Intraocular Tumors By Dr. Ricky Mittal

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Slide 1:

Dr Ricky Mittal Institute of ophthalmology, JNMC AMU Aligarh

Intraocular Tumors:

Intraocular Tumors A mass lesion that involves, occupies, or replaces the interior tissues of the eye

Neoplasm:

Neoplasm Lesion composed of cells that no longer respond to the normal growth restraining mechanisms of the body Benign Malignant

Benign Neoplasms:

Benign Neoplasms Well differentiated, mature cells, resemble the tissue from which they arose Limited pleomorphism Infrequent mitotic figures Grow expansively ( vs. infiltration) Component cells maintain cohesiveness ( prevents intravascular/ intralymphatic dispersion) Inability to metastasize

Malignant Neoplasms:

Malignant Neoplasms Rapidly growing cells, less well differentiated than benign neoplasms Component cells are highly pleomorphic Numerous mitotic figures Grow expansively + infilterate, intravasate, disseminate & metastasize

Carcinoma:

Carcinoma Malignant neoplasm derived from cells of epithelial origin (entoderm/ ectoderm)

Sarcoma:

Sarcoma Malignant neoplasm derived from cells of mesenchymal origin Bones Muscles Blood vessels Lymphatics Blood forming tissues

Teratoma:

Teratoma Malignant neoplasm that arises from multipotential cells ( various combination of entoderm, ectoderm & mesoderm)

Tumor :

Tumor Localized swelling or mass Localized Exudative fluid within a tissue Localized Accumulation of inflammatory cells Localized Vascular dilatation / congestion Localized accumulation of neoplastic cells Combination of above factors

Neoplastic tumors:

Neoplastic tumors Abnormal mass of cells/ tissues that arises without obvious cause from pre-existent normal tissues Benign Malignant

Hamartoma :

Hamartoma Benign neoplastic tumor composed of well differentiated, histologically normal cells & tissues that occur normally, but in smaller number or amounts in the involved site or tissues

Choristoma :

Choristoma Benign neoplastic tumor composed of well differentiated, histologically normal cells & tissues that occur normally in some bodily site or tissues, but not normally in the involved site or tissue

Classification of Neoplasms :

Classification of Neoplasms Anatomic ( lung, colon skin,eye etc. ) Tissue ( retina, choroid etc. ) Histopathologic features Embryologic tissue of origin Tumor - node - metastasis staging Specific tumor classification

Intraocular tumors (Clinical evaluation - History):

Intraocular tumors (Clinical evaluation - History) Generally non specific symptoms Nature Duration Progression Previous medical/ surgical episodes

Clinical evaluation (Physical examination):

Clinical evaluation (Physical examination) Slit lamp biomicroscopy Gonioscopy fundus examination - Indirect ophthalmoscopy - Direct ophthalmoscopy - fundus biomicroscopy Trans pupillary/ trans conjunctival ocular transillumination

Clinical evaluation (Ancillary Diagnostic Studies):

Clinical evaluation (Ancillary Diagnostic Studies) Color photography to document appearance of observed lesion Fluorescein angiography to assess vascularity & filling pattern of the lesion Indocyanine green angio/ videography provides better delineation of vascularity & filling pattern under infrared spectrum

Clinical evaluation (Ancillary Diagnostic Studies):

Clinical evaluation (Ancillary Diagnostic Studies) Ultrasonography to assess cross sectional shape, internal reflectivity, thickness and intralesional blood flow Computed tomography for imaging of neoplasm and calcification ( retinoblastoma & choroidal osteoma), not particularly useful in differential diagnosis of soft tissue neoplasms

Clinical evaluation (Ancillary Diagnostic Studies):

Clinical evaluation (Ancillary Diagnostic Studies) Magnetic resonance imaging ( MRI) Radioactive phosphorus - 32 uptake, now rarely used

Clinical evaluation (Ancillary Diagnostic Studies):

Clinical evaluation (Ancillary Diagnostic Studies) Biopsy - accurate but infrequently done because I) clinical diagnostic accuracy more than 98% for experienced ophthalmologists II) gross incisional biopsies of malignant tumors associated with high orbital recurrence and death from metastasis III) biopsy with fine needle associated with histopathological needle track seeding & complications like bleeding

Clinical evaluation (Ancillary Diagnostic Studies):

Clinical evaluation (Ancillary Diagnostic Studies) Biopsy indicated in Some observers strongly suspect malignancy but major disagreement about histopathologic diagnosis Suspected metastatic carcinoma with no h/o extraocular malignancy or any concurrent primary /metastatic foci amenable to biopsy Patient insists on biopsy confirmation before therapeutic intervention

Clinical evaluation (Ancillary Diagnostic Studies):

Clinical evaluation (Ancillary Diagnostic Studies) Biopsy Fine needle aspiration - preferred Microsurgical incisional biopsy ( transconjunctival, transscleral & transvitreal) Diagnostic vitrectomy

