Ocular Oncology [1st ed.] 978-981-13-2335-5;978-981-13-2336-2

Table of contents :
Front Matter ....Pages i-vii
Choroidal Nevus (Carol L. Shields, Jerry A. Shields)....Pages 1-12
Choroidal Melanoma (Carol L. Shields, Jerry A. Shields)....Pages 13-26
Choroidal Metastasis (Greg Bever, Armin Afshar, Bertil Damato)....Pages 27-33
Choroidal Hemangioma (Duangnate Rojanaporn)....Pages 35-44
Choroidal Osteoma (Tero T. Kivelä)....Pages 45-59
Retinal Vascular Tumors (Jose J. Echegaray, Rubens Belfort Neto, Arun D. Singh)....Pages 61-65
Vasoproliferative Tumors of the Retina (Duangnate Rojanaporn)....Pages 67-75
Astrocytic Hamartoma of the Retina (Andrew W. Stacey, Mandeep S. Sagoo)....Pages 77-81
Intraocular Lymphoma (Hiroshi Goto)....Pages 83-90
Retinoblastoma (Carol L. Shields, Sara E. Lally)....Pages 91-99
Tumors and Related Lesions of the Retinal Pigmented Epithelium (Carol L. Shields, Jerry A. Shields)....Pages 101-114
Retinal Metastasis (Sachin M. Salvi, Soyang E. Kim, Arun D. Singh)....Pages 115-118
Peripheral Exudative Haemorrhagic Chorioretinopathy (Peter Heydon, Mandeep S. Sagoo)....Pages 119-122
Optic Disc Melanocytoma (Minoru Furuta)....Pages 123-130
Intraocular Medulloepithelioma (Shweta Rathi Gupta, Swathi Kaliki)....Pages 131-138
Paraneoplastic-Related Retinopathy (Tharikarn Sujirakul, Stephen H. Tsang)....Pages 139-146
Sclerochoroidal Calcification (Tero T. Kivelä)....Pages 147-158
Correction to: Ocular Oncology (Duangnate Rojanaporn)....Pages C1-C1

Citation preview

Duangnate Rojanaporn Editor

Ocular Oncology

123

Ocular Oncology

Duangnate Rojanaporn Editor

Ocular Oncology

Editor Duangnate Rojanaporn Department of Ophthalmology Faculty of Medicine Ramathibodi Hospital Mahidol University Bangkok Thailand

ISBN 978-981-13-2335-5    ISBN 978-981-13-2336-2 (eBook) https://doi.org/10.1007/978-981-13-2336-2 © Springer Nature Singapore Pte Ltd. 2019 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Singapore Pte Ltd. The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721, Singapore

Contents

1 Choroidal Nevus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Carol L. Shields and Jerry A. Shields 2 Choroidal Melanoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 Carol L. Shields and Jerry A. Shields 3 Choroidal Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Greg Bever, Armin Afshar, and Bertil Damato 4 Choroidal Hemangioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35 Duangnate Rojanaporn 5 Choroidal Osteoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 Tero T. Kivelä 6 Retinal Vascular Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61 Jose J. Echegaray, Rubens Belfort Neto, and Arun D. Singh 7 Vasoproliferative Tumors of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 Duangnate Rojanaporn 8 Astrocytic Hamartoma of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77 Andrew W. Stacey and Mandeep S. Sagoo 9 Intraocular Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83 Hiroshi Goto 10 Retinoblastoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Carol L. Shields and Sara E. Lally 11 Tumors and Related Lesions of the Retinal Pigmented Epithelium . . . . . . . . . . 101 Carol L. Shields and Jerry A. Shields 12 Retinal Metastasis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 Sachin M. Salvi, Soyang E. Kim, and Arun D. Singh 13 Peripheral Exudative Haemorrhagic Chorioretinopathy. . . . . . . . . . . . . . . . . . . 119 Peter Heydon and Mandeep S. Sagoo 14 Optic Disc Melanocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 Minoru Furuta 15 Intraocular Medulloepithelioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 Shweta Rathi Gupta and Swathi Kaliki 16 Paraneoplastic-Related Retinopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139 Tharikarn Sujirakul and Stephen H. Tsang 17 Sclerochoroidal Calcification. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147 Tero T. Kivelä v

