Hemangioblastomen

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Hemangioblastomen zijn goedaardige tumoren van het centrale zenuwstelsel en treden meestal in de kleine hersenen, ruggenmerg of hersenstam op. Vaak worden deze tumoren eerst symptomatisch wanneer ze een begeleidende cyste vormen die expansief op de omgeving drukt. Deze tumoren kunnen sporadisch optreden maar ook in het kader van een familiaal erfelijke ziekte, bijvoorbeeld de von Hippel-Lindau (VHL) ziekte.
Symptomen van tumoren in de kleine hersenen kunnen hoofdpijn, misselijkheid, evenwichtsproblemen en duizeligheid zijn. In extreme gevallen kan een obstructie van de circulatie van het hersenvocht veroorzaakt worden met eventueel bewustzijnsverlies. In deze gevallen moet in urgentie geopereerd worden. Hemangioblastomen van het ruggenmerg zullen pijn, gangstoringen, gevoelsstoring of krachtverlies veroorzaken ensoms ook urine of defecatie problemen..
Hemangioblastomen groeien eerder langzaam. Wanneer ze symptomatisch worden of wanneer een evolutie aangewezen wordt, is de microchirurgische verwijdering geïndiceerd. Het risico van de operatie is klein en tumoren die totaal verwijderd werden gedurende de operatie komen niet terug.
Hemangioblastomen kunnen een component van de ziekte van von Hippel Lindau zijn. Patiëntn met deze erfelijke ziekte hebben multipele tumoren in de ogen (retinale angiomen), in de nieren ( nierkankers), in de bijnieren (pheochromocytomen). Ook eventueel in het binnenoor, de testikels en de pancreas.
Patiënten met de VHL ziekte vereisen een speciale klinische begeleiding met regelmatige screening van alle mogelijk betroffene organen. Ons team heeft een bijzondere jarenlange ervaring met de verzorging van deze patiënten en is lid van de nationale en internationale “Clinical Advisory Board for VHL patiënts”. Individuele raadgeving betreffende de behandeling wordt steeds in een interdisciplinair behandelingsteam besproken.

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Hemangioblastomas are benign tumors of the central nervous system. They occur mostly in the posterior fossa, brain stem and spinal cord. Frequently the tumors produce a major cyst which is frequently responsible for the symptoms by a space-occupying effect. These tumors can occur sporadically or familial as a manifestation of von Hippel-Lindau (VHL) disease.

Symptoms of hemangioblastomas of the cerebellum mainly include headache, nausea, coordination difficulties, gait difficulty and dizziness. In advanced tumors, obstruction of craniospinal fluid circulation may occur, resulting in headache, nausea, vomiting and eventually loss of consciousness. If hemangioblastomas occur in the spinal cord, patients may develop pain, gait imbalance, numbness and paralysis. In addition, disturbances during defecation and urination may occur.

In general, the tumors grow slowly, so that an annual check-up by contrast enhanced MRI is sufficient to intervene in time. A microsurgical operation is the treatment of first choice. Surgical morbidity is low and tumors that have been completely removed do not recur. The effect of radiation therapy is controverse, but it may be an alternative for patients with inoperable tumors. Pharmaceutical therapies are under investigation.

Hemangioblastomas may be a component of VHL disease, a hereditary tumor syndrome. In addition to hemangioblastomas, affected patients develop retinal angiomas, renal carcinomas and adrenalin-producing tumors of the adrenal gland (pheochromocytoma). In addition, tumors in the inner ear, the epididymis and the pancreas can occur.

Patients with von Hippel-Lindau disease frequently develop multiple hemangioblastomas and therefore need a different clinical approach. Our team has special experience with hemangioblastoma patients and we are member national and international Clinical Advisory Boards for patients with this disease. Treatment decisions are discussed in our interdisciplinary treatment team.

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Hemangioblastomas are benign tumors of the central nervous system. They occur mostly in the posterior fossa, brain stem and spinal cord. Frequently the tumors produce a major cyst which is frequently responsible for the symptoms by a space-occupying effect. These tumors can occur sporadically or familial as a manifestation of von Hippel-Lindau (VHL) disease.

Symptoms of hemangioblastomas of the cerebellum mainly include headache, nausea, coordination difficulties, gait difficulty and dizziness. In advanced tumors, obstruction of craniospinal fluid circulation may occur, resulting in headache, nausea, vomiting and eventually loss of consciousness. If hemangioblastomas occur in the spinal cord, patients may develop pain, gait imbalance, numbness and paralysis. In addition, disturbances during defecation and urination may occur.

In general, the tumors grow slowly, so that an annual check-up by contrast enhanced MRI is sufficient to intervene in time. A microsurgical operation is the treatment of first choice. Surgical morbidity is low and tumors that have been completely removed do not recur. The effect of radiation therapy is controverse, but it may be an alternative for patients with inoperable tumors. Pharmaceutical therapies are under investigation.

Hemangioblastomas may be a component of VHL disease, a hereditary tumor syndrome. In addition to hemangioblastomas, affected patients develop retinal angiomas, renal carcinomas and adrenalin-producing tumors of the adrenal gland (pheochromocytoma). In addition, tumors in the inner ear, the epididymis and the pancreas can occur.

Patients with von Hippel-Lindau disease frequently develop multiple hemangioblastomas and therefore need a different clinical approach. Our team has special experience with hemangioblastoma patients and we are member national and international Clinical Advisory Boards for patients with this disease. Treatment decisions are discussed in our interdisciplinary treatment team.

Links:

[:de]

Hemangioblastomas are benign tumors of the central nervous system. They occur mostly in the posterior fossa, brain stem and spinal cord. Frequently the tumors produce a major cyst which is frequently responsible for the symptoms by a space-occupying effect. These tumors can occur sporadically or familial as a manifestation of von Hippel-Lindau (VHL) disease.

Symptoms of hemangioblastomas of the cerebellum mainly include headache, nausea, coordination difficulties, gait difficulty and dizziness. In advanced tumors, obstruction of craniospinal fluid circulation may occur, resulting in headache, nausea, vomiting and eventually loss of consciousness. If hemangioblastomas occur in the spinal cord, patients may develop pain, gait imbalance, numbness and paralysis. In addition, disturbances during defecation and urination may occur.

In general, the tumors grow slowly, so that an annual check-up by contrast enhanced MRI is sufficient to intervene in time. A microsurgical operation is the treatment of first choice. Surgical morbidity is low and tumors that have been completely removed do not recur. The effect of radiation therapy is controverse, but it may be an alternative for patients with inoperable tumors. Pharmaceutical therapies are under investigation.

Hemangioblastomas may be a component of VHL disease, a hereditary tumor syndrome. In addition to hemangioblastomas, affected patients develop retinal angiomas, renal carcinomas and adrenalin-producing tumors of the adrenal gland (pheochromocytoma). In addition, tumors in the inner ear, the epididymis and the pancreas can occur.

Patients with von Hippel-Lindau disease frequently develop multiple hemangioblastomas and therefore need a different clinical approach. Our team has special experience with hemangioblastoma patients and we are member national and international Clinical Advisory Boards for patients with this disease. Treatment decisions are discussed in our interdisciplinary treatment team.

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