Posterior fossa hemangioblastomas

Constans, Jean-Paul; Meder, François; Maiuri, Francesco; Donzelli, R; Spaziante, Renato; Divitiis, Enrico de

doi:10.1016/0090-3019(86)90238-7

Cited by 72 publications

(27 citation statements)

References 22 publications

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“…Previous studies report recurrence rates of 20 to 33% with sporadic hemangioblastomas and in patients with VHL disease after CNS hemangioblastoma resection. 10,11,17,24,61 Factors that have been directly correlated with an increased risk of recurrence include younger age (Ͻ 30 years) at diagnosis and association with VHL disease. 17 Serial MR imaging studies of the region of cerebellar hemangioblastoma resection were performed in all patients and confirmed the absence of residual tumor or recurrence at last imaging follow-up.…”

Section: Current Studymentioning

confidence: 99%

Surgical management of cerebellar hemangioblastomas in patients with von Hippel–Lindau disease

Jagannathan

Lonser

Smith

et al. 2008

JNS

131

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Object Despite the frequency of cerebellar hemangioblastomas in von Hippel–Lindau (VHL) disease, their optimum contemporary management has not been defined, and is made complex because of the multiple, progressive, and protean nature of the tumors found in patients with this disorder. To examine modern management and outcomes of cerebellar hemangioblastomas in VHL disease, the authors reviewed findings in patients with this disease who underwent resection of cerebellar hemangioblastomas. Methods Consecutive patients with VHL disease who underwent surgery for cerebellar hemangioblastoma(s) at the National Institutes of Health were included. Eighty consecutive patients (44 female and 36 male patients) underwent 126 operations for removal of 164 cerebellar hemangioblastomas (age at surgery 37.8 ± 10.3 years, follow-up duration 96.0 ± 60.3 months). Serial clinical examinations, imaging studies, and operative records were analyzed. Results Symptoms and signs included headache (94 operations; 75%), ataxia (55%), dysmetria (29%), and hydrocephalus (28%). Although the primary objective of surgery was resection of the hemangioblastoma considered responsible for symptoms (136 of the hemangioblastomas [83%]), 28 additional hemangioblastomas (17%) were removed during the same surgeries. Tumors associated with symptoms were larger (diameter 1.8 ± 1.9 cm; volume 2.8 ± 3.4 cm3; p < 0.05) and more likely to be associated with peritumoral edema or peritumoral cysts (100% associated with edema and/or cyst; p < 0.05) than asymptomatic tumors (diameter 1.1 ± 0.9 cm; volume 0.7 ± 0.4 cm3; 18%). More tumors were located in the posterior (74%) compared with the anterior (26%) half of the cerebellum (p < 0.05). Three months after resection, symptom improvement/stabilization had occurred following 124 of the operations (98%). Preoperative hydrocephalus resolved after tumor removal in 33 cases (94%) and did not require cerebrospinal fluid diversion. Long-term imaging follow-up (61.5 ± 15.0 months) revealed no recurrences. Conclusions Symptoms and signs caused by cerebellar hemangioblastomas in VHL disease are associated with edema and peritumoral cyst formation/propagation and are treated safely and effectively with resection. Cerebrospinal fluid diversion is rarely necessary after complete tumor removal in patients with preoperative hydrocephalus. Cerebellar hemangioblastomas are preferentially distributed in the posterior half of the cerebellum, as they are in the brainstem and spinal cord. Tumor recurrence is avoided by meticulous extracapsular resection.

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Section: Current Studymentioning

confidence: 99%

Surgical management of cerebellar hemangioblastomas in patients with von Hippel–Lindau disease

Jagannathan

Lonser

Smith

et al. 2008

JNS

131

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“…The incidence of hearing disturbance in patients with cerebellar hemangioblastoma is estimated to be less than 5%. 2,7) In our patient, the cranial nerve VIII was compressed by the tumor in the CP cistern, and hearing acuity recovered dramatically after total removal of the tumor. These observations clearly indicated that our patient's symptoms were caused by the cerebellar hemangioblastoma protruding into the CP cistern.…”

Section: Discussionmentioning

confidence: 59%

“…1,4,5) Hemangioblastomas most commonly arise from the cerebellum, followed by the spinal cord and brainstem, 1) and usually manifest as headache or nausea, associated with elevated intracranial pressure (ICP) or cerebellar ataxia. 2,7) Hearing disturbance is a common symptom of acoustic tumors or meningiomas expanding in the cerebellopontine (CP) cistern, but is a rare clinical manifestation of hemangioblastomas, especially as an initial symptom. We report a rare case of cerebellar hemangioblastoma manifesting as only hearing disturbance.…”

Section: Introductionmentioning

confidence: 99%

Cerebellar Hemangioblastoma Manifesting as Hearing Disturbance -Case Report-

Amano

Tokunaga

Shono

et al. 2009

Neurol. Med. Chir.(Tokyo)

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A 49-year-old man presented with a rare case of cerebellar hemangioblastoma manifesting as only hearing disturbance. He had suffered from hearing difficulty in the right ear for a few months. Magnetic resonance imaging revealed a cystic mass lesion with an internal fluid level and surrounding flow voids in the right cerebellopontine (CP) angle. Cerebral angiography disclosed a vascular-rich tumor fed by both the superior cerebellar and anterior inferior cerebellar arteries. En bloc resection of the tumor was planned under a preoperative diagnosis of cerebellar hemangioblastoma. The tumor protruded into the CP cistern and compressed cranial nerve VIII. The feeding arteries were meticulously coagulated and the tumor was successfully removed. The histological diagnosis was hemangioblastoma. After the operation, the patient's hearing acuity improved dramatically. Cerebellar hemangioblastoma should be considered in the differential diagnosis of CP angle tumors associated with hearing disturbance.

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“…The posterior fossa is a fairly common location for this tumor, but hiccuping is an extremely rare associated symptom. 7 Presumably, in this case, bleeding from the tumor had irritated the solitary tract nucleus and stimulated the so-called hiccup center within the medulla oblongata, leading to a functional imbalance in the vagally mediated hiccup neural reflex arc. Both the protean manifestation of hemangioblastoma and the value of MRI in highlighting the central cause of intractable hiccups are well illustrated in this case.…”

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confidence: 90%

Resolution of intractable hiccups caused by cerebellar hemangioblastoma

Seyama

Kurita²,

Noguchi³

et al. 2001

Neurology

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A 38-year-old otherwise healthy man presented with hiccups that had persisted for 8 weeks. The bouts were nearly continuous and severely distressing, depriving the patient of nutrition and sleep. On admission, he was afebrile with a blood pressure of 116/76 mm Hg and had no neurologic deficits. He had no headache, vertigo, or vomiting. Physical and ocular examination revealed no abnormalities, and the results of routine laboratory tests were normal. He had no history of familial disorders. Several physical maneuvers including breath holding, sneezing, and palatal stimulation did not relieve the symptom. Pharmacologic treatment with metoclopramide and diazepam was also ineffective.

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Posterior fossa hemangioblastomas

Cited by 72 publications

References 22 publications

Surgical management of cerebellar hemangioblastomas in patients with von Hippel–Lindau disease

Surgical management of cerebellar hemangioblastomas in patients with von Hippel–Lindau disease

Cerebellar Hemangioblastoma Manifesting as Hearing Disturbance -Case Report-

Resolution of intractable hiccups caused by cerebellar hemangioblastoma

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