www.ophtalmique.ch

Pournaras Jean-Antoine C

Vitreoretinal Surgery Unit

Vitreoretinal surgical management

In ocular oncology

1. Surgical resection after proton

beam therapy

2. Ocular Biopsy

3. RD in advanced

Retinablastoma

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Surgical tumor resection after proton beam therapy

Conservative treatment of uveal melanomas

COMS study 1986-2001

Enucleation : 660 cases

125I brachytherapy : 657 cases

Absence of similar study on surgical management

Three lines treatment

• 1. Tumor Control

• 2. Eye retention probability

• 3. Functional result

1. Sterilization of tumor site

• Proton Beam Radiotherapy

Egger et al. Int J Radiat Oncol Biol Phys 2001

Local tumor control

• 91% before 1993

• 96% 1989-1993

• 98.8% after 1998

n = 2435 MM

Proton beam irradiation 10 years

Local tumor control 98.8 %

Eye retention probability 95.6 %

84- 87

88- 93 88- 93

94- 05 94- 05

Complications

• Radiation induced retinopathy and maculopathy

• Secondary Neovascular Glaucoma

Toxic Tumor Syndrome

• caused by the presence of a large volume of irradiated

tumor

either because it becomes ischemic or

because it causes extensive retinal detachment or

both

• Inflammatory cytokines release

• Exudation from irradiated / incompetent vessels

• VEGF from ischemic tissue

2. Management of complications

• Panphotocoagulation

• Anti-VEGF, Corticosteroids

• Secondary enucleation

• VR surgery exoresection / endoresection of toxic tumor

Exoresection

• Indications: large tumor size, anterior location, exudative

retinal detachment – Two matched group studies have reported that with large tumors, better

results after exoresection than after iodine plaque radiotherapy

Kivelä et al. Ophthalmology 2003;Bechrakis et al. Ophthalmology 2002

• Complex surgery; rarely use

• Adjunctive brachytherapy or proton beam therapy

Excision of tumor with internal scleral lamellae

Risk of recurrence 50% in external scleral lamellae

Damato et al. 2012

Endoresection

• Primary Endoresection Damato et al. BJO 1998; Garcia-Arumi et al. Retina 2001; Garcia-Arumi et al. BJO 2008; Konstantinidis et al. BJO 2014

• small case series

• short follow-up period

• recent study concern <12mm in 90 % cases observation required

• Impossible to attest complete resection

• If tumor recurrence, higher mortality rate

• Neoadjuvant combined radiotherapeutic-surgical approach Bornfeld et al. 2002; Bechrakis et al.2006

Endoresection: Surgical technique

• 1 23 G pars plana vitrectomy

• 2 retinotomy over the tumor

• 3 piecemeal tumor removal (increased intraocular

pressure)

• 4 endodiathermy to the margins

• 5 fluid–air exchange to flatten the retina

• 6 endolaser retinopexy to attach retina around tumor site

• 7 air–silicone exchange to maintain retinal flattening and

to prevent postoperative hemorrhage

Peyman et al. 1988; Damato et al. BJO 1998

Case 1. Toxic tumor syndrome

• Complex surgery

• Common

retinectomy and

tumorectomy

• 1 or more surgeries

• Phtisis

Case 2: Massive

ischemia, extensive

exudative RD

• Man, 44 yo

• Thickness 8.2

• 60Gy in 4 fractions (2.2012)

• Indication: – progression exsudative RD

– Intravitreal hemorrhage 2.2013

• Surgery 2.2013

9.2013

Case 3: Knapp-

Ronne type

• Man, 48 yo

• 60Gy in 4 fractions

• Indication: – Risk progression exsudative RD

– Risk Intravitreal hemorrhage

• Tumorectomy +Silicon oil 5.2013

• Silicon oil removal 8.2013

Case 4: Recurrent vitreous hemorrhage

• Woman, 42 yo

• Thickness 6.1mm

• Vx silicon oil 25-28.1.2012

• 60Gy in 4 fractions (27.2-2.3.2012)

• Tumorectomy 12.6.12

• Phaco + silicon oil removal 23.10.12

• VA 0.8 cc 2014

Endoresection: Indications

• Risk of hemorrhage, pigment dispersion, retinal infiltration

early tumorectomy

• Risk of hemorrhage, pigment dispersion, retinal infiltration

Without previous episode

wait for tumor regression 3 to 6 months

No changes at 3 or 6 months:

tumorectomy

• Any tumor with diffuse exudative RD, lipid deposits,

necrosis, excessive inflammation

early tumorectomy

Primary vitrectomy

• Diagnosis and Prognosis

• Transillumination gives information about basal infiltration

in order to define tumor borders, impeded by vitreous

hemorrhage

• 1. US of tumor with AL calculation.

