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Pournaras Jean-Antoine C

Vitreoretinal Surgery Unit

Vitreoretinal surgical management In ocular oncology

1. Surgical resection after proton beam therapy

2. Ocular Biopsy

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Surgical tumor resection after proton beam therapy

Conservative treatment of uveal melanomas

COMS study 1986-2001 Enucleation : 660 cases125I 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 yearsLocal 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 tumoreither because it becomes ischemic orbecause it causes extensive retinal detachment orboth

• 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 radiotherapyKivelä 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 lamellaeRisk 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 approachBornfeld 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 infiltrationearly tumorectomy

• Risk of hemorrhage, pigment dispersion, retinal infiltrationWithout 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 melanoma50% 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 procedurePeyman 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 / Chimiothwithout 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 finerMcCannel et al. Ophthalmology 2012

• As diagnostic tool, no histological information, only cytology

• Prognostic informations

Fine-needle biopsy: surgical technique

• 22G–30G needles for intraocular aspirationJakobiec 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