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Reading: An overview of the management of renal malignancies in von Hippel-Lindau syndrome

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An overview of the management of renal malignancies in von Hippel-Lindau syndrome

Authors:

Umesh Jayarajah ,

Faculty of Medicine, Colombo, LK
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Oshan Basnayake,

National Hospital of Sri Lanka, LK
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Vimalakanthan Thanusan,

Teaching Hospital Jaffna, LK
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Balasingam Balagobi,

University of Jaffna, LK
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Madura Ambegoda,

Colombo South Teaching Hospital, LK
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Anuruddha M Abeygunasekera

Colombo South Teaching Hospital, LK
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Abstract

Von Hippel-Lindau [VHL] syndrome is a hereditary
multisystem cancer syndrome characterized by a wide range
of benign and malignant tumours. Renal neoplasms are the
commonest solid organ malignancies associated with VHL
seen in 25–45% of patients. Management of renal tumours is
determined by multiple tumour related and patient-related
factors and decision making is often difficult due to its
multifocal nature and bilateral involvement. Its tendency for
frequent recurrences further complicates the clinical picture.
We review the existing literature on the management of renal
malignancies in VHL syndrome focussing on observational
strategies, minimally invasive approaches, nephron-sparing
surgery and radical surgery. Furthermore, we provide the Sri
Lanka perspective in the management of renal malignancies
in VHL syndrome. Surgeons that manage such patients should
be cognizant of extra-renal manifestations of VHL and the
importance of a multidisciplinary team and referral pathways.
Proper follow-up and selecting patients for suitable as well as
minimal interventions such as minimally invasive techniques
and nephron-sparing approaches is crucial in the management
to provide acceptable cancer control while preserving renal
functions. In Sri Lanka, establishing proper referral pathways
to dedicated centres with a multidisciplinary team equipped
with facilities to deal with renal as well as extra-renal
manifestations would improve expertise, quality of care and
reduce patient discomfort and default rate.

How to Cite: Jayarajah U, Basnayake O, Thanusan V, Balagobi B, Ambegoda M, Abeygunasekera AM. An overview of the management of renal malignancies in von Hippel-Lindau syndrome. Sri Lanka Journal of Surgery. 2021;39(3):65–70.
Published on 30 Nov 2021.
Peer Reviewed

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