Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease

Conway, James E.; Chou, Dean; Clatterbuck, Richard E.; Brem, Henry; Long, Donlin M.; Rigamonti, Daniele

doi:10.1097/00006123-200101000-00009

Cited by 160 publications

(189 citation statements)

References 23 publications

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“…Regrowth and recurrence have been reported to be similar in patients with sporadic and VHL-related HBs [4]. However, a recent study reported that despite having their similar histologic characteristics, VHL-related HBs may show more aggressive development, including more rapid growth and the development of multifocal lesions, and may have a strong negative effect on long-term patient outcomes [6,35].…”

Section: Discussionmentioning

confidence: 99%

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Hemangioblastomas with leptomeningeal dissemination: case series and review of the literature

et al. 2016

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Section: Discussionmentioning

confidence: 99%

“…About one-fourth of HBs are sporadic. In general, sporadic HBs are solitary and curable by total resection [6], but have a recurrence rate of 13-20 % [10, 20, 25]. Most recurrences after gross total resection are at the original tumor site [10,20].…”

Section: Introductionmentioning

confidence: 99%

Hemangioblastomas with leptomeningeal dissemination: case series and review of the literature

et al. 2016

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“…In that case, of course, the cystic-space-occupying lesion would recur because of the presence of residual tumor tissue, and the clinical symptoms would reappear. 2 Color Doppler flow imaging provides valuable help in identifying arteriovenous malformation lesions and their boundaries.Although the lesion and the normal tissue could barely be differentiated using conventional two-dimensional grayscale images in the present study, color Doppler flow imaging proved helpful in detecting rich blood-flow signals in arteriovenous malformation lesions (Fig. 6).…”

Section: Casesmentioning

confidence: 99%

“…1 Today intraoperative ultrasound guidance is routinely used during neurosurgery. 2 It provides considerable benefits in terms of reducing the time required to explore lesions and preventing unnecessary tissue injury to normal brain tissues. In the present study we evaluated the usefulness of intraoperative ultrasonography in neurosurgery.…”

Section: Introductionmentioning

confidence: 99%

The value of intraoperative ultrasonography in neurosurgery

Wang

et al. 2006

J Med Ultrasonics

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Intraoperative ultrasonography is widely used in neurosurgery today. Here we attempt to clarify and demonstrate its usefulness in the operating room. Of 113 lesions, 112 were detected during surgery. The undetected lesion was a spinal arachnoid hematoma that had been absorbed during an intraoperative maneuver. No infection or complication occurred after surgery. Ultrasound guidance ensures safe tumor removal by minimizing injury to cerebral tissue and shortening operation time. Intraoperative ultrasonography improves the safety and efficiency of neurosurgery.

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Pregnancy associated quadriparesis in a patient with Von-Hippel Lindau disease

Kurne¹,

Bakar

Arsava

et al. 2003

Journal of Neurology

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Sirs: Von-Hippel Lindau (VHL) disease is a dominantly inherited disease characterized by hemangioblastomas of the central nervous system, retina and viscera and is also associated with cysts and tumors of the viscera [1,4,7]. The course of the disease during pregnancy is not well established, but reports in the literature favor a benign course during that period. This case report presents a patient with VHL and recurrent deterioration in the neurological status during her pregnancies.A 34-year-old woman, pregnant for the third time, was admitted to our medical center at 33 weeks gestation with complaints of progressive quadriparesis, urinary and fecal incontinence. Her initial complaints had started 12 years previously, during her second pregnancy, with severe pain radiating to upper and lower extremities followed by paresthesia and weakness in the right leg. Her symptoms were partially relieved during the post-partum period. Two years after the onset of initial complaints, she experienced a sudden onset of weakness progressing to total quadriplegia, within 24 hours. Computed tomography, performed in another medical center, revealed a hemorrhagic cervical mass lesion. She was operated for this lesion, but the nature of the mass was not established by pathological examination. In the post-operative period, following neuro-rehabilitation, she was ambulatory with aid and had partial motor deficit in her upper extremities.During the first trimester of her third pregnancy, progression in lower extremity weakness and severe numbness below the nipple level involving the whole body occurred. She was totally bed-ridden and complained of fecal and urinary incontinence at the end of second trimester. Upper extremity weakness also progressed during the third trimester. No report of consanguinity or family history of similar disease was present.In her initial neurological evaluation, retinal hemangioma in the left eye, bilateral horizontal nystagmus, paraplegia, asymmetric paresis of the upper extremities, anesthesia below T10 level, increased deep tendon reflexes and bilateral extensor plantar responses were detected. Routine laboratory examination including complete blood count, renal and liver function tests, serum electrolytes, electrocardiography and chest radiographs were within normal limits. In her cranial and spinal MRI, multiple lesions consistent with hemangioblastomas were detected in the cerebellum, and at upper cervical and lower thoracic levels (Figs. 1 and 2). As these findings were suggestive of VHL, the patient was evaluated further for other systemic involvement. By abdominal ultrasonograpy, bilateral renal multiple cysts and hepatic hemangioma were detected. Multiple pancreatic cysts were also seen in abdominal computed tomography examination. No adrenal pathology was evident and urine measurement of catecholamines and their metabolites were within normal limits. The patient gave birth to a male fetus by vaginal delivery with an Apgar score of 0, one week after hospitalization. No abnormality was found in ...

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Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease

Cited by 160 publications

References 23 publications

Hemangioblastomas with leptomeningeal dissemination: case series and review of the literature

Hemangioblastomas with leptomeningeal dissemination: case series and review of the literature

The value of intraoperative ultrasonography in neurosurgery

Pregnancy associated quadriparesis in a patient with Von-Hippel Lindau disease

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