Management Considerations:

Management Considerations Management depends on multiple factors Nature of tumor Extent of intraocular lesion Presence & extent of extraocular lesion Laterality of tumor Anatomic effects of tumor on adjacent & remote intraocular tissues Visual status of affected eye/s

Management Considerations:

Management Considerations Visual status of fellow eye Recognized potential benefits vs. risks & complications of available therapies Age of patient General health of patient Patient’s expectations & belief Affordability

Intraocular tumors:

Intraocular tumors Three major IO tumors are - retinoblastomas - ocular metastases - ocular melanomas

Intraocular tumors:

Intraocular tumors Others - nevi - hemangiomas - tumors of RPE - phakomatoses - lymphoid tumors - leukemias - histiocytic lesions - ocular syndromes associated with systemic malignancies

Uveal melanomas:

Uveal melanomas Primary acquired malignant neoplasm of uveal melanocytes Arises from neuroectodermal melanocytes Most common primary intraocular neoplasm of adults Hematogenous metastasis mostly to liver Fatal in large no. of cases Choroidal melanoma more common Iris melanoma substantially less malignant than ciliary body/ choroidal melanomas

Uveal melanomas:

Uveal melanomas Epidemiology More common in white races Very rare before 30 years of age More common in elderly, increase in frequency with each decade of life Slightly more common in women Intense, recurrent sunlight exposure, dermatologic conditions, dysplastic nevus syndrome & congenital ocular hyperpigmentation – known risk factors

Uveal melanomas:

Uveal melanomas Epidemiology (contd.) No association with reproductive factors, estrogen therapy & cigarette smoking No strong familial inheritance pattern Chromosomal abnormalities seen in some cases

Choroidal melanomas:

Choroidal melanomas Ocular manifestations Asymptomatic – majority Symptoms – blurred vision, visual field defects, flashes & floaters No pain – generally Pain in late stages due to secondary glaucoma/ spontaneous tumor necrosis Dark brown to golden solid tumor Biconvex/ mushroom shaped

Choroidal melanomas:

Choroidal melanomas Ocular manifestations Darkly melanocytic melanomas show prominent clumps of orange pigment on surface Commonly associated with secondary non rhegmatogenous ( exudative) retinal detachment with clear & often shifting subretinal fluid Hemorrhage in SRF Vitreous hemorrhage Indentation of the lens in equatorial region

Fundus in Choroidal Melanoma:

Fundus in Choroidal Melanoma

Fundus - Choroidal Melanoma :

Fundus - Choroidal Melanoma

Malignant Melanoma With R.D.:

Malignant Melanoma With R.D.

(Differential diagnosis):

(Differential diagnosis) Choroidal nevus Metastatic carcinoma Disciform lesion (central/ peripheral) Subretinal/ sub RPE hematoma Localized suprachoroidal hematoma Circumscribed choroidal hemangioma Nodular posterior scleritis Choroidal osteoma

Differential diagnosis:

Differential diagnosis Congenital hypertrophy of RPE Reactive hyperplasia of pigment epithelium Syndrome of bilateral diffuse uveal melanocytic proliferation associated with systemic carcinoma Massive gliosis Ocular melanocytosis

Pathology of Melanoma :

Pathology of Melanoma

Spindle Type - A:

Spindle Type - A Cohesive cells Spindled nuclei – dark central stripes No distinct nucleoli Indistinct cytoplasm Cell border not easily identified Mitotic figures extremely rare Not more than 5% spindle B cells 92% survival rate

Malignant melanoma:

Malignant melanoma

Spindle Type – B & fascicular:

Spindle Type – B & fascicular Cohesive cells prominent spindled nuclei Distinct nucleoli Indistinct cytoplasm Cell border not easily identified Decreased pleomorphism & nuclear area as compared to epithelioid cells Not more than 5% spindle A cells & no epitheloid cells Mitotic figures rare 75% survival rate

Epithelioid Melanoma:

Epithelioid Melanoma Non cohesive cells Large round nuclei Prominent nucleoli Abundant eosinophilic cytoplasm Marked pleomorphism & nuclear area Mitotic figures common Survival 28%

Mixed Cell Melanoma:

Mixed Cell Melanoma

Pathology of Melanoma:

Pathology of Melanoma Necrotic – cell type not defined, indicates tumor immunity, better prognosis

Necrotic Uveal Melanoma :

Necrotic Uveal Melanoma

Pathology of Melanoma:

Pathology of Melanoma Prognostic markers Cell type Fibrovascular loops & network Cytomorphic parameters ( m. nuclear diameter, m. nuclear area, standard deviation of nuclear area, standard deviation of nucleolar area) Presence of scleral invasion Transscleral tumor extension Involvement of ciliary body Higher mitotic index Greater level of pigmentation

Ancillary Testing:

Ancillary Testing Ultrasonography A, B scan and color doppler Fluorescein angiography Indocyanine green angio/ videography CT scan MRI scanning P-32 test Biopsy Baseline systemic evaluation

