review course in ocular oncology

Review Course inReview Course in

Ocular OncologyOcular Oncology

Hatem Krema, MD, MSc, FRCSEdHatem Krema, MD, MSc, FRCSEdConsultant in Ocular Oncology Consultant in Ocular Oncology

Cairo – EgyptCairo – Egypt

I - Tumors of the EyelidsI - Tumors of the Eyelids

Malignant or BenignMalignant or Benign ? ?

1- First Clinical Impression !1- First Clinical Impression !2- Involvement of other layers.2- Involvement of other layers.3- Growth Rate.3- Growth Rate.4- Surface.4- Surface.5- Cilia at eyelid margin.5- Cilia at eyelid margin.6- Previous treatment, Recurrence.6- Previous treatment, Recurrence.7- Biopsy.7- Biopsy.

I – Eyelid Epidermal TumorsI – Eyelid Epidermal Tumors

1-Squamous Papilloma.1-Squamous Papilloma.2-Seborrheic Keratosis.2-Seborrheic Keratosis.3-Inverted Follicular Keratosis.3-Inverted Follicular Keratosis.4-Cutaneous Horn.4-Cutaneous Horn.5-Keratacanthoma.5-Keratacanthoma.

Benign Epidermal TumorsBenign Epidermal Tumors

Malignant Epidermal TumorsMalignant Epidermal Tumors

1- Basal Cell Carcinoma.1- Basal Cell Carcinoma.

2- Squamous Cell Carcinoma2- Squamous Cell Carcinoma..

Epidermal Tumors : Epidermal Tumors : Benign LesionsBenign Lesions

Squamous PapillomaSquamous Papilloma))SessileSessile((

Seborrheic KeratosisSeborrheic Keratosis Cutaneous HornCutaneous Horn

KeratoacanthomaKeratoacanthomaInverted FollicularInverted FollicularKeratosisKeratosis

Squamous PapillomaSquamous Papilloma)Pedunculated()Pedunculated(

Epidermal Tumors : Epidermal Tumors : Basal Cell CarcinomaBasal Cell Carcinoma

- - Its four morphological types are the Its four morphological types are the Nodular, Ulcerative, Pigmented Nodular, Ulcerative, Pigmented & &

Morpheaform Morpheaform tumor. Rarely, tumor. Rarely, CysticCystic or or Multicenteric.Multicenteric.

- It mostly involves the lower eyelid. More likely to affect - It mostly involves the lower eyelid. More likely to affect fair skinnedfair skinned persons persons with high with high solar exposure.solar exposure.

- - Tumors at the Tumors at the medial canthusmedial canthus warrant a special attention. warrant a special attention.

Pigmented Nodular Pigmented Nodular BCCBCC

Rodent UlcerRodent Ulcer Morpheaform BCCMorpheaform BCC

Epidermal Tumors : Epidermal Tumors : BCC SyndromesBCC Syndromes

Gorlin- Goltz Syndrome:Gorlin- Goltz Syndrome: A.D., multiple BCC, palmer dyskeratosis, bifid ribs, A.D., multiple BCC, palmer dyskeratosis, bifid ribs, odontogenic keratotic cysts and ectopic calcifications.odontogenic keratotic cysts and ectopic calcifications.

Xerodermia Pigmentosa:Xerodermia Pigmentosa: A.R., defective DNA repair mechanisms multiple A.R., defective DNA repair mechanisms multiple cancers in areas exposed to normal dose of UV rays.cancers in areas exposed to normal dose of UV rays.

Organoid Nevus Syndrome:Organoid Nevus Syndrome: Oculo-Neuro-Cutaneous syndrome, skin nodules, Oculo-Neuro-Cutaneous syndrome, skin nodules, parietal alopecia, linear pigmentation, multiple BCCparietal alopecia, linear pigmentation, multiple BCC..

Bazex Syndrome:Bazex Syndrome: A.D., Multiple facial BCCs with typical atrophic dermal A.D., Multiple facial BCCs with typical atrophic dermal changes on the extremities “Ice-pick marks”.changes on the extremities “Ice-pick marks”.

