Intraocular tumors. Zsuzsa Récsán

Transcription

1 Intraocular tumors Zsuzsa Récsán

2 Definition Uveal melanoma Primary acquired malignant neoplasm of uveal melanocytes Epidemiology: most common primary mal tu in adults Much more common in lighter-skinned races: 1 in Caucasian individuals Cumulative lifetime incidence ys: 2.5/1 million inhabitants/ year above the age of 65 ys: 25/ 1 million inhabitants/ year

3 Uveal melanoma: Key features Dark brown/golden tumor of choroid, CB, iris Size at the time of diagnosis choroidal/ CB tumors: BD 7 mm P 2 mm; iris tumors: much smaller Unilateral, unifocal Well-established tendency to metastatize, liver, bone, lung Much more common in lighter skinned patients

4 80% of all uveal melanomas 60% within 3mm of the optic disc Grey-brown/ amelanotic Orange pigment (lipofuscin) Non-rhegmatogeneous retinal detachment (shifting) Choroidal melanoma

5 Choroidal melanoma, symptoms No symptom Visual disturbances Blurry vision (retinal detachment, tu on post pole vitreous hemorrhage) Visual field defect (retinal detacment, tumor) Floaters (vitreous hemorrhage) Photopsia) Pain (glauc sec)

6 Growing, spread Vitreous body Bruch membrane Retina Orbita Vortex vein Sclera: direct usuratio, cannels: vessels, nerves N. opticus

7 Pathologia, Callender s classification Spindle cells: 45%, fascicularis Epitheloid cells: 5% Mixed: 45% Necrotic form: 5%,

8 Prognosis Histopathology: epitheloid cell ly infiltráció vascular loops high mytotic activity Above the age 56 ys DM man Peripheral to the equator Tu basal diameter 10mm (diffuse/ focale tu) Genetics: somatic mutations in the tumor suppressor gene BAP1: greater chance for metastasis

9 Therapeutic options Enucleation Radiation (rhutenium, proton) Microsurgical resection Laser coagulation Thermotherapy Cryocoagulation Exenteration Chemotherapy

10 Corpus ciliare melanoma growing ring melanoma Extraocular spread episcleralis pigmentation Sentinel vessels Anterior chamber sec glauc Lens: dislocation, lenticular astigmatism, cataracta (patient: often change of spectacles)

11 Symptoms and signs Visual disturbances blurry vision Change spectales Visual field defect Red eye Pain Pigmented/ amelanotic cataracta, subluxatio Serosus retina detachment Invasion into anterior chamber Extraocular spread Sentinel vessels

12 Iris melanoma Cosmetic problem Visual problem (cataract) Pain(glauc sec) Color: vascularization, pigmentation Surface: smooth/ irreg Localization: lower quadrants Complications Ectropion uveae rig-like spread, glauc sec spread to ciliary body Attacment winth corneal endothelium: edema cyst on pupillary margin (rare)

13 Treatment Observation (photo!) laser, cryopexia Brachytherapia rhuthenium-106 (P: 5mm), jód-125 (P:8-10mm) Proton/ electron beam irradion Surgery Chorioidectomia, iridocyclectomia, iridectomia Endoresectio Enucleatio Exenteratio

14 Differential diagnosis: Pseudomelanoms met cc Choroidal haemangioma Subretinal haemorrhage osteoma congen RPE hyperplasia Melanocytoma Macraneurysma hamartoma

15 Metastatic carcinoma to the eye Clinically evident extraocular tumor is capable of metastatizing Abruptly developing and expanding within various intraocular tissues (choroid!) 80% unifocal-monocular; 20% multifocal-binocular Hematologic spread Poor survival rate after diagnosis

16 Symptoms, signs Visual disturbance: blurred and distorted vision Generally no pain Appearence:?primary tumor eg: breast cancer met: golden brown Cutaneous mal tu met: dark brown Renal cell cc met: orange Non-rhegmatogeneous retinal detachment: quick shift Rapid growing

17 Metastatic carcinoma Treatment options:?primary tumor Systemic chemotherapy Hormone therapy Radiation therapy External beam Placque radiotherapy: iodine-125, ruthenium106 enucleation

18 Retinoblastoma Most common primary malignant neoplasm in childhood 1/18000 living birth) Origin: immature retinal cells endo/exophytic growth Diagnosis: 1-3 ys Bilateral: 1. y unilateral: 2. ys Unilateral (unifocal):60-70% Bilateral (multifocal): 30-40% Germinal (heritable, autosome dominant pattern) form: 40%; Somatic (nonheritable ) form:60% Loss or inactivation of Rb gene (tumor suppressor gene on the long arm of chr 13)

19 Symptoms White reflex 56% Strabismus20% Glaucoma7% Poor vison 5% Rutin examination 3% Orbital cellulitis3% Unilateralis mydriasis2% Heterochromia iridis1% Hyphema1% Other2%

20 Diagnosis Case/family history General anaesth fundus UH, CT: calcification MR: intraocular appearence, extraocul spread, ectopic intracranial retinoblastoma (corpus pineale, spinal chord)

21 Differential diagnosis Leucocoria Coats disease Primer perzistant hyperplastic vitreous Toxocariasis ROP st 5 Familiar exudative vitreoretinopathy Incontinentia pigmenti Norrie s disease Vitreous spread Pars planitis Endophthalmitis Leukemic infiltration Discret retina tu Chorioretinal toxocara granuloma Fibrae medullares Retinal haemangioma Astrocytoma medulloepithelioma

22 Rb: therapeutic options Intravenous/arterial chemotherapy Intravitreal chemotherapy Enucleation Radiation therapy Photocoagulation or laser Cryoptherapy Observation

23 Treatment small tu (P: 1.5mm, basal diameter 3-4mm): TTT, cryopexia, chemotherapia (intravitreal/ systemic) Medium tu (P: 6mm, BM 12mm): kemoreduction; brachytherapia+chemo, Large tumor: chemotherapia, enucleatio Extraocularis spread: outer beam irradiaton, chemoreduction+enucleatio Family care

24 Retinoblastoma secunder malignancies Osteogenic sarcoma (most common) Fibrosarcoma Rhabdomyosarcoma Chondrosarcoma Spindle cell sarcoma Neuroblastoma Glioma Leukemia Sebaceous gland and planocellular cc Malignus melanoma