Update on the management of vasoproliferative tumour

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ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia

Short communication

Update on the management of vasoproliferative tumour夽,夽夽 I. Temblador-Barba ∗ , E.M. Delgado-Alonso, M. Toribio-García, L. Martínez-Campillo, I. Molina-Leyva, M. Martínez-Jiménez Servicio de Oftalmología, Hospital Universitario Virgen de las Nieves, Complejo Hospitalario Universitario, Granada, Spain

a r t i c l e

i n f o

a b s t r a c t

Article history:

Case report: Here we report a 19-year-old female patient who presented a vasoprolifera-

Received 18 September 2017

tive tumor. It caused complications, such as epiretinal membrane, macular edema, vitreous

Accepted 10 January 2018

hemorrhage, and exudative retinal detachment. The patient was treated with 3 injections of

Available online xxx

intravitreal bevacizumab, an intravitreal dexamethasone implant, tocilizumab, and double freeze-thaw cryotherapy.

Keywords:

Discussion: Therapeutic options are: observation, if it is small, if it is a peripheral lesion, and

Vasoproliferative tumor

if there seems to be no threat to vision. If it requires treatment, laser photocoagulation,

Intravitreal bevacizumab

intravitreal bevacizumab, trans-conjunctival cryotherapy, transpupillary thermotherapy,

Dexamethasone implant

photodynamic therapy, brachytherapy plaques and surgery are the different options avail-

Tocilizumab

able. Recently, tocilizumab and intravitreal dexamethasone implants have been reported to

Cryotherapy

be beneficial. ˜ ˜ S.L.U. All rights © 2018 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana, reserved.

Actualización sobre el manejo del tumor vasoproliferativo r e s u m e n Palabras clave:

˜ Caso clínico: Presentamos el caso de una paciente de 19 anos con un tumor vasoprolifera-

Tumor vasoproliferativo

tivo, en cuya evolución presentó una membrana epirretiniana, edema macular, hemovítreo

Bevacizumab intravítreo

y desprendimiento de retina exudativo. Se trató con 3 inyecciones intravítreas de beva-

Implante de dexametasona

cizumab, implante intravítreo de dexametasona, tocilizumab y 2 sesiones de crioterapia.

夽 Please cite this article as: Temblador-Barba I, Delgado-Alonso EM, Toribio-García M, Martínez-Campillo L, Molina-Leyva I, Martínez-Jiménez M. Actualización sobre el manejo del tumor vasoproliferativo. Arch Soc Esp Oftalmol. 2018. https://doi.org/10.1016/ j.oftal.2018.01.005 夽夽 This paper was presented at the 92nd Congress of the Ophthalmological Society of Spain held in Málaga on September 2016 as a Clinic Case Report. ∗ Corresponding author. E-mail address: [email protected] (I. Temblador-Barba). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2018 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,

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Tocilizumab

˜ periféricos Discusión: Las opciones terapéuticas son: observación en los de menor tamano,

Crioterapia

y sin amenaza para la visión. Si se necesita tratamiento, fotocoagulación con láser, crioterapia transconjuntival, inyecciones intravítreas de bevacizumab, termoterapia transpupilar, terapia fotodinámica, placas de radioterapia y cirugía son diferentes opciones disponibles. Recientemente se ha descrito que el tocilizumab y los implantes intravítreos de dexametasona pueden ser beneficiosos. ˜ ˜ S.L.U. Todos de Oftalmolog´ıa. Publicado por Elsevier Espana, © 2018 Sociedad Espanola los derechos reservados.

Introduction Vasoproliferative tumors (VPRT) are benign vascular nodular glial tumors that originate in the neurosensory retina. VPRT normally present as a peripheral solitary retinal mass, more frequently in the lower temporal quadrant, associating telangiectasia, lipidic exudation and subretinal fluid.1 VPRT are primary in approximately 75–80% of cases. The rest are secondary to other ocular diseases such as pars planitis, retinitis pigmentosa, toxoplasmosis, ocular toxocariasis, Coats disease or retinopathy of prematurity, among others.2 Patients generally refer loss of vision, photopsia or myodesopsia.1 Some exhibit a nonprogressive asymptomatic course, while others have progression with severe exudation, above all the extended tumors. Between 50 and 60% associate epiretinal membranes (EPRM) or cystoid macular edema. VPRT can also course with hemorrhages or exudative retina detachments (RD). In advanced cases, neovascular events could emerge.3

Clinic case Female patient, aged 19, with progressive vision loss in the left eye (LE). Visual acuity (VA) was 1 in the right eye (RE) and 0.2 in LE, without improvement utilizing stenopeic. LE biomicroscopy showed Tyndall+, without further details. Intraocular pressure was normal in both eyes. Ocular fundus (OF) examination was normal in RE but the LE showed a yellowish solid mass with telangiectasia in the superonasal peripheral retina, vitritis++ and inferior snow bank (Fig. 1A–C). Angiography with fluorescein (AGF) was attempted but the images were not conclusive. By means of other supplementary ophthalmological examinations (OCT and ocular echographies) together with systemic exploration with normal supplementary tests (expanded serology, Mantoux and chest X-ray), the diagnostic was VPRT. After establishing the diagnostic, macular traction with edema was observed (Fig. 2A). Initially, 2 intravitreal bevacizumab injections were administered and subsequently an intravitreal dexamethasone implant. After 2 months, VA was 0.4 with less exudation. The use of cryotherapy or placement of brachytherapy plates was considered because the extension of the tumor was at the limits of both treatments. Photodynamic therapy (PDT) was discarded due to tumor location. Subsequently, due to the appearance of hemovitreous with peripheral RD, an additional bevacizumab injection was pre-