Gorlin-Goltz Gorlin-Goltz SyndromeSyndrome

Xerodermia Xerodermia PigmentosaPigmentosa

Organoid NevusOrganoid NevusSyndromeSyndrome

Epidermal Tumors : Epidermal Tumors : GGrowth Rate & Mortalityrowth Rate & Mortality

EarlyEarly Squamous Cell CarcinomaSquamous Cell Carcinoma

Extensive Neglected Extensive Neglected Basal Cell CarcinomaBasal Cell Carcinoma

II – Eyelid Melanocytic TumorsII – Eyelid Melanocytic Tumors

1- Eyelid melanosis.1- Eyelid melanosis.2- Eyelid melanocytic nevus2- Eyelid melanocytic nevus3- Eyelid primary malignant melanoma3- Eyelid primary malignant melanoma

Melanocytic Tumors:Melanocytic Tumors: ExamplesExamples

Junctional NevusJunctional Nevus

Compound NevusCompound Nevus Nodular MelanomaNodular Melanoma

CongenitalCongenital “ “Kissing” NevusKissing” Nevus

Oculodermal Oculodermal MelanosisMelanosis

Dermal &Dermal &Compound NeviCompound Nevi

III – Eyelid Sebaceous TumorsIII – Eyelid Sebaceous Tumors

11- - Sebaceous cystSebaceous cyst..

22- - Sebaceous adenomaSebaceous adenoma..

33- - Sebaceous gland carcinomaSebaceous gland carcinoma..

Sebaceous Tumors:Sebaceous Tumors: Benign & MalignantBenign & Malignant

Sebaceous Adenoma: Sebaceous Adenoma: Lobulated mass, commonly of Meibomian origin. It carries Lobulated mass, commonly of Meibomian origin. It carries

no malignant potential.no malignant potential.

Sebaceous gland Cacinoma: Sebaceous gland Cacinoma: Meibomian, Zeis or carancle originMeibomian, Zeis or carancle origin

Clinically, Circumscribed mass Clinically, Circumscribed mass or or Diffuse intraepithelial spread involving Diffuse intraepithelial spread involving

conjunctiva, requires map biopsy. conjunctiva, requires map biopsy.

Muir Torre Syndrome:Muir Torre Syndrome: A.D., Sebaceous tumor & visceral neoplasm A.D., Sebaceous tumor & visceral neoplasm

Sebaceous cystSebaceous cyst Sebaceous AdenomaSebaceous Adenoma Sebaceous gland carcinomaSebaceous gland carcinoma

IV – Eyelid Appendages TumorsIV – Eyelid Appendages Tumors

1-Trichoepithelioma.1-Trichoepithelioma.2-Trichofolliculoma.2-Trichofolliculoma.3-Ticholemmoma.3-Ticholemmoma.4-Pilomatrixoma.4-Pilomatrixoma.

1-Hidrocystoma )Eccrine & Apocrine(.1-Hidrocystoma )Eccrine & Apocrine(.2-Syringoma.2-Syringoma.3-Eccrine acrospiroma.3-Eccrine acrospiroma.4-Pleomorphic adenoma of sweat gland.4-Pleomorphic adenoma of sweat gland.5-Adenocarcinoma of the sweat gland.5-Adenocarcinoma of the sweat gland.

Sweat Gland TumorsSweat Gland Tumors

Hair Follicle TumorsHair Follicle Tumors

Appendages Tumors:Appendages Tumors: ExamplesExamples

Subcutaneous Subcutaneous conjunctival conjunctival retention cystretention cyst

SyringomaSyringoma

Bluish Eccrine Bluish Eccrine HidrocystomaHidrocystoma

Multiple Eccrine Multiple Eccrine HidrocystomaHidrocystoma

V – Eyelid Neural TumorsV – Eyelid Neural Tumors

11- - NeurofibromaNeurofibroma..

22- - SchwannomaSchwannoma..

33- - Merkel cell tumorMerkel cell tumor..

Neural Tumors : Neural Tumors : NeurofibromaNeurofibroma

It is proliferation of It is proliferation of Axons, Schwann cells & endoneural fibroblasts.Axons, Schwann cells & endoneural fibroblasts.

1- Plexiform Neurofibroma1- Plexiform Neurofibroma : : Pathognomonic NF1, tends to recur.Pathognomonic NF1, tends to recur.

2- Solitary Neurofibroma2- Solitary Neurofibroma : : Not associated with NF1Not associated with NF1

3- Multiple Neurofibromata3- Multiple Neurofibromata : : Mostly associated with NF1Mostly associated with NF1

CafCafé au laité au laitPatchesPatches

Absent greater wing Absent greater wing of Sphenoidof Sphenoid

Plexiform Plexiform NeurofibromaNeurofibroma

Neural Tumors : Neural Tumors : SchwannomaSchwannoma

- It is proliferation ofIt is proliferation of Schwann cells )Neurilemoma(. Schwann cells )Neurilemoma(.

