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 left side body for the past 6 months, and seizure since 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,
we advised her 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 exten
ding to the suprasellar cistern, left parasellar area, and the
leptomeninges around the whole spine. She under-
went surgical resection for the markedly enlarged left medial
temporal tumor.
Only two dura-based3,5 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 sphinter control. Medical
and family histories were unremarkable, neurological deficits were
absent, and laboratory findings were normal.
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.
Till date, approximately 139 cases of supratentorial HBLs have been
described in the literature.
Gamma Knife radiosurgery (GKRS), 15% tumors were stable
in volume, 54% decreased, and 31% increased. Local tumor
control rates at 1, 5, and 10 years was 89%, 74%, and 50%,
respectively. There was a trend toward tumor progression in
sporadic patients (P = 0.10), women (P = 0.09), and larger
tumors(P = 0.10). In patients with multiple HBLs as compared
to those with only a solitary HBL, the radiosurgically treated
lesion was 7.9 times more likely to progress after GKRS
treatment (P = 0.018). They concluded that stereotactic
radiosurgery offers a reasonable rate of tumor control and
preservation of neurologic function in patients with HBLs, but
patients with multiple HBLs are less likely to exhibit long-term
tumor control of treated lesions following radiosurgery.
However, which treatment modality is most definitive in
supratentorial HBL is not yet known because of the rarity of
this tumor and available literature. MRI is recommended as
regular follow-up for sporadic supratentorial HBL. Complete
resection of hemangioblastoma is curative and is associated
with minimum morbidity and 2% mortality.
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, we 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 radio-
surgery 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.
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 sudden onset of neurological
symptoms demanding neurosurgical intervention.
The exact mechanism of hemangioblastomatosis is unknown.
There are fourteen published cases on hemangioblastomatosis
and 11 cases had no relationship with VHL disease. The disse-
mination of HB in all reported cases occurred after surgical
resection. Accordingly, the spillage and spread of tumor cells
through the cerebrospinal fluid(CSF) space are the mainstreams
that explain the dissemination of HB4)
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.3,6) Multiple hemangioblastomas are rarer
in supratentorial (3%) than in infratentorial regions
(11%).7) Previous cases include a case of supraten-
torial multiple hemangioblastomas, two dural cases
identified by MR imaging, and three cases identified
at operation.The origin of hemangioblastomas is uncertain, but the presence of endothelial cells, pericytes, and stromal cells with hypervascular structures is thought to indicate derivation
from the dural vascular structures as in our case.
Dural involvement can be seen in posterior fossa
hemangioblastomas, especially in recurrent cases.
Supratentorial hemangioblastomas usually become symptomatic in the third and fourth decades of life with male predominance. Supratentorial hemangioblastomas generally manifest as symptoms
of intracranial hypertension, epilepsy, especially in meningeal localizations, hemiparesis, hydrocephalus, and intracerebral and subarachnoid hemorrhage.
The outcomes after dissemination were very poor and most patients died within 3 years of diagnosis. The most common cause of death was respiratory failure due to pontomedullary or cervical cord compression.
If tumor cells dislodge from the primary site in the fourth ventricle, the majority of them will descend along the CSF flow to the spinal subarachnoid space and then ascend to the supratentorial subarachnoid space.
Hemangioblastomas have been reported at various locations in the central nervous system. Supratentorial
hemangioblastomas are extremely rare. Between 1902 and
2013, approximately 132 cases of supratentorial hemangioblastomas were reported. Approximately seven cases of
isolated dural-based tumors were reported before
2007.
Ref:
Supratentorial haemangioblastoma without von
Hippel–Lindau syndrome in an adult: A rare tumor
with review of literature
Sharad Pandey, Vivek Sharma, Deepa Pandey, Vikul Kumar, Mohan Kumar2
Departments of Neurosurgery and 2
Pathology, Sir Sunderlal Hospital, IMS, BHU, 1
Department of Clinical Microbiology, Central
Hospital, DLW, Varanasi, Uttar Pradesh, India
A Very Aggressive Hemangioblastomatosis Without VHL Gene Mutation
Jeong Goo Park, Sang Woo Song, Young-Cho Koh
Department of Neurosurgery, Konkuk University College of Medicine, Seoul, Korea
Hemangioblastoma of the central nervous system may arise as sporadic lesions or as a manifestation of von Hippel-Lindau
(VHL) disease. Hemangioblastomatosis, a disseminated form of hemangioblastoma, is an extremely unusual type of the
disease which had been reported mainly in non-VHL disease. It is known to exhibit similar clinical features. Most patients
underwent surgical resection of the primary lesion, and hemangioblastomatosis developed after variable intervals. Because
there is no specific treatment, it has been reported very poor prognosis. A 55-year-old woman initially diagnosed as sporadic
hemangioblastoma because satellite left temporal mass was considered as meningioma and evaluation for VHL disease
including VHL gene study was negative. 3 years later, she diagnosed as disseminated hemangioblastomatosis and resected
left temporal mass was diagnosed as hemangioblastoma, which was compatible with VHL disease. We report a rare case
of hemangioblastomatosis in a VHL gene negative woman with some beneficial effect of bevacizumab.
Neurol Med Chir (Tokyo) 46, 294¿297, 2006
Parasagittal Leptomeningeal Hemangioblastoma
—Case Report—
Murat COSAR, Mustafa Aziz HATIBOGLU*, A. Celal IPLIKCIOGLU*,
and Deniz OZCAN**
Department of Neurosurgery, Afyon Kocatepe University, Afyon, Turkey;
Departments of *Neurosurgery and **Pathology, Okmeydani Training Hospital,
Istanbul, Turkey
CASE REPORT
Supratentorial leptomeningeal
hemangioblastoma resection after
preoperative embolization
Ching-Yi Lee a
, Shiu-Jau Chen a,b,
*
a Department of Neurosurgery, Mackay Memorial Hospital, Taipei, Taiwan
b Department of Medicine, Mackay Medicine College, New Taipei City, Taiwan
Received 12 August 2015; received in revised form 12 October 2015; accepted 10 November 2015
Leptomeningeal hemangioblastoma
Case illustration
CLAUDIO AGOSTINELLI, M.D., FEDERICO RONCAROLI, M.D., ERCOLE GALASSI, M.D., BRUNO BERNARDI, M.D.,
NICOLA ACCIARRI, M.D., AND GIOVANNI TANI, M.D.
Departments of Oncology, Section of Anatomic Pathology, and Neuroscience, Sections of Neurosurgery and Neuroradiology, Bellaria
Hospital; Department of Neuropathology, Division of Neuroscience and Psychological Medicine, Imperial College, Faculty of Medicine,
London, United Kingdom; and Institute of Radiology, St. Orsola Hospital, Bologna Italy
Case Report
A report of supratentorial leptomeningeal
hemangioblastoma and a literature review
Hiroaki Takeuchi, Norichika Hashimoto, Ryuhei Kitai and Toshihiko Kubota
Department of Neurosurgery, Faculty of Medical Sciences, University of Fukui, Fukui, Japan