scribed. One month later the condition remitted, with VA reading at 0.3 (Fig. 2B). Despite clinic improvement tumor size remained similar, for which reason treatment with tocilizumab was requested. Four doses of 400 mg were repeated at one month intervals. Vitreous inflammation diminished slightly but without large changes, for which reason 2 cryotherapy sessions were carried out, which diminished exudation and vitreous turbidity and slight tumor size reduction and improved control of superficial vessels. The hospital committee decided to suspend tocilizumab due to the lack of significant clinic improvement. At present, after 24 months, VA is 0.4. Lesion management was achieved, although posterior synechiae persist at 360◦ around the pupil edge, for which reason prophylactic iridotomy was performed. The mass is checked with echography and OCT, which evidences EPR without traction (Fig. 3A and B).

Discussion Supplementary ophthalmological tests that assist diagnostic comprise OCT, autofluorescence, AGF and ocular echography, which can help to differentiate difficult amelanotic melanoma cases. This shows a solid tumor with medium/high reflectiveness and lack of choroidal shadow. It is also used for measuring and following up response to treatment. OCT plays a limited role but is useful for documenting and following secondary findings such as the formation of edema or EPRM.4 Even though the ideal treatment is yet to be established in the form of a protocol, observation is the norm in small, peripheral tumors that do not compromise vision. If treatment is necessary, the available therapeutic options comprise laser photocoagulation, which plays a limited and supplementary role in small tumors, intravitreal bevacizumab as additional treatment to diminish edema and vitreomacular traction,5 trans-pupil thermotherapy, PDT,6 which has proved effective in choroidal and retinal tumors, including in moderate sized VPRT (approximately 2–4.5 mm thickness).3 Its main limitation is technical difficulty.2 The most widely used procedure is transconjunctival cryotherapy, considered mainly in cases with a diameter <2 mm. Frequently, more than one session is needed although it could involve persistence of the edema and the appearance of adjacent exudative RD.3 A further option are brachytherapy plates with 106 Ru or 125 I, mainly indicated for large tumors with thicknesses exceeding 2.5 mm and lesions associated to extensive RD. In the first group, lower radiation dosages are utilized and it seems that VA outcome is better. However, when neovascular glaucoma

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Fig. 1 – (A) LE posterior pole. (B) Whitish-yellowish mass with surface telangiectasia in superonasal peripheral retina compatible with VPRT. (C) Snow bank at 6 o’clock.

Fig. 2 – (A) OCT showing EPR pulling the macular area. (B) OCT after applying all treatments, showing that vitreomacular traction has disappeared.

Fig. 3 – (A) LE with posterior pupil synechiae at 360◦ . (B) Comparison of both maculae with OCT at time of writing this paper.

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emerges, brachytherapy fails in control. In addition, it involves risk due to radiation applied on the eye.7 Surgery is highly aggressive and is reserved for uncertain diagnostics or in thicker tumors associating complications with poor prognosis.8 It was recently reported that tocilizumab and intravitreal dexamethasone implants could be beneficial9,10 as the latter prevent macular edema and, applied together with PDT, diminish tumor vascular leaks.10

Conflict of interests No conflict of interests was declared by the authors.

references

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3. Kenawy N, Groenwald C, Damato B. Treatment of a vasoproliferative tumour with intravitreal bevacizumab (Avastin). Eye. 2007;21:893–4. ˜ ˜ 4. Pineiro-Ces A, Blanco-Teijeiro MJ, Mera-Yánez MP, Capeans-Tome C. Diagnóstico ecográfico de los tumores vasoproliferativos del fondo de ojo. Arch Soc Esp Oftalmol. 2011;86:247–53. 5. Marback EF, Guerra RL, Maia Junior Ode O, Marback RL. Retinal vasoproliferative tumor. Arq Bras Oftalmol. 2013;76:200–3. 6. Jain K, Berger AR, Yucil YH, McGowan HD. Vasoproliferative tumours of the retina. Eye. 2003;17:364–8. 7. Anastassiou G, Bornfeld N, Schueler AO, Schilling H, Weber S, Fluehs D, et al. Ruthenium-106 plaque brachytherapy for symptomatic vasoproliferative tumours of the retina. Br J Ophthalmol. 2006;90:447–50. 8. Smith J, Steel D. The surgical management of vasoproliferative tumours. Ophthalmologica. 2011;226 Suppl 1:S42–5. 9. Adán A, Mesquida M, Llorenc¸ V, Modesto C. Tocilizumab for retinal vasoproliferative tumor secondary to juvenile idiopathic arthritis-associated uveitis: a case report. Graefes Arch Clin Exp Ophthalmol. 2014;252:163–4. 10. Cebeci Z, Oray M, Tuncer S, Tugal Tutkun I, Kir N. Intravitreal dexamethasone implant (Ozurdex) and photodynamic therapy for vasoproliferative retinal tumours. Can J Ophthalmol. 2014;49:e83–4.