- It can be multiple in association with NF1 It can be multiple in association with NF1 oror a solitary lesion. a solitary lesion.

- Encapsulted with perineural capsule.Encapsulted with perineural capsule.

- Aggressive recurrence if incompletely excised.Aggressive recurrence if incompletely excised.

VI – Eyelid Vascular TumorsVI – Eyelid Vascular Tumors

1.1. Congenital capillary hemangiomaCongenital capillary hemangioma

2.2. Acquired capillary hemangiomaAcquired capillary hemangioma

3.3. Cavernous hemangiomaCavernous hemangioma

4.4. LymphangiomaLymphangioma

5.5. Diffuse Angioma )Nevus Flammeus(Diffuse Angioma )Nevus Flammeus(

6.6. Vascular malformationsVascular malformations

7.7. HemangioendotheliomaHemangioendothelioma

8.8. Kaposi’s sarcoma.Kaposi’s sarcoma.

Vascular Tumors: Vascular Tumors: Congenital Capillary HemangiomaCongenital Capillary Hemangioma

- Cutaneous, Subcutaneous, Diffuse or Orbital.- Cutaneous, Subcutaneous, Diffuse or Orbital.

- Involution in 50% by fifth year, 70% by seventh year.- Involution in 50% by fifth year, 70% by seventh year.

- Early interference if strabismus or occlusion – induced amblyopia.- Early interference if strabismus or occlusion – induced amblyopia.

- Associations: . - Associations: . Sublingual or nasopharyngeal hemangioma.Sublingual or nasopharyngeal hemangioma.

. . Kassabach – Meritt Syndrome.Kassabach – Meritt Syndrome.

CutaneousCutaneousCapillary HemangiomaCapillary Hemangioma

SubcutaneousSubcutaneousCapillary HemangiomaCapillary Hemangioma

DiffuseDiffuseCapillary HemangiomaCapillary Hemangioma

- Multiple small cysts filled with clear fluid Multiple small cysts filled with clear fluid

and blood.and blood.

- Eyelid affection is mostly associated with Eyelid affection is mostly associated with

conjunctival lymphangioma.conjunctival lymphangioma.

- Bouts of engorgement with hemorrhage or Bouts of engorgement with hemorrhage or

local infections.local infections.

- Associated with buccal or labial Associated with buccal or labial

lymphangioma.lymphangioma.

- Shows no involution with age and Poor Shows no involution with age and Poor

response to steroids.response to steroids.

Vascular Tumors: Vascular Tumors: LymphangiomaLymphangioma

Vascular Tumors: Vascular Tumors: Hemangiomas & MalformationsHemangiomas & Malformations

Acquired Capillary Acquired Capillary hemangiomahemangioma

Cavernous Cavernous hemangiomahemangioma

Arteriovenous Arteriovenous MalformationMalformation

Racemose AngiomaRacemose Angioma

Nevus Nevus FlammeusFlammeus

Non- Surgical Management Non- Surgical Management of Eyelid Tumorsof Eyelid Tumors

Eyelid Tumors:Eyelid Tumors: Non- Surgical ManagementNon- Surgical Management

1- Cryotherapy 1- Cryotherapy )Not for Extensive neoplasm, Cryoglobulinimia()Not for Extensive neoplasm, Cryoglobulinimia(

2- Steroids: 2- Steroids: Topical, Lesional & Systemic.Topical, Lesional & Systemic.

3- Interferon alpha-2a3- Interferon alpha-2a

4- Radiotherapy 4- Radiotherapy )Not for Sebaceous Ca. & Xerodermia P.()Not for Sebaceous Ca. & Xerodermia P.(

5- Chemotherapy: 5- Chemotherapy: )Not for Skin Melanoma()Not for Skin Melanoma(

6- Sclerosing agents.6- Sclerosing agents.

7- Immunotherapy: 7- Immunotherapy: Imiquimod 5%Imiquimod 5%

Principles inPrinciples inSurgical Management Surgical Management

of Eyelid Tumorsof Eyelid Tumors

Excision of a subcutaneous Excision of a subcutaneous CircumscribedCircumscribed Mass Mass

Surgical Management: Surgical Management: Incision & Excision BiopsyIncision & Excision Biopsy

Diffuse Lesions:Diffuse Lesions:

- Incision Biopsy.Incision Biopsy.

- Punch Biopsy.Punch Biopsy.

Circumscribed Lesions:Circumscribed Lesions:

- Excision Biopsy.Excision Biopsy.