About The Editor

Duangnate  Rojanaporn, MD, FICO graduated from Mahidol University, Bangkok, Thailand, before completing an International Council of Ophthalmology (ICO) fellowship in medical and surgical retina at New  York Eye and Ear Infirmary and a fellowship in ocular oncology at Wills Eye Hospital, USA. She is the Director of the Ocular Oncology Service, and course instructor for the Retina Service at the Department of Ophthalmology, Ramathibodi Hospital. Dr. Rojanaporn is a pioneer of plaque brachytherapy, and intra-arterial chemotherapy for retinoblastoma in the South East Asia region. She coordinates the ocular oncology program for several international conferences and has been invited to deliver presentations at numerous local, regional, and international meetings. Dr. Rojanaporn received an ICO fellowship grant in 2008, a Professor Yasuo Tano travel grant for the Asia-Pacific Academy of Ophthalmology (APAO) meeting in 2013, and an Asia-Pacific Academy of Ophthalmology Achievement Award in 2018.

vii

‘Retina Atlas’ series includes the following 9 Volumes: 1. 2. 3. 4. 5. 6. 7. 8. 9.

Retinal Imaging Retinal Vascular Disorders Macular Disorders Surgical Retina Inflammatory and Infectious Ocular Disorders Hereditary Chorioretinal Disorders Pediatric Retinal Diseases Ocular Oncology Trauma in Retina

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1

Choroidal Nevus Carol L. Shields and Jerry A. Shields

1.1

Introduction

Choroidal nevus is the most common benign intraocular tumor, found predominantly in Caucasian patients (Chien et  al. 2017; Shields and Shields 2015; Kaliki and Shields 2015). This tumor is generally asymptomatic but can carry risk for visual acuity loss, especially if located near the foveola, and risk for transformation into malignant melanoma. Several features allow clinical identification of choroidal nevus and differentiation from melanoma.

1.2

Prevalence

There have been a few population-based studies reporting on the prevalence of choroidal nevus including the Blue Mountains Eye Study (6.5%), Beijing Eye Study (BES) (2.9%), Singapore Malay Eye Study (SiMES) (1.4%), Multi-­ Ethnic Study of Atherosclerosis (MESA) (2.1%), Central India Eye and Medical Study (CIEMS) (0.3%), and National Health and Nutrition Examination Survey (NHANES) (4.7%) (Sumich et al. 1998; Jonas et al. 2008; Ng et al. 2009; Greenstein et al. 2011; Nangia et al. 2012; Qiu and Shields 2015). Most of the population-based studies have focused on specific ethnic groups such as Australian (Sumich et al. 1998), Chinese (Jonas et al. 2008), or Asian Indian (Ng et  al. 2009) population. The NHANES study reviewed all ethnic groups in the United States (US) and found overall prevalence of choroidal nevus in US adult population≥40  years old was 4.7%, and this lesion was C. L. Shields (*) · J. A. Shields (*) Ocular Oncology Service, Wills Eye Hospital, Philadelphia, PA, USA Department of Ophthalmology, Thomas Jefferson Unversity, Philadelphia, PA, USA

most commonly detected in Whites at 5.6% (Ng et al. 2009) (Table 1.1). Population-based studies often limit inclusion criteria to study a certain age group, generally adults without inclusion of younger age group, or study a prominent ethnic group, or study only a limited portion of the fundus. The NHANES study included all ethnicities but was deficient in that it studied only individuals >40  years old and was limited to two 45-degree photographs of the macula and optic disc (Qiu and Shields 2015). This study potentially underrepresented the true prevalence of choroidal nevus in US adults as one might speculate that if the entire fundus was included, the prevalence of choroidal nevus could reach as high as 20–25% (Qiu and Shields 2015).

1.3

Clinical Features

Choroidal nevus is a flat or minimally elevated mass with brown pigmentation (melanotic; pigmented) or without (amelanotic; nonpigmented) (Chien et  al. 2017; Sumich et al. 1998; Jonas et al. 2008; Ng et al. 2009; Greenstein et al. 2011; Nangia et  al. 2012; Qiu and Shields 2015; Shields et al. 2008a; Shields et al. 2010; Shields and Shields 2015; Kaliki and Shields 2015). This tumor is classically in the post-equatorial fundus (91%) or pre-equatorial fundus (9%), and most nevi are pigmented (77%) (Shields et  al. 2008a) (Table  1.2). The distribution of choroidal nevus is equivalent in all quadrants (Shields et al. 2008a). Most choroidal nevi are extrafoveolar (94%) compared to subfoveolar (6%). Tumor size varies depending on the study, but one tertiary ocular oncology clinic-based center found mean basal tumor diameter was 5 mm and thickness was 1.5 mm, compared to a population-based study where choroidal nevus was found to have mean basal diameter of 1.25 mm (Sumich et al. 1998; Shields et al. 2008a).

