Eye tumors
Most common Intraocular malignancy in children is Retinoblastoma
Most common Intraocular malignancy in adults is Metastasis > Melanoma choroid
Most common Malignant orbital tumor / Primary orbital malignancy / Soft tissue malignancy in children is Rhabdomyosarcoma
Most common Malignant orbital tumor / Primary orbital malignancy / Soft tissue malignancy in adults is Lymphoma
Most common Benign orbital tumor / Periorbital tumor in children is Capillary hemangioma
Most common Benign orbital tumor / Periorbital tumor in adults is Cavernous hemangioma
Most common cause of orbital metastasis in children is Neuroblastoma
Most common cause of orbital metastasis in adults is Breast > Bronchus > Prostate > Skin
RETINOBLASTOMA :-
Most common Primary intraocular malignant tumor of childhood
Most common neoplasm associated with retinoblastoma is Pinealoblastoma
Most common secondary malignant neoplasm associated with retinoblastoma is Osteosarcoma
Autosomal dominant - RB1 gene - 13q14 chromosome
Follows Knudson's two hit hypothesis (Explains loss of Heterozygosity by second hit and thus malignant transformation)
93% of RB are Sporadic , 1/3rd of sporadic cases are Heritable
Symptoms - Leukocoria (Most common) , Strabismus (Most commonly Esotropia)
Leukocoria in a child less than 6 yrs with Heterogenous mass and Calcification on USG (or) CT scan is diagnostic of Retinoblastoma
Characteristic features - Pseudorosette , Flexner-Wintersteiner Rosette , Homer-Wright Rosette , Flurettes
Most common route of spread of Retinoblastoma is Intraneural spread (i.e.., Direct invasion of optic nerve with extension into CNS)
LDH of Aqueous humor is marker for Retinoblastoma
Complications - Retinal detachment , Secondary glaucoma
Treatment -
Ideal treatment of Bilateral Retinoblastoma is Enucleation and Chemotherapy
Enucleation means Removal of Entire Globe along with Portion of Optic nerve
In retinoblastoma , after enucleation 8 to 12 mm piece of optic nerve is sectioned to find out systemic metastasis
Treatment of metastatic disease in retinoblastoma is Chemotherapy
Most bilateral multifocal retinoblastoma require Multiple modality therapy
MALIGNANT MELANOMA OF CHOROID :-
1st most common primary intraocular malignancy in adults
2nd most common intraocular malignancy in adults
Histopathology - Penetration of Bruch's membrane in Collar stud fashion
Tumor spread -
Invasion of Vortex veins leading to its compression causes Glaucoma
Most common site of Metastasis is Liver (Hematogenous spread)
Indirect ophthalmoscopy is Gold standard investigation for choroidal melanoma
Plaque Brachytherapy is most common treatment modality
Spindle cell melanoma has best prognosis
Callender's classification is used for choroidal melanoma
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