parts of the body for different reasons, and the composition of cyst fluid may vary depending on the type of cyst and its location.
In some cases, cyst fluid might contain proteins, sugars, hormones, or even cells from the surrounding tissue. Infected cysts may have pus-like fluid, while cancerous or precancerous cysts might contain abnormal cells or tumor markers. The analysis of cyst fluid can help medical professionals diagnose and manage various medical conditions, including infections, inflammatory diseases, genetic disorders, and cancers.
It is important to note that the term 'cyst fluid' generally refers to the liquid content within a cyst, but the specific composition and appearance of this fluid may vary significantly depending on the underlying cause and type of cyst.
The cerebellopontine angle (CPA) is a narrow space located at the junction of the brainstem and the cerebellum, where the pons and cerebellum meet. This region is filled with several important nerves, blood vessels, and membranous coverings called meninges. The CPA is a common site for various neurological disorders because it contains critical structures such as:
1. Cerebellum: A part of the brain responsible for coordinating muscle movements, maintaining balance, and fine-tuning motor skills.
2. Pons: A portion of the brainstem that plays a role in several vital functions, including facial movements, taste sensation, sleep regulation, and respiration.
3. Cranial nerves: The CPA is home to the following cranial nerves:
* Vestibulocochlear nerve (CN VIII): This nerve has two components - cochlear and vestibular. The cochlear part is responsible for hearing, while the vestibular part contributes to balance and eye movement.
* Facial nerve (CN VII): This nerve controls facial expressions, taste sensation in the anterior two-thirds of the tongue, salivary gland function, and lacrimation (tear production).
4. Blood vessels: The CPA contains critical blood vessels like the anterior inferior cerebellar artery (AICA), which supplies blood to various parts of the brainstem, cerebellum, and cranial nerves.
5. Meninges: These are protective membranes surrounding the brain and spinal cord. In the CPA, the meninges include the dura mater, arachnoid mater, and pia mater.
Disorders that can affect the structures in the cerebellopontine angle include acoustic neuromas (vestibular schwannomas), meningiomas, epidermoids, and arteriovenous malformations. These conditions may cause symptoms such as hearing loss, tinnitus (ringing in the ears), vertigo (dizziness), facial weakness or numbness, difficulty swallowing, and imbalance.
Microsurgery is a surgical technique that requires the use of an operating microscope and fine instruments to perform precise surgical manipulations. It is commonly used in various fields such as ophthalmology, neurosurgery, orthopedic surgery, and plastic and reconstructive surgery. The magnification provided by the microscope allows surgeons to work on small structures like nerves, blood vessels, and tiny bones. Some of the most common procedures that fall under microsurgery include nerve repair, replantation of amputated parts, and various types of reconstructions such as free tissue transfer for cancer reconstruction or coverage of large wounds.
Peripheral nervous system (PNS) neoplasms refer to tumors that originate in the peripheral nerves, which are the nerves outside the brain and spinal cord. These tumors can be benign or malignant (cancerous). Benign tumors, such as schwannomas and neurofibromas, grow slowly and do not spread to other parts of the body. Malignant tumors, such as malignant peripheral nerve sheath tumors (MPNSTs), can invade nearby tissues and may metastasize (spread) to other organs.
PNS neoplasms can cause various symptoms depending on their location and size. Common symptoms include pain, weakness, numbness, or tingling in the affected area. In some cases, PNS neoplasms may not cause any symptoms until they become quite large. Treatment options for PNS neoplasms depend on several factors, including the type, size, and location of the tumor, as well as the patient's overall health. Treatment options may include surgery, radiation therapy, chemotherapy, or a combination of these approaches.
Cerebral ventricle neoplasms refer to tumors that develop within the cerebral ventricles, which are fluid-filled spaces in the brain. These tumors can arise from various types of cells within the ventricular system, including the ependymal cells that line the ventricles, choroid plexus cells that produce cerebrospinal fluid, or other surrounding tissues.
Cerebral ventricle neoplasms can cause a variety of symptoms depending on their size and location, such as headaches, nausea, vomiting, vision changes, imbalance, weakness, or difficulty with mental tasks. The treatment options for these tumors may include surgical resection, radiation therapy, and chemotherapy, depending on the type and extent of the tumor. Regular follow-up care is essential to monitor for recurrence and manage any long-term effects of treatment.
Ependymoma is a type of brain or spinal cord tumor that develops from the ependymal cells that line the ventricles (fluid-filled spaces) in the brain, or the central canal of the spinal cord. These tumors can be benign or malignant, and they can cause various symptoms depending on their location and size.
