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Chapter-20 Orbital Retinoblastoma

BOOK TITLE: Retinoblastoma

Author
1. Honavar Santosh G
ISBN
9789350257845
DOI
10.5005/jp/books/11629_20
Edition
1/e
Publishing Year
2012
Pages
14
Author Affiliations
1. Medical Services, CFS Group; Ophthalmic and Facial Plastic Surgery and Ocular Oncology, Center for Sight, Hyderabad, Andhra Pradesh, India, Ocular Oncology Service, Centre for Sight, Hyderabad, Telangana, India, Ocular Oncology Service, Centre for Sight, Hyderabad, Telangana, India, National Retinoblastoma Foundation; Centre for Sight, Hyderabad, Telangana, India, Centre for Sight Superspecialty, Eye Hospital, Hyderabad, Telangana, India, LV Prasad Eye Institute, Hyderabad, Andhra Pradesh, India, Centre for Sight, Hyderabad, Telangana, India, Centre for Sight Superspeciality Eye Hospital, Hyderabad, Andhra Pradesh, India, Super Specialty Hospital Center for Sight, Hyderabad, Andhra Pradesh, India, LV Prasad Eye Institute, Hyderabad, India, Oculoplasty and Ocular Oncology LV Prasad Eye Institute, LV Prasad Marg, Hyderabad, Andhra Pradesh, India, LV Prasad Eye Institute, LV Prasad Marg, Banjara Hills, Hyderabad, Andhra Pradesh, India, LV Prasad Eye Institute, Hyderabad, LV Prasad Eye Institute Hyderabad, Andhra
Chapter keywords

Abstract

Clinically, radiologically or histopathologically detected extension of retinoblastoma outside the confines of the eye is termed as orbital retinoblastoma. Survival of patients with retinoblastoma has dramatically improved in the last few decades, essentially due to early diagnosis and better management protocols. Five-year survival rates of 88%, 91% and 93% have been reported from developed countries such as United Kingdom, Japan, and United States respectively. Orbital retinoblastoma encompasses the spectrum of primary clinical manifestation (primary), orbital recurrence following enucleation (secondary), inadvertent perforation or intraocular surgery in an eye with unsuspected retinoblastoma (accidental), intraoperative discovery of extraocular or optic nerve extension (overt), and full-thickness scleral, extrascleral and optic nerve transection involvement on histopathology (microscopic). Each clinical situation is unique with a gross variation in tumor load. The current preferred management is multimodal with a combination of chemotherapy, surgery, and external beam radiotherapy, with excellent prognosis for patient survival. This chapter provides an update on the current concepts in the management of orbital retinoblastoma.

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