Image Based Question-65989

A child presents with Squint and Deterioration of Vision.On examination of the eye,following picture is seen.On ophthalmoscopic examination,a nodular cream colored mass with increased vascularization is seen.On histopathological examination of the mass,Rossetes are seen.

On CT scan-large heterogeneous posterior chamber mass with calcifications ,filling >50% of the globe is seen.In the managment,during the removal of the eye,which of the following structure is removed?

A. Central retinal artery.

B. Sclera and episclera.

C. Optic nerve.

D. Vortex vein.

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Correct Answer » C Explanation

Ans:C. Optic nerve.

The condition shown in the picture above represents Retinoblastoma-Group E .The patient is planned for Enucleation.

The International Classification for Intraocular Retinoblastoma :

• Group A: Small intraretinal tumors (< 3mm) away from foveola and disc.
• Group B: Tumors > 3mm, macular or juxtapapillary location, or with subretinal fluid.
• Group C: Tumor with focal subretinal or vitreous seeding within 3mm of tumor.
• Group D: Tumor with diffuse subretinal or vitreous seeding > 3mm from tumor.
• Group E: Extensive retinoblastoma occupying >50% of the globe with or without neovascular glaucoma, hemorrhage, extension of tumor to optic nerve or anterior chamber.

Treatment:

• Focally destructive therapy (cryopexy, laser photocoagulation, hyperthermia and plaque irradiation) for small tumors.

• Primary neoadjuvant chemotherapy or chemoreduction by vincristine, high-dose carboplatin, and etoposide -the principle mode of treatment for eyes in intraocular groups C and D.

• Eyes with large tumor burden (Group E ) and eyes that progress despite conservative treatments require enucleation.

• When an eye is enucleated for retinoblastoma, the goal is to remove as much optic nerve as possible to try ensure that the cut end of the nerve is free from tumor.