© Springer Nature Singapore Pte Ltd. 2019 D. Rojanaporn (ed.), Ocular Oncology, https://doi.org/10.1007/978-981-13-2336-2_1

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C. L. Shields and J. A. Shields

Table 1.1  Prevalence of choroidal nevus from a population-based study (National Health and Nutrition Examination Survey 2005–2008) in the United States: grouped by age, sex, and racea Sex Male (n = 2785)

Female (n = 2790)

P valueb

Race White (n = 3012)

Black (n = 1133)

Hispanic (n = 1249)

Other (n = 181)

P valueb

Total

1483 1322 1384 915 471

5.0% 3.3% 6.5% 6.8% 7.5% 0.07

4.4% 2.9% 4.4% 6.5% 7.5% 0.1

0.7 0.7 0.2 0.9 0.9 n/a

5.8% 3.7% 6.3% 7.3% 7.7% 0.08

0.7% 0.3% 0.9% 0.0% 0.1% 0.6

1.3% 2.9% 4.1% 5.6% 8.5% 0.07

4.4% 0.0% 0.8% 1.6% 11.6% 0.08

0.005 0.04 0.002 0.2 0.6 n/a

4.7% 3.1% 5.4% 6.6% 7.5% n/a

3012 1133 1249 181

6.2% 1.0% 2.8% 0.5% 50 years old) (n = 2564) [n (%)]

22 (35) 40 (63) 1 (2)

186 (23) 548 (69) 61 (8)

504 (20) 1821 (71) 239 (9)

22 (35) 7 (11) 11 (17) 8 (13) 15 (23)

186 (23) 140 (18) 162 (20) 116 (15) 191 (24)

504 (20) 460 (18) 631 (25) 410 (16) 559 (22)

9 (14) 54 (86) 5.0 (5.6, 0.75–24) 1 (1.2, 0.6–22)

53 (7) 742 (93) 4.5 (4.7, 0.5–14) 1.5 (1.5, 0.7–3.7)

143 (6) 2421 (94) 5 (5.2, 0.4–20) 1.5 (1.6, 0.7–4.5)

56 (89) 4 (6) 3 (5)

591 (74) 83 (10) 121 (15)

1981 (77) 269 (10) 313 (12)

Information adapted from an ocular oncology clinic-based case series, 10 Shields CL, et al. Clinical spectrum of choroidal nevi based on age at presentation in 3422 consecutive eyes. Ophthalmology 2008;115:546–52

1.3.1 Low-Risk Choroidal Nevus Choroidal nevus can be categorized into low or high risk for transformation into melanoma. Low-risk nevus is one that carries low likelihood for transformation into melanoma. Features include thickness ≤2 mm and absence of subretinal fluid, orange pigment, and symptoms. These lesions classically appear echodense on ultrasonography and demonstrate overlying retinal pigment epithelium (RPE) alterations such as drusen, RPE atrophy, and dependent RPE trough from previous subretinal fluid, RPE hyperplasia, RPE detachment, RPE fibrous metaplasia, and RPE osseous metaplasia (Shields et al. 2008a; Kaliki and Shields 2015) (Figs. 1.1, 1.2, 1.3, 1.4 and 1.5). Rarely, choroid nevus can produce chronic RPE damage that leads to development of choroidal neovascular membrane (Shields et al. 2008a) (Fig. 1.6).

1.3.2 High-Risk Choroidal Nevus High-risk nevus is one that carries high likelihood for transformation into melanoma. Features include thickness >2 mm, presence of subretinal fluid, orange pigment and/or symptoms, acoustic hollowness on ultrasonography, and absence of chronic features such as drusen or surrounding halo (Shields et al. 2009) (Table 1.3) (Figs. 1.7 and 1.8). Location can be a factor as nevi within 3 mm of the optic disc are more likely to evolve into melanoma. High-risk nevus classically appears with little to no overlying RPE alterations such as drusen.

