Supratentorial leptomeningeal dissemination

Supratentorial hemangioblastomas (HBLs) are rare, benign vascular tumors of the central nervous system neoplasms. 

Very scarce literature is available regarding supratentorial HBL without von Hippel–Lindau (VHL) syndrome in an adult. 

Being a rare entity, not much clinical data is currently available regarding supratentorial HBLs, thus necessitating the need for further reporting and review of such cases.

A 40-year-old male patient presented with a headache, weakness of the left side body for the past 6 months, and seizure for 2 months. 

There was no history of any inherited disease or cancer in his family. He was well orientated to time, place, and person. 

Abnormal physical signs were limited to the CNS. Neurological examination revealed decreased the power of 4a/5 in both left upper and lower limb with no sensory involvement. The tendon reflexes were normal and plantar responses flexor.

A 55-year-old woman presented with severe headache and 

dizziness in 2010 showing a large cerebellomedullary well 

enhancing tumor and a small homogenously well enhancing tumor at the left medial temporal base on MRI . 

She underwent a surgical resection for the large cerebellar 

mass and the pathological diagnosis was HB. She had 

no family history of VHL disease, and the evaluation for VHL disease was negative. Because the radiological features of the left temporal lobe tumor were compatible with meningioma, we initially thought that the patient had a sporadic form of HB. Postoperative course was uneventful but one month after surgery, she complained of neck pain and was investigated with spine MRI. The spine MRI showed multiple enhancing nodules in the cervico-thoracic spine and the patient was included in the criteria for the diagnosis of VHL disease. 

Although her neck pain was relieved soon after the spine MRI, she was advised a regular follow-up for the left temporal tumor and the intraspinal tumors. Unfortunately, she had been lost to follow-up until December 2014, when she complained diplopia, left facial hypoesthesia and truncal ataxia. On the brain and spine MRI, multiple enhancing tumors were observed in the bilateral cerebellopontine angle, prepontine cistern extending to the suprasellar cistern, left parasellar area, and the leptomeninges around the whole spine. She underwent surgical resection for the markedly enlarged left medial temporal tumor.

Supratentorial HBLs, which are quite rare,were first 

described by Bielschowsky in 1902.They are most commonly found in the frontal lobe of the cerebrum followed by parietal and temporal lobe. There is a handful of reported cases of congenital HBLs.

Intracranial hemangioblastomas are rarely located

in the supratentorial region, such as the pituitary

stalk and anterior lobe of the pituitary gland,

hypothalamus, corpus callosum, wall of the third

ventricle, temporal horn of the lateral ventricle, and

meninges.Multiple hemangioblastomas are rarer

in supratentorial (3%) than in infratentorial regions

(11%). Previous cases include a case of supratentorial multiple hemangioblastomas, two dural cases

identified by MR imaging, and three cases identified

at operation

Till date, approximately 139 cases of supratentorial HBLs have been described in the literature.

On reviewing the literature, it was found that 

supratentorial HBL affected patients ranging from 3 months to 80 years with maximum cases belonging to 20–30 years and 40–50 years age group. Among the infants, only one case was found, thus making it a rare tumor in infancy. Most of the cases were found in males. The reported cases either presented as isolated or multiple lesions. On analyzing the tumor consistency, it was found most of them were solid whereas only less than one-third were cystic. In this review, it was found supratentorial HBL in various locations viz., frontal, parietal, temporal, occipital, third ventricle, lateral ventricle, pituitary, pituitary stalk, suprasellar, hippocampus, falx cerebri, corpus callosum, meninges, and choroidal fissure.

The current available treatment did not have a significant 

effect on the progression of the hemangioblastomatosis in 

reported cases. Conventional radiotherapy or stereotactic radiosurgery was often used for disseminated lesions, however,long-term tumor control was not achieved and it is difficult to perform high-dose radiation therapy or radiosurgery for numerous lesions scattered throughout the entire neuraxis. Some studies reported notable achievements in the treatment of hemangioblastomatosis using growth factors antagonist or blocker, such as sunitinib or erlotinib, but long term disease control was not achieved. We used several cycles of bevacizumab and the patient’s neurological condition improved significantly. Long term follow-up was not established and further investigation is needed.

Only two dura-based and two leptomeningeal hemangioblastomas have been documented. The authors describe a 10-year-old girl who presented with warm sensation in her face, blurred bilateral vision, loss of consciousness, and loss of sphincter control. Medical and family histories were unremarkable, neurological deficits were absent, and laboratory findings were normal. 

In HBLs, first and second peaks of incidence are in the third and fifth decades of life respectively. HBLs are more common in males than in females (1.3:1 ratio). They may be either asymptomatic or symptomatic.

Clinical characteristics of supratentorial HBL are specific to 

their location and growth patterns. They are benign lesions 

thus sign, and symptoms manifest late.Patients usually have no history or the long history of minor neurological symptoms. 

In maximum cases, it presents as a sudden onset of neurological symptoms demanding neurosurgical intervention.

In supratentorial HBLs, cyst formation seems to be independent of tumor size.In this, peritumoral cysts which originate from peritumoral edema occur more frequently in regions.