Treatment :

Treatment Enucleation Radiation therapy ruthenium-106, iodine-125, proton beam irradiation, Photocoagulation Noncoagulative laser therapy Microsurgical resection Exentration Hyperthermia therapy Photodynamic therapy Cryotherapy Chemotherapy Multimodal therapy

Enucleation :

Enucleation More commonly employed Aggressive treatment to rid the body of cancer Longest practiced treatment option Simplest & least expensive No convincing evidence that it improves survival prognosis compared with no treatment at all, enucleation may actually worsen a patients survival prognosis

Enucleation :

Enucleation Enucleation strongly indicated painful blind eye, extremely large intraocular tumor, tumor that surrounds or invades optic disc For such cases or with clinically detected metastasis pre-enucleation radiation therapy (20 Gy in five fractions of 4Gy over 5-7 days immediately before enucleation) as ‘adjuvant therapy’ to reduce risk of postoperative orbital recurrence & improve patient survival – no substantial improvement noticed in clinical trials

Enucleation:

Enucleation Enucleation is curative if no metastasis before or at the time of enucleation Microscopic metastasis can not be detected reliably by current available methods Failure of of baseline medical tests to show metastatic disease before enucleation does not guarantee metastasis will not develop in future Approximately one-half of all patients die of metastasis

Radiation Therapy:

Radiation Therapy Most commonly employed method today Used as 1. plaque radiotherapy- delivers 80-100Gy to the apex of tumor in 3-7 days 2. Particle (proton) beam radiotherapy- less widely available,50-70Gy delivered in 4-5 sittings 0f 30-90 seconds Useful if tumor <15mm at greatest diameter & <8mm in height, 3mm or more from fovea /optic disc Majority of eyes retain / improve vision

Radiation Therapy:

Radiation Therapy Some patients have reduction in vision Side effects include – radiation retinopathy, optic papillopathy, cataract, & neovascular glaucoma

Observation:

Observation In doubtful cases of nevus / melanoma Terminally ill cases where even surgery under LA is contraindicated Highly risky in other cases

Photocoagulation:

Photocoagulation High intensity light for destroying the melanoma For tumor less than 7mm wide & 3 mm high Substantial vision retained if away from disc & macula vice versa otherwise

Non-coagulative Laser Therapy:

Non-coagulative Laser Therapy Also called transpupillary thermo therapy (TTT) Low power long duration laser used to produce heating of the tumor (40 – 60 o C) Generally diode laser used

Microsurgical resection:

Microsurgical resection Only for selected cases Trans-scleral or trans vitreal Complications include – massive IO hemorrhage, choroidal & retinal detachment, phthiasis About ½ eyes lost due to surgery Best for young otherwise healthy individuals with thick peripheral tumor

Exentration :

Exentration Useful for massive extrascleral extension/ orbital recurrence No significant improvement in survival Generally avoided in favor of radiotherapy

Hyperthermia :

Hyperthermia Increase the temperature inside the tumor to induce cell death

PDT, Cryotherapy, Chemotherapy:

PDT, Cryotherapy, Chemotherapy In various stages of trial Not very effective alone

Multimodal therapy:

Multimodal therapy Combination of various treatment modes

METASTATIC TUMORS:

METASTATIC TUMORS Primary extraophthalmic neoplasm that spreads hematogenously to the eye – most common intraocular neoplasm Typically appear as relatively thin, amelanotic, round to oval choroidal mass Unifocal & unilateral in 80% cases Multifocal/ bilateral/ both in 20% cases Usually associated with clinically evident extraophthalmic neoplasm & other metastatic foci Nonrhegmatogenous retinal detachment surrounding & overlying the tumor Generally carry poor prognosis for life

Metastatic tumors:

Metastatic tumors Presenting symptoms include blurred/distorted vision Pain usually absent, present in cases with extensive intraocular tumor, retinal detachment & secondary glaucoma Primary carcinomas generally from breast, lung,gastrointestinal tract, skin, renal cell carcinoma May also present as hypopyon, hyphema & vitreous cellular infiltration

Ancillary testing:

Ancillary testing FFA ICG Ultrasonography CT scan MRI Fine needle aspiration biopsy

Differential diagnosis:

Differential diagnosis Amelanotic nevus Amelanotic malignant melanoma Circumscribed choroidal hemangioma Choroidal osteoma Primary intraocular lymphoma Astrocytoma of retina Idiopathic sclerochoroidal calcification Bullous CSR Uveal effusion syndrome Vitelliform dystrophy Posterior scleritis Granuloma ( TB, sarcoid )

Treatment :

Treatment Radiotherapy Chemotherapy Hormonal therapy

Visual Prognosis :

Visual Prognosis No. & size of intraocular tumor/s Location in relation to disc & fovea Effect on retina Response to radiation/ chemotherapy Site & pathological nature of original tumor Ocular side effects of therapy