- Shave Biopsy.Shave Biopsy.

Surgical Management: Surgical Management: Tumor Margin ControlTumor Margin Control))Frozen Section, Mohs’ surgery & Safety Margin(Frozen Section, Mohs’ surgery & Safety Margin(

Frozen SectionFrozen Section)Perpendicular slices()Perpendicular slices(

Mohs’ surgeryMohs’ surgery))Tangential slicesTangential slices((

Safety MarginSafety Margin

Pentagon Excision & Reese Lateral CanthotomyPentagon Excision & Reese Lateral CanthotomyFor up to 40% eyelid margin defectFor up to 40% eyelid margin defect

Flaps & GraftsFlaps & Grafts

Tenzel Rotational FlapTenzel Rotational Flap

Post-auricular free skin graftPost-auricular free skin graft

Management of a large Management of a large Medial CanthalMedial Canthal Basal Cell Carcinoma Basal Cell Carcinoma

Excision and Reconstruction planExcision and Reconstruction plan

Excision of the TumorExcision of the Tumor

Dissection of Mustardé Rotational FlapDissection of Mustardé Rotational Flap

Glabellar Flap and Transnasal WiringGlabellar Flap and Transnasal Wiring

Implantation of a Free Tarso-Conjunctival GraftImplantation of a Free Tarso-Conjunctival Graft

Appearance at the End of SurgeryAppearance at the End of Surgery

One Week Post OperativeOne Week Post Operative

II - Tumors of the conjunctivaII - Tumors of the conjunctiva

II- Conjunctiva:II- Conjunctiva: Benign Tumors Benign Tumors

Benign amelanotic masses include Benign amelanotic masses include squamous squamous

cell papilloma, conjunctival granuloma and cell papilloma, conjunctival granuloma and

amelanotic nevus.amelanotic nevus.

Clinical experience with their various Clinical experience with their various

presentations helps to avoid unnecessary presentations helps to avoid unnecessary

aggressive interference.aggressive interference.

Limbal dermoid is Limbal dermoid is a choriostoma.a choriostoma.

If it has visual or cosmetic symptoms, excision If it has visual or cosmetic symptoms, excision

with partial lamellar corneal grafting then with partial lamellar corneal grafting then

may be needed.may be needed.

II- Conjunctiva:II- Conjunctiva: Melanocytic Lesions Melanocytic Lesions

Pigmented melanocytic lesions involving the conjunctiva includePigmented melanocytic lesions involving the conjunctiva include

melanocytic nevusmelanocytic nevus that mostly does not show malignant changes. Also, that mostly does not show malignant changes. Also, primary primary

acquired melanosis )PAM(acquired melanosis )PAM( with possible malignant potentials and with possible malignant potentials and conjunctival conjunctival

malignant melanoma.malignant melanoma.

Knowing of the malignant potentials or metastases, and the final cosmetic Knowing of the malignant potentials or metastases, and the final cosmetic

outcome, determines the extent of surgical management.outcome, determines the extent of surgical management.

II- Conjunctiva:II- Conjunctiva: Malignant Tumors Malignant Tumors

SSquamous cell carcinomaquamous cell carcinoma is the most is the most

common primary malignant tumor of common primary malignant tumor of

conjunctiva, manifests usually as a fleshy conjunctiva, manifests usually as a fleshy

vascularized mass at the limbus.vascularized mass at the limbus.

Lymphocytic proliferative disordersLymphocytic proliferative disorders can can

involve the conjunctiva as a involve the conjunctiva as a “salmon patch”. “salmon patch”.

It is essential here to exclude other systemic It is essential here to exclude other systemic

or ocular tissue involvement before deciding or ocular tissue involvement before deciding

the full treatment plan.the full treatment plan.

II- Conjunctiva:II- Conjunctiva: Surgical Management Surgical Management

Alcohol epitheliectomy with lamellar Alcohol epitheliectomy with lamellar

keratosclerectomykeratosclerectomy is the standard procedure is the standard procedure

for malignant tumors at the limbus.for malignant tumors at the limbus.

Conjunctival tumors are treated by complete Conjunctival tumors are treated by complete

excision biopsy with excision biopsy with frozen section controlfrozen section control of of

tumor margins and tumor margins and cryotherapycryotherapy of the tumor of the tumor

bed.bed.

Reconstruction can be done then by Reconstruction can be done then by simple simple

closure,closure, conjunctival grafting conjunctival grafting oror amniotic amniotic

membrane membrane transplant.transplant.

