Past, Present, and Future of Ocular Oncology in India

“Learn the past, watch the present and create the future” — Jess Conrad

Over the last century, Ophthalmology has evolved in unimaginable ways and the last few decades have witnessed a revolution in the world of ocular oncology, the sub-speciality which includes all neoplastic lesions involving the eye, adnexal structures, and the orbit. Ocular oncology has traversed quickly from the zone of unknown to known and we bow to the pioneers who have surfaced ocular oncology all over the world. This sub-speciality encompasses a grand scope of progress along with innumerable challenges faced by the patients as well as the clinicians. Little was known about the several ocular tumors — benign and malignant leading to a major spectrum gone unnoticed amounting to high morbidity and mortality rates, primarily owing to the lack of awareness and management protocols.

Dr. Algernon B. Reese (Fig. 1) was the one to introduce the concept of ocular oncology in the twentieth century [1] and emphasized on the dire need of a separate sub-speciality as it requires a targeted management approach, dedicated patient counseling, regular follow-up visits, and cosmetic rehabilitation. These tumors were deemed untreatable in the earlier days. However, legends in the field of ocular oncology like Dr. Robert M. Ellsworth, Dr. David Abramson, Dr. Francis Munier, Dr. Jerry Shields, and Dr. Carol Shields (Fig. 2) [2] have proven otherwise and have laid down the foundation by defining appropriate management protocols along with training a network of ocular oncologists. The transformation in the diagnostic approaches seen over the years has contributed immensely to the growth in this field along with a major role played by the advancements in ocular pathology enabling a perfect amalgamation of clinical-radiological-histopathological diagnosis.

Fig. 1.

A rare picture with three legends in one frame. From left to right: Drs. Robert M. Ellsworth, Algernon B. Reese, and David Abramson [1]

Fig. 2.

Drs. Jerry and Carol Shields (sitting, left to right) with their fellows (left to right): Drs. Hatem Krema, Hakan Demirci, Miguel Materin, Cynthia Carvalho, Noel Perez, Santosh Honavar, and faculty Dr. Arun Singh at the Wills Eye Hospital, Philadelphia, PA, USA, during an event in 2000 [3]

The most important task for the pioneers of ocular oncology in India was to train specialists in this field with precise knowledge, surgical skills, dedication, zeal, and a common outlook with limited availability of resources. It was important not only to ensure the establishment of a network throughout the country, but also to spread awareness amongst patients and general ophthalmologists about the peculiarity of these diseases and the need for a collaborated approach to timely notice the red flag signs. Following the footsteps of his mentors, the Shieldses, Dr. Santosh G. Honavar has been the trailblazer of this field in India who over the past 26 years of his dedicated clinical experience has selflessly and effortlessly trained over 80 fellows and helped them establish their career in different parts of the nation. His trained fellows are excelling in their respective regions and have given origin to a strong chain reaction by training their own fellows and residents. He has not just set the ball rolling, but has ensured a class apart provision of clinical care to ocular oncology patients along with contributions from various non-governmental organizations. He is also the mastermind behind introducing a cost-effective make-in-India Ruthenium-106 plaque for treating various ocular tumors by surface plaque brachytherapy [4].

The future holds scope of progress along with facing the key challenges like conducting clinical trials tailored as per the Indian population, laying down well-crafted protocols, addressing the lack of awareness, abolishing the unreasonable taboos, and bypassing the financial difficulties as this field requires a multi-disciplinary approach which can often be expensive and unaffordable in a developing country like ours.

A striking example of evolution in the field of ocular oncology is the management pattern of uveal melanoma. The early 1900s was the era of primary treatment as enucleation for large symptomatic uveal melanomas. As per the “Zimmerman-McLean-Foster hypothesis” popularized in the 1970s, a higher intra-ocular pressure during enucleation attributed to higher chances of liver metastasis via the vortex veins causing a peak in mortality [5, 6], and following this for several years, the “no-touch enucleation technique,” i.e., freezing the tumor at the time of enucleation, was practiced as advised by Fraunfelder et al. in order to prevent dissemination [7]. A trend of practicing local treatment took over, i.e., laser photocoagulation and radiotherapy with systemic prognosis being unaffected. The Collaborative Ocular Melanoma Study (COMS) indicated that the 5-year mortality rates in choroidal melanoma cases were similar after enucleation as well as plaque brachytherapy [8–10]. Singh et al. stated that the mortality rates for uveal melanoma managed by enucleation or plaque brachytherapy of proton beam therapy or helium ion therapy or resection appeared to be similar and can be attributed to a possibility of early micro-metastasis [11, 12]. Brewington et al. in 2018 commented that brachytherapy has evolved as the standard of care ensuring globe salvage in uveal melanoma and ocular screening along with adjunct modalities like genetic analysis by fine needle aspiration biopsy [13]. Shields et al. refined the management of posterior uveal melanomas as stated in the 2014 Charles L. Schepens lecture and declared the need for early diagnosis, prompt local treatment, and targeted therapies to avoid micro-metastasis and improve survival by interrupting the specific tumor pathway [14]. A similar evolution has been noticed in all intra-ocular and extra-ocular tumors as a result of the tireless efforts of several legendary ocular oncologists over the years.

Ocular oncology has evolved through the era of enucleation/exenteration and excision/incisional biopsies to chemotherapies and radiation therapies including brachytherapy, proton beam therapy, and stereotactic radiotherapy to genetics-based targeted therapy. It has been possible to tailor the management based on the presentation and individual needs of the patient with the available resources. A significant upsurge has occurred in terms of infrastructure dedicated to ocular oncology with a multi-disciplinary approach involving ophthalmologists, pathologists, radiologists, and medical and radiation oncologists, thereby reducing the morbidity and mortality owing to ocular cancers thus warranting high rates of vision salvage, globe salvage, and life salvage. We thank the pioneers who have let the light shine out of the darkness and lit up the world of ocular oncology.

Declarations

Competing Interests

The author declares no competing interests.