1  Choroidal Nevus

a

3

b

c d

Fig. 1.1  Choroidal nevus with overlying drusen. (a) Drusen covering the entire aspect of the nevus. (b) Drusen covering the central apex of the nevus. (c) Drusen extending beyond the nevus margins. (d) Larger choroidal nevus with central retinal pigment epithelial atrophy and drusen

1.3.3 Halo Nevus Halo nevus is an unusual form of cutaneous nevus, displaying a central pigmented portion with surrounding ­depigmented halo, most often found in young patients and believed to represent an immune response. Halo choroidal nevus is characterized by a pigmented nevus surrounded by a nonpigmented nevus halo, representing 5% of all choroidal nevi (Shields et al. 2010) (Fig. 1.9). Choroidal halo nevus has been found most often to signify a “low-risk” nevus (Shields et al. 2009). In one analysis of 150 patients with halo choroidal nevus, Shields et  al. found relationship to previous history of skin melanoma (p  2 mm (vs. ≤2 mma,b) Subretinal fluid present (vs. absent a) Symptoms    Decreased vision (vs. none a)    Flashes/floater (vs. none a) Orange pigment present (vs. absent a) Margin distance to optic nerve ≤3 mm (vs. >3 mm a) Ultrasonographic acoustic hollowness (vs. solid a) Halo absent (vs. present a) Drusen absent (vs. present)

Hazard ratio (compared to nevus without feature) P value 2 15.0 12.1–15.0 9.1–12.0 6.1–9.0 3.1–6.0 ≤ 3.0

Tumor subcategory Primary tumor (T) T1  T1a  T1b  T1c  T1d T2  T2a  T2b  T2c  T2d T3  T3a  T3b  T3c  T3d T4  T4a  T4b  T4c  T4d  T4e

Tumor category (T1–T4)

3 2 2 1 1 1 1 3.1–6 0.0 ≤3.0 Melanoma basal diameter (mm)

3 2 1 1 6.1–9.0

3 3 2 2 1 9.1–12.0

4 3 3 3 2 2 12.1–15.0

Ciliary body involvement

Extraocular extension

No Yes No Yes

No No Yes ≤5 mm diameter Yes ≤5 mm diameter

No Yes No Yes Yes or no No Yes No Yes

No No Yes ≤5 mm diameter Yes ≤5 mm diameter Yes or no No No Yes ≤5 mm diameter Yes ≤5 mm diameter

4 4 3 3 3 2 15.1–18.0

4 4 4 4 4 4 >18.0

No No Yes No No Yes ≤5 mm diameter Yes Yes ≤5 mm diameter Any tumor size with extraocular extension >5 mm diameter

Tumor stage Tumor category (T) Node (N) Metastasis (M) Stage T1a N0 MO I T1b–d N0 MO IIA T2a N0 MO IIA T2b N0 MO IIB T3a N0 MO IIB T2c–d N0 MO IIIA T3b–c N0 MO IIIA T4a N0 MO IIIA T3d N0 MO IIIB T4b–c N0 MO IIIB T4d–e N0 MO IIIC Any T N1 MO IV Any T Any N M1 IV Adapted from Kivela T, Simpson ER, Grossniklaus HE, et al. Uveal melanoma. In Amin MB, Edge SB, Greene FL, et al.,, eds. In: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer; 2017:805–15

2  Choroidal Melanoma

23

a

b

d

c

Fig. 2.9  Optical coherence tomography (OCT) of small choroidal melanoma. (a) Small, juxtapapillary choroidal melanoma with (b) OCT features of dome-configuration and overlying trace subretinal fluid with other retinal hyper-reflectivity. (c) Small, submacular choroidal mela-

noma with (d) OCT features of dome configuration and overlying moderate subretinal fluid with shaggy photoreceptors and thickening of the retinal pigment epithelial layer which could represent lipofuscin (orange pigment)

Table 2.4  Uveal melanoma prognosis based on high-risk cytogenetics based on chromosomes 3, 6, and 8 alterations Chr 3 0 0

Chr 6q loss/gain 0 0

Chr 6p loss/gain 0 Gain

Chr 8q gain 0 0

Chr 8p loss/gain 0 0

# Cases (%) 189 (35) 38 (7)

# Metastasis (%) 2 (1) 2 (5)

0 0

Loss Loss

0 Gain

Gain Gain

Gain 0

1 (