Ependymomas are relatively rare, accounting for about 2-3% of all primary brain and central nervous system tumors. They most commonly occur in children and young adults, but they can also affect older individuals. Treatment typically involves surgical removal of the tumor, followed by radiation therapy or chemotherapy, depending on the grade and location of the tumor. The prognosis for ependymomas varies widely, with some patients experiencing long-term survival and others having more aggressive tumors that are difficult to treat.
Syringomyelia is a medical condition characterized by the formation of a fluid-filled cavity or cavities (syrinx) within the spinal cord. This syrinx can lead to various symptoms depending on its size and location, which may include pain, muscle weakness, numbness, and stiffness in the neck, back, shoulders, arms, or legs. In some cases, it may also affect bladder and bowel function, sexual performance, and the ability to maintain normal body temperature. Syringomyelia is often associated with Chiari malformation, a condition where the lower part of the brain extends into the spinal canal. However, other conditions such as spinal cord injuries, tumors, or infections may also cause syringomyelia.
A meningioma is a type of slow-growing tumor that forms on the membranes (meninges) surrounding the brain and spinal cord. It's usually benign, meaning it doesn't spread to other parts of the body, but it can still cause serious problems if it grows and presses on nearby tissues.
Meningiomas most commonly occur in adults, and are more common in women than men. They can cause various symptoms depending on their location and size, including headaches, seizures, vision or hearing problems, memory loss, and changes in personality or behavior. In some cases, they may not cause any symptoms at all and are discovered only during imaging tests for other conditions.
Treatment options for meningiomas include monitoring with regular imaging scans, surgery to remove the tumor, and radiation therapy to shrink or kill the tumor cells. The best treatment approach depends on factors such as the size and location of the tumor, the patient's age and overall health, and their personal preferences.
Multiple primary neoplasms refer to the occurrence of more than one primary malignant tumor in an individual, where each tumor is unrelated to the other and originates from separate cells or organs. This differs from metastatic cancer, where a single malignancy spreads to multiple sites in the body. Multiple primary neoplasms can be synchronous (occurring at the same time) or metachronous (occurring at different times). The risk of developing multiple primary neoplasms increases with age and is associated with certain genetic predispositions, environmental factors, and lifestyle choices such as smoking and alcohol consumption.
Spinal cord diseases refer to a group of conditions that affect the spinal cord, which is a part of the central nervous system responsible for transmitting messages between the brain and the rest of the body. These diseases can cause damage to the spinal cord, leading to various symptoms such as muscle weakness, numbness, pain, bladder and bowel dysfunction, and difficulty with movement and coordination.
Spinal cord diseases can be congenital or acquired, and they can result from a variety of causes, including infections, injuries, tumors, degenerative conditions, autoimmune disorders, and genetic factors. Some examples of spinal cord diseases include multiple sclerosis, spina bifida, spinal cord injury, herniated discs, spinal stenosis, and motor neuron diseases such as amyotrophic lateral sclerosis (ALS).
The treatment for spinal cord diseases varies depending on the underlying cause and severity of the condition. Treatment options may include medication, physical therapy, surgery, and rehabilitation. In some cases, the damage to the spinal cord may be irreversible, leading to permanent disability or paralysis.
Medical Definition:
Magnetic Resonance Imaging (MRI) is a non-invasive diagnostic imaging technique that uses a strong magnetic field and radio waves to create detailed cross-sectional or three-dimensional images of the internal structures of the body. The patient lies within a large, cylindrical magnet, and the scanner detects changes in the direction of the magnetic field caused by protons in the body. These changes are then converted into detailed images that help medical professionals to diagnose and monitor various medical conditions, such as tumors, injuries, or diseases affecting the brain, spinal cord, heart, blood vessels, joints, and other internal organs. MRI does not use radiation like computed tomography (CT) scans.