II- Conjunctiva:II- Conjunctiva: Non-Surgical Options Non-Surgical Options

Management of conjunctival neoplasia may Management of conjunctival neoplasia may

require the primary or adjunctive use of local require the primary or adjunctive use of local

treatment with some treatment with some chemotherapeutic agents chemotherapeutic agents

such as Mitomycin-C, 5- Flourouracil and such as Mitomycin-C, 5- Flourouracil and

Interferon alpha 2-b.Interferon alpha 2-b.

In some diffuse radiosensitive tumors as In some diffuse radiosensitive tumors as

lymphoma, lymphoma, fractionated external beam fractionated external beam

radiotherapyradiotherapy or application of a or application of a radioactive radioactive

plaqueplaque may be employed. may be employed.

III – Intraocular TumorsIII – Intraocular Tumors

III- Intraocular:III- Intraocular: Iris Tumors Iris Tumors

Iris masses could be melanocytic as Iris masses could be melanocytic as

nevus and melanoma.nevus and melanoma.

They also include inflammatory masses They also include inflammatory masses

as as granulomagranuloma, vascular as , vascular as hemangioma, hemangioma,

muscular as muscular as leiomyoma,leiomyoma, infiltrative infiltrative

such as such as lymphoma, metastases,lymphoma, metastases, and and

extension from a ciliary body tumor.extension from a ciliary body tumor.

III- Intraocular:III- Intraocular: Pigmented Tumors Pigmented Tumors

Choroidal melanomaChoroidal melanoma is the most common intraocualr tumor in adults. is the most common intraocualr tumor in adults.

Clinical examination is the method of choice to diagnose choroidal melanoma Clinical examination is the method of choice to diagnose choroidal melanoma

and to differentiate a and to differentiate a choroidal nevuschoroidal nevus from a small choroidal melanoma. from a small choroidal melanoma.

Other common intraocular pigmented tumors include optic nerve head Other common intraocular pigmented tumors include optic nerve head

melanocytomamelanocytoma, retinal pigment epithelium , retinal pigment epithelium adenomaadenoma and and combined combined

hamartomahamartoma of retina and retinal pigment epithelium. of retina and retinal pigment epithelium.

III- Intraocular:III- Intraocular: Non-pigmented TumorsNon-pigmented Tumors

Choroidal non-pigmented masses include Choroidal non-pigmented masses include amelanotic melanoma,amelanotic melanoma, uveal uveal

granuloma, lymphoma, osteomagranuloma, lymphoma, osteoma and and choroidal metastaseschoroidal metastases..

Choroidal hemangiomaChoroidal hemangioma has a characteristic orange color and is usually has a characteristic orange color and is usually

associated with sub-retinal fluid transudation.associated with sub-retinal fluid transudation.

Retinal non-pigmented masses include Retinal non-pigmented masses include retinoblastoma, astrocytoma,retinoblastoma, astrocytoma, and and

toxocara granuloma.toxocara granuloma.

III- Intraocular:III- Intraocular: Retinoblastoma Retinoblastoma

Retinoblastoma is the most common Retinoblastoma is the most common

pediatric intraocular malignancy,pediatric intraocular malignancy,

presents mostly with leukocoria or presents mostly with leukocoria or

strabismus. strabismus.

Retinoblastoma is Retinoblastoma is bilateral in 33%bilateral in 33% of of

patient and can be multifocal. It may show patient and can be multifocal. It may show

tendency to tendency to vitreous seedingvitreous seeding and and

extraocular extraocular spread along the optic nerve.spread along the optic nerve.

Management requires a great deal of Management requires a great deal of

experience with the potentials of the experience with the potentials of the new new

treatment optionstreatment options available and with available and with

accurate regimen for accurate regimen for follow-up.follow-up.

III- Intraocular:III- Intraocular: Investigations Investigations

Help to Help to differentiatedifferentiate some some

tumors from other tumor-tumors from other tumor-

simulating masses. simulating masses.

Basic for precise measurement of Basic for precise measurement of

tumor dimension, tumor dimension, and accurate and accurate

follow-up.follow-up.

They include They include IntraocularIntraocular

Transillumination, RetcamTransillumination, Retcam

Ultrasonography,Ultrasonography, Fundus Fundus

Photography and AngiographyPhotography and Angiography

and and Doppler UltrasonographyDoppler Ultrasonography. .

1- Tumor Resection.1- Tumor Resection.

2- Radioactive Plaques.2- Radioactive Plaques.

