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A Throwback on Hemangioblastomas with a Special Reference to Von Hippel-Lindau Syndrome A Rarity

Rama K , Nivetha S1 , Rama K2 , Devanand Senthilkumar3 , Bharanidharan M4

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Indian Journal of Pathology: Research and Practice 9(1(Part 2)):p 171-176, January – April 2020. | DOI: http://dx.doi.org/10.21088/ijprp.2278.148X.9120.26

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Nivetha S, Rama K, Devanand Senthilkumar, et al. A Throwback on Hemangioblastomas with a Special Reference to Von HippelLindau Syndrome A Rarity. Indian J Pathol Res Pract. 2020;9(1 Part II):171–176.
 

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Received : N/A         Accepted : N/A          Published : N/A

Abstract

 

Background: Hemangioblastomas are benign vascular tumors with
large majority of them arising within the cerebellum and produce the
neurologic manifestations. They can appear either sporadically or due
to an association with von Hippel-Lindau (VHL) disease. This study
was done with the aim of knowing the current spectrum of incidence,
varied locations, age and gender distribution in hemangioblastomas
and their association with von-Hippel-Lindau (VHL) disease. Methods:
A 5 year retrospective study was conducted in the Department
of Neuropathology, Institute of Neurosurgery from January 2014
to December 2018 and histopathologically confirmed cases of
Hemangioblastomas were compiled. Results: A total of 20 cases of
hemangioblastomas were identified during the study, all of which were
histopathologically confirmed. Among them 18 were intracranial and 2
were spinal hemangioblastoma. The most common age group affected
in sporadic cases were 41–50 years (7 cases) and in VHL associated
case was 11–20 years (1 case). Cerebellar location was the commonest
seen in 13 cases (65%), followed by medullary, cerebellopontine angle,
parieto occipital and cervical spine in decreasing order of frequency.
Male predominance was seen unlike previous studies. Conclusion:
The current spectrum of cases of hemangioblastomas with respect to
incidence, various locations encountered, age and gender distribution
were seen. The nature of presentation of paediatric hemangioblastomas
is different, genetic heterogenicity and association with VHL has to
be ruled out. Histological subtyping of the hemangioblastomas is
necessary, after ruling out the differentials. Proper follow-up of the
cases of hemangioblastomas are needed to look for recurrence.
Keywords: Hemangioblastomas; Cerebellar location; Von
Hippel-Lindau disease.

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Cite this article

 

Nivetha S, Rama K, Devanand Senthilkumar, et al. A Throwback on Hemangioblastomas with a Special Reference to Von HippelLindau Syndrome A Rarity. Indian J Pathol Res Pract. 2020;9(1 Part II):171–176.
 

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DOI: http://dx.doi.org/10.21088/ijprp.2278.148X.9120.26

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