Hemangioblastoma
Differential diagnoses of anorexia nervosa
Obex
Side effects of cyproterone acetate
Percival Bailey
Phakomatosis
Vascular tumor
Von Hippel-Lindau disease
HIF1A
Endolymphatic sac tumor
Spinal tumor
Neprilysin
Benign tumor
Artery of Adamkiewicz
Astroblastoma
Ganglioglioma
Hemangioblast
Von Hippel-Lindau tumor suppressor
Vertebral hemangioma
List of MeSH codes (C04)
WHO classification of tumours of the central nervous system
Eli Keshet
International Classification of Diseases for Oncology
List of diseases (H)
Index of oncology articles
T-box transcription factor T
Eugen von Hippel
Hereditary cancer syndrome
Dextroscope
Belzutifan
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Hippel-Lindau7
- The genetic hallmark of hemangioblastoma is loss of function of the von Hippel-Lindau (VHL) tumor suppressor protein. (medscape.com)
- Collision tumors are rarely reported in patients with von Hippel-Lindau (VHL) disease, even though VHL patients often present with multi-organ tumor syndromes, like hemangioblastoma and renal cell carcinoma (RCC). (figshare.com)
- Historically, hemangioblastomas are linked to von Hippel-Lindau (VHL) disease. (medscape.com)
- Patients with von Hippel-Lindau (VHL) syndrome with diffuse CNS hemangioblastomas have morbidity related to their disease and require a lifetime of surgical resections. (londonspine.com)
- There was reported history of Von Hippel-Lindau disease (VHL) in his mother, who was operated for a cerebellar hemangioblastoma. (ijhnp.org)
- Tumors called hemangioblastomas are characteristic of von Hippel-Lindau syndrome. (medlineplus.gov)
- Differences in genetic and epigenetic alterations between von Hippel-Lindau disease-related and sporadic hemangioblastomas of the central nervous system. (riken.jp)
Tumors12
- Hemangioblastomas, or haemangioblastomas, are vascular tumors of the central nervous system that originate from the vascular system, usually during middle age. (wikipedia.org)
- Hemangioblastomas usually occur in adults, yet tumors may appear in VHL syndrome at much younger ages. (wikipedia.org)
- According to the World Health Organization classification of tumors of the nervous system, hemangioblastomas are classified as meningeal tumors of uncertain origin. (medscape.com)
- [ 16 ] Cerebellar hemangioblastomas are frequently referred to as Lindau tumors because Swedish pathologist Arvid Vilhelm Lindau first described them in 1926. (medscape.com)
- Hemangioblastomas are considered to be benign neoplasms and represent 1-2% of all primary central tumors. (medscape.com)
- [ 5 ] Of patients presenting with hemangioblastomas, 70% do not have a family history, and 3-25% of these patients have tumors associated with VHL. (medscape.com)
- Hemangioblastoma appears to be associated more with VHL than previously reported, and it has been suggested that all patients with sporadic nonhereditary tumors should be evaluated for evidence of VHL disease. (medscape.com)
- From a macroscopic point of view, hemangioblastomas can appear as solid or cystic tumors with mixed forms. (medscape.com)
- Hemangioblastoma, though rare in the CPA, should be considered in the differential diagnosis of CPA tumors. (surgicalneurologyint.com)
- Hemangioblastomas are well-differentiated, vascular, benign tumors primarily located in the posterior cranial fossa. (surgicalneurologyint.com)
- Stereotactic radiosurgery, such as Gamma Knife, is often used to treat noncancerous (benign) and cancerous (malignant) brain tumors, including meningioma, paraganglioma, hemangioblastoma and craniopharyngioma. (mayoclinic.org)
- Double Strand VHL Cancer Research Foundation is looking to fund an independently functioning research project involving nanotechnology in the biomedical and biotechnical fields to drastically improve the treatment of hemangioblastoma tumors and cancers. (gofundme.com)
Retinal2
- Juxtapapillary retinal capillary hemangioblastoma (JRCH), a benign intraocular vascular tumor, is usually progressive and may lead to severe vision loss due to various complications. (oncotarget.com)
- The clinical manifestations include the development of clear cell RCC, pheochromocytomas, retinal angiomas, and central nervous system hemangioblastomas. (renalandurologynews.com)
Cerebellar hemangioblastoma2
- Micrograph of cerebellar hemangioblastoma. (wikipedia.org)
- In patients with a positive family history, a single cerebellar hemangioblastoma is sufficient to make the diagnosis. (medscape.com)
Benign1
- Christina's tumor - a hemangioblastoma - was benign. (mayoclinichealthsystem.org)
Intracranial2
- Hemangioblastomas are rare, and according to various series, they account for 1-2.5% of all intracranial neoplasms. (medscape.com)
- Hemangioblastoma is rarely located in a supratentorial location, and intracranial lateral ventricular is also not a common site of metastasis for RCC. (figshare.com)
Capillary hemangioblastoma1
- Ezrin expression in stromal cells of capillary hemangioblastoma. (google.co.in)
Posterior fossa3
- On a scan, hemangioblastoma shows as a well-defined, low attenuation region in the posterior fossa with an enhancing nodule on the wall. (wikipedia.org)
- Hemangioblastoma is the most common primary adult intra-axial posterior fossa tumor. (medscape.com)