• 2. Vx + silicon oil (particles speed modified in silicon oil)

• 3. Proton beam therapy

• 4. Silicon removal + Vx + tumorectomy + silicon oil

• 5. Removal of silicon oil

Conclusions

• 1. Local tumor control

– Primary vitrectomy may be discussed in vitreous hemorrhage cases

without tumorectomy

• 2. Secondary Endoresection

• Development of VR instrumentation

• Surgery may become essential in tumor management

• Oncologists decision with high variability among centers

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Ocular biopsy

Role of biopsy

• Diagnostic tool when all investigations failed and if management may be influenced

Biospy reveals 50% Choroidal melanoma

50% uveal metastasis

• Molecular and genomic analysis

– Characterisation of the tumor (cell type, mutational status)

– Prognostic information

• monosomy 3 + 8q gain in choroidal melanoma are clinically relevant and strongly associated with metastatic disease and death

Prescher et al. Lancet 1996; White et al. Cancer 1998; Scholes et al. IOVS 2003; Cassoux et al. 2013

– Targeted therapy

Biopsies

• Transcleral choroidal biopsy

• Transvitreal choroidal biopsy

• Fine Needle Aspiration Biopsy

Transcleral biopsy

Indications:

– If access to the tumor (avoid VR complications)

– Benign tumor or adjuvant radiotherapy should be performed

Do D, Nguyen QD. In Ryan, Retina, 5th.

Transcleral biopsy

• Dissection of 6 × 6 mm scleral flap, nearly full-thickness and hinged

(usually posteriorly)

• Near Full-thickness scleral flap is retracted

• biopsy specimen is grasped at one edge

• Incision of choroidal tissue with a sharp blade. Scissors may be used to

complete the dissection

• Careful separation of the retina from the choroid

• Suture of the scleral flap

• Pericard patch

• Vitrectomy reduces bulging of the retina during procedure Peyman et al. 1978

Do D, Nguyen QD. In Ryan, Retina, 5th.

Transvitreal biopsy

Indications:

– no access by transcleral route

– posterior plan tumor

– malign tumor lead directly to enucleation / RX / Chimioth

without dispersion by transcleral approach

Transvitreal biopsy

• Pars plana vitrectomy

• Endolaser around the margins of the intended biopsy site

• Increased intraocular pressure

• Retinotomy

• Biopsy of the tumor

• Fluid/gas exchange, 20% SF6

• A newly developed instrument, the Essen biopsy forceps was reported to

be effective in the diagnosis of choroidal tumors in 20 patients Bechrakis et al. 2002; Akgul et al. BJO 2011

• photo

• Photo avant après

• Film 1

Intraocular metastasis of

pulmonary adenocarcinoma

(cords and ductal structures;

TTF1 +; naspin A +)

• Film 2

Marginal zone B cell lymphoma

Fine-needle biopsy

• Extensively used in the diagnostic evaluation including

tumors of the orbit and eye

• Major concern about risk of dissemination

– No tumor dissemination along the needle track, no such

occurrence has been documented with a needle of 25G or finer McCannel et al. Ophthalmology 2012

• As diagnostic tool, no histological information, only

cytology

• Prognostic informations

Fine-needle biopsy: surgical technique

• 22G–30G needles for intraocular aspiration Jakobiec et al. 1979; Augsburger JJ, Shields JA, et al. 1985

• Needle length depends on tumor intraocular location of

the tumor and planned biopsy route

• Biopsy needle is connected to a plastic disposable

aspirating syringe via a standard plastic tubing

Complications

• Usual vitreoretinal surgery complications

• increased intraocular pressure

• Cataract progression

• Peripheral retinal tears

• Retinal detachment

• Choroidal hemorrhage

• Vitreous hemorrhage

• Endophthalmitis

• Exacerbation of the underlying inflammatory disease

• Proliferative vitreoretinopathy

Young TA, et al. AJO 2008

Conclusions

• No diagnosis by current modalities (clinical observation,

angiogram, ulttrasonography)

• Biopsy is recommended if there is substantial likelihood

that the results will improve patient management

• These procedures can be safely performed by retina

surgeons according to experienced oncologists