3- Thermotherapy.3- Thermotherapy.

4- Cryotherapy.4- Cryotherapy.

5- Chemotherapy.5- Chemotherapy.

6- External beam radiotherapy.6- External beam radiotherapy.

7- Enucleation.7- Enucleation.

Methods of TreatmentMethods of Treatment

Treatment :Treatment : I- Tumor Resection I- Tumor Resection

Local resectionLocal resection of unifocal intraocular tumors, with preservation of the eye, is of unifocal intraocular tumors, with preservation of the eye, is

advocated foradvocated for small to medium small to medium sized sized tumors of the iris, ciliary body and tumors of the iris, ciliary body and

peripheral choroid.peripheral choroid.

Treatment :Treatment : I- Resection surgery I- Resection surgery

Treatment :Treatment : II- Radioactive Plaque II- Radioactive Plaque

The treatment of choice for most The treatment of choice for most mediummedium sized intraocular neoplasms. The sized intraocular neoplasms. The

radioactive isotopes currently used in manufacturing plaques areradioactive isotopes currently used in manufacturing plaques are

RutheniumRuthenium106 106 and and IodineIodine125125..

DosimetryDosimetry, is calculated by a sophisticated computer, is calculated by a sophisticated computer software.software.

Plaque application:Plaque application: DosimetryDosimetry

Plaque application:Plaque application: PeriotomyPeriotomy

Plaque application:Plaque application: Bridle suturesBridle sutures

Plaque application:Plaque application: Transillumination Transillumination

Plaque application:Plaque application: Dummy plaque Dummy plaque

Plaque application:Plaque application: Preplaced sutures Preplaced sutures

Plaque application:Plaque application: Radioactive fixation Radioactive fixation

Plaque application:Plaque application: Radioactive plaque in place Radioactive plaque in place

Plaque application:Plaque application: Resuturing conjunctiva Resuturing conjunctiva

Plaque application:Plaque application: Treatment Outcome Treatment Outcome

Before Treatment After Treatment

Treatment:Treatment: III- Transpupillary Thermotherapy III- Transpupillary Thermotherapy

Transpupillary ThermotherapyTranspupillary Thermotherapy is using a modified large spot is using a modified large spot infrared laserinfrared laser to to

elevate temperature of tumor cells up to 60elevate temperature of tumor cells up to 60 oo C, enough to cause damage to C, enough to cause damage to

malignant cells.malignant cells.

It is useful for It is useful for small central tumorssmall central tumors in the fundus with the least damage to in the fundus with the least damage to

surrounding tissuessurrounding tissues

Combined Ruthenium Plaque Irradiation with Combined Ruthenium Plaque Irradiation with

Transpupillary ThermotherapyTranspupillary Thermotherapy “ “ Sandwich Technique !”Sandwich Technique !”

TranspupillaryTranspupillaryThermotherapyThermotherapy

MelanomaMelanoma

106106Ru PlaqueRu Plaque5 mm5 mm

3.9 mm3.9 mm

Published StudyPublished Study

Arch Ophthalmol 2002; 120: 933-940Arch Ophthalmol 2002; 120: 933-940

Treatment:Treatment: IV- Cryotherapy IV- Cryotherapy

To ablate To ablate small peripheralsmall peripheral fundus tumor, by performing triple freeze-thaw fundus tumor, by performing triple freeze-thaw

cycles technique.cycles technique.

Useful in Useful in unresectableunresectable peripheral retinal tumors as peripheral retinal tumors as retinal angiomasretinal angiomas, and is , and is

very effective in multifocal peripheral very effective in multifocal peripheral retinoblastoma.retinoblastoma.

Treatment:Treatment: V- Chemotherapy V- Chemotherapy It has no role for melanoma treatment.It has no role for melanoma treatment.

Initial treatment for medium to large, and multifocal Initial treatment for medium to large, and multifocal Retinoblastoma, Retinoblastoma, and in and in somesome choroidal metastases. choroidal metastases.

For retinoblastoma:For retinoblastoma: 6 cycles6 cycles of Vincristine + Etoposide + Carboplatin of Vincristine + Etoposide + Carboplatin ++ Cyclosproin A.Cyclosproin A.

Adjunctive Cryotherpay and Thermotherapy with ChemotherapyAdjunctive Cryotherpay and Thermotherapy with Chemotherapy..

Treatment:Treatment: VI- External Beam Irradiation VI- External Beam Irradiation

For intraocular lymphoma, metastatic carcinoma and vitreous seeds in For intraocular lymphoma, metastatic carcinoma and vitreous seeds in retinoblastomaretinoblastoma..