- Hemangioblastomas are the most common primary tumor of the posterior fossa. (surgicalneurologyint.com)
Abnormality1
- The etiology of hemangioblastoma is obscure, but its presence in various clinical syndromes may suggest an underlying genetic abnormality. (medscape.com)
Occur4
- Hemangioblastomas are most commonly composed of stromal cells in small blood vessels and usually occur in the cerebellum, brainstem or spinal cord. (wikipedia.org)
- Hemangioblastomas can also occur in the light-sensitive tissue that lines the back of the eye (the retina ). (medlineplus.gov)
- Although rare, hemangioblastomas can occur in the spinal cord. (apollohospitals.com)
- 17 ] Very rarely, hemangioblastoma may occur in the cerebellopontine angle (CPA). (surgicalneurologyint.com)
Stromal cells1
- Hemangioblastomas are composed of endothelial cells, pericytes and stromal cells. (wikipedia.org)
Differential diagnosis1
- Hemangioblastoma should be included in the differential diagnosis in patients with an enhancing tumor and adjacent engorged vessels of the spinal cord. (tmu.edu.tw)
Pituitary1
- Pituitary stalk hemangioblastoma: the fourth case report and review of the literature. (saintluc.be)
Diagnosis3
- If no known family history exists, at least 2 cerebellar hemangioblastomas or 1 hemangioblastoma plus 1 visceral tumor are necessary to justify the diagnosis of VHL. (medscape.com)
- Histological findings were compatible with the diagnosis of hemangioblastoma. (tmu.edu.tw)
- Histopathological examination and immunohistochemical workup confirmed the clinical diagnosis of hemangioblastoma. (ijhnp.org)
Excision2
- The treatment for hemangioblastoma is surgical excision of the tumor. (wikipedia.org)
- Complete excision of bilateral cerebellar hemangioblastomas was performed. (ijhnp.org)
Brain2
- On investigations, magnetic resonance imaging brain showed bilateral cerebellar hemangioblastomas and a tiny spinal hemangioblastoma. (ijhnp.org)
- Hemangioblastomas that develop in the brain and spinal cord can cause headaches, vomiting, weakness, and a loss of muscle coordination (ataxia). (medlineplus.gov)
Supratentorial2
- See the image of supratentorial hemangioblastoma, below. (medscape.com)
- Supratentorial hemangioblastoma proved by histologic analysis. (medscape.com)
Rarely1
- Presence of a hemangioblastoma rarely, if ever, alters normal anatomy. (medscape.com)
Metastatic1
- Based on our case report, we systematically reviewed the characteristics of collision tumor composed of hemangioblastoma and metastatic RCC in VHL patients. (figshare.com)
Erythropoietin1
- Hemangioblastomas can cause an abnormally high number of red blood cells in the bloodstream due to ectopic production of the hormone erythropoietin as a paraneoplastic syndrome. (wikipedia.org)
Cystic2
- Of hemangioblastomas, 60-70% are cystic, and the remainder are solid. (medscape.com)
- therefore, 75% of infratentorial hemangioblastomas have a cystic component of variable size (see the image below). (medscape.com)
Lesion1
- The gross appearance of the tumor was suggestive of a highly vascular lesion, and so intraoperatively, a decision was made to attempt an en-bloc resection. (surgicalneurologyint.com)
ABSTRACT1
- abstract = "Hemangioblastoma of the conus medullaris with MRI has not been reported before. (tmu.edu.tw)
Findings1
- When present in this location, hemangioblastoma presents a diagnostic challenge as its imaging findings closely resemble those of vestibular schwannoma (VS), which is much more common in the CPA. (surgicalneurologyint.com)
Surgical1
- This, in addition to significant improvement in surgical approaches and microsurgical technique, has made hemangioblastoma, although dangerous, a potentially treatable and curable disease. (medscape.com)
Rare1
- Hemangioblastomas are uncommon but not rare in patients older than 65 years. (medscape.com)
Patients4
- Asymptomatic lesions that sometimes are encountered in patients with multiple hemangioblastomas may be safely observed with frequent MRI scans to rule out tumor enlargement. (medscape.com)
- For the first time, we have used infratentorial craniospinal radiation therapy (ICSRT) for VHL patients with CNS hemangioblastomas. (londonspine.com)
- Consecutive VHL patients treated at the National Institutes of Health with radiographic evidence of hemangioblastomas were included if they received ICSRT. (londonspine.com)
- Seven patients with 84 hemangioblastomas met eligibility criteria. (londonspine.com)
Treat2
- Gamma Knife Radiosurgery as well as LINAC have also been employed to successfully treat recurrence and control tumor growth of cerebellar hemangioblastomas. (wikipedia.org)
- Doctors informed Anderson that she was suffering from a hemangioblastoma and that they needed to treat it quickly before it was too late. (inquisitr.com)
Description1
- Since their original description, hemangioblastomas have been found in multiple regions of the CNS. (medscape.com)
Cases1
- There are few cases of hemangioblastoma of the cerebellopontine angle (CPA). (surgicalneurologyint.com)
Loss1
- Distinction between these diagnoses is important due to the significant blood loss that may result from internal debulking of hemangioblastoma. (surgicalneurologyint.com)