35%35% secondary cancerssecondary cancers in irradiated region in irradiated region.. Orbital bones Orbital bones deformitydeformity..

Treatment:Treatment: VII- Enucleation VII- Enucleation

In advanced In advanced melanomamelanoma, unilateral large , unilateral large retinblastomaretinblastoma with no visual with no visual potential, failed treatment, or tumor-induced glaucoma.potential, failed treatment, or tumor-induced glaucoma.

Primary orbital implant gives excellent cosmetic outcome.Primary orbital implant gives excellent cosmetic outcome.

Enucleation:Enucleation: Procedure Procedure

Enucleation:Enucleation: Procedure Procedure

Enucleation:Enucleation: Procedure Procedure

Enucleation )OS(Enucleation )OS(

Enucleation )OSEnucleation )OS((

Enucleation )ODEnucleation )OD((

Enucleation )ODEnucleation )OD((

Enucleation:Enucleation: Treatment Outcome Treatment Outcome

IV –Tumors of the OrbitIV –Tumors of the Orbit

IV- Orbit:IV- Orbit: Circumscribed Tumors in AdultsCircumscribed Tumors in Adults

The most common circumscribed orbital The most common circumscribed orbital

tumors in adults include tumors in adults include Cavernous Cavernous

Hemangioma, Neurofibroma, orbital Hemangioma, Neurofibroma, orbital

dermoid, Fibrous Histiocytoma, dermoid, Fibrous Histiocytoma, andand

Hemangiopericytoma. Hemangiopericytoma.

The most commonThe most common lacrimal gland tumors lacrimal gland tumors

include include Pleomorphic adenomaPleomorphic adenoma and and

Adenoid Cystic Carcinoma.Adenoid Cystic Carcinoma.

CT scansCT scans are preferred here, to study are preferred here, to study

orbital orbital bone affectionbone affection and to decide the and to decide the

most suitable most suitable surgical approach.surgical approach.

IV- Orbit:IV- Orbit: Diffuse Tumors in AdultsDiffuse Tumors in Adults

The most common lesions that show as The most common lesions that show as

diffuse masses, molding around orbital diffuse masses, molding around orbital

structures, on orbital imaging include structures, on orbital imaging include

Orbital Pseudotumor, Lymphoproliferative Orbital Pseudotumor, Lymphoproliferative

tumors, Orbital Metastases, tumors, Orbital Metastases,

Granulomatous inflammations Granulomatous inflammations andand

Cellulitis.Cellulitis.

Assessment for the possible systemic Assessment for the possible systemic

associations in these cases can give clues to associations in these cases can give clues to

the natural history of the suspected mass the natural history of the suspected mass

prior to confirmation by prior to confirmation by incision biopsy. incision biopsy.

IV- Orbit:IV- Orbit: Pediatric TumorsPediatric Tumors

Pediatric primary orbital masses include Pediatric primary orbital masses include

cystic lesions as cystic lesions as dermoid cyst,dermoid cyst, vascular vascular

tumors as tumors as capillary hemangioma and capillary hemangioma and

lymphangioma, Inflammatorylymphangioma, Inflammatory lesions, lesions,

lymphocyticlymphocytic and and leukemicleukemic infiltrates, and infiltrates, and

Juvenile pilocytic astrocytomaJuvenile pilocytic astrocytoma of the optic of the optic

nerve.nerve.

RhabdomyosarcomaRhabdomyosarcoma may show rapid may show rapid

progression as orbital Cellulitis, but has an progression as orbital Cellulitis, but has an

excellent cure rate with combined excellent cure rate with combined

radiotherapy and chemotherapy.radiotherapy and chemotherapy.

Examples of Orbital SurgeriesExamples of Orbital Surgeries

1- Lacrimal Gland Swelling1- Lacrimal Gland Swelling

Initial PresentationInitial Presentation

ImagingImaging

CT )Axial view(CT )Axial view( CT )Coronal view( CT )Coronal view(

Marking skin incisionMarking skin incision

Skin crease incisionSkin crease incision

Orbital lobe exposed beneath the septumOrbital lobe exposed beneath the septum

Trans-septal orbital lobe deliveryTrans-septal orbital lobe delivery

Trans-septal orbital lobe excisionTrans-septal orbital lobe excision

Closure of the WoundClosure of the Wound

HistopathologyHistopathology

Lacrimal Gland LymphomaLacrimal Gland Lymphoma

Lacrimal gland tumors are easily approached through the orbital Lacrimal gland tumors are easily approached through the orbital septum or periosteumseptum or periosteum..

No bone surgery is needed in non-advanced casesNo bone surgery is needed in non-advanced cases

22- - Extraconal Circumscribed MassExtraconal Circumscribed Mass

Initial PresentationInitial Presentation

ImagingImaging

MRI )Axial view(MRI )Axial view( MRI )Coronal view(MRI )Coronal view(

Trans-septal Inferior OrbitotomyTrans-septal Inferior Orbitotomy

HistopathologyHistopathology

Cavernous HemangiomaCavernous Hemangioma

Before surgery and a week after surgeryBefore surgery and a week after surgery

Inferior orbital tumors, likewise, are Inferior orbital tumors, likewise, are easily delivered through the easily delivered through the septum or periosteumseptum or periosteum..

No bone surgery is needed in most casesNo bone surgery is needed in most cases

33- - Diffuse orbital mass in a childDiffuse orbital mass in a child

Inferior Orbital HemangiomaInferior Orbital Hemangioma)Inducing Right Hypertropia)Inducing Right Hypertropia((

ImagingImaging

MRI T1 )Axial view(MRI T1 )Axial view( MRI T2 )Sagittal view(MRI T2 )Sagittal view(

Sub-ciliary skin incision and ExposureSub-ciliary skin incision and Exposure

Trans-septal Inferior OrbitotomyTrans-septal Inferior Orbitotomy

Total excision by Inferior OrbitotomyTotal excision by Inferior Orbitotomy

Closure of the WoundClosure of the Wound

HistopathologyHistopathology

Capillary HemangiomaCapillary Hemangioma

Resolution of hypertropiaResolution of hypertropia )Two weeks after surgery)Two weeks after surgery((

Pre-operative Post-operative

Case2:Case2: Diffuse Capillary HemangiomaDiffuse Capillary Hemangioma)Involving Eyelids, Conjunctiva & Orbit)Involving Eyelids, Conjunctiva & Orbit((

ImagingImaging

MRI T1 MRI T1 )Axial view()Axial view(

MRI T1MRI T1

)Sagittal view()Sagittal view( MRI T1 MRI T1

)Coronal view()Coronal view(

Marking skin incisions and tumor extensionMarking skin incisions and tumor extension

Delivery of the eyelids part of the tumorDelivery of the eyelids part of the tumor

Delivery of the orbital part of the tumorDelivery of the orbital part of the tumor

Total excision by sliding the tumor outTotal excision by sliding the tumor out

Closure of the WoundClosure of the Wound

HistopathologyHistopathology

Capillary HemangiomaCapillary Hemangioma

Two weeks after SurgeryTwo weeks after Surgery

Pre-operative Post-operative

Pediatric orbital capillary hemangioma can be excised, when Pediatric orbital capillary hemangioma can be excised, when indicated, indicated, with intact tumor wall with intact tumor wall to prevent amblyopiato prevent amblyopia..

4- Anterior4- AnteriorIntraconal Circumscribed MassIntraconal Circumscribed Mass

Initial PresentationInitial Presentation

ImagingImaging

CT )Axial view(CT )Axial view( CT ) Coronal view(CT ) Coronal view(

Fornix approach - Inferolateral OrbitotomyFornix approach - Inferolateral Orbitotomy

Fornix approach - Inferolateral OrbitotomyFornix approach - Inferolateral Orbitotomy

HistopathologyHistopathology

Cavernous HemangiomaCavernous Hemangioma

Two Weeks After SurgeryTwo Weeks After Surgery

Shortest route to Shortest route to anterior intraconalanterior intraconal masses is masses is

by transconjunctival approachby transconjunctival approach..

5- Large Intraconal Circumscribed Mass5- Large Intraconal Circumscribed Mass

Initial PresentationInitial Presentation

ImagingImaging

Previous CT )Axial view(Previous CT )Axial view( MRI )T2(MRI )T2(

Transconjunctival Superolateral OrbitotomyTransconjunctival Superolateral Orbitotomy

Transconjunctival Superolateral OrbitotomyTransconjunctival Superolateral Orbitotomy

HistopathologyHistopathologyNeurofibroma with myxoid degenerationNeurofibroma with myxoid degeneration

Three Weeks After SurgeryThree Weeks After Surgery

Preoperative and Postoperative ComparedPreoperative and Postoperative Compared

66- - Cryoextraction of Cryoextraction of A Large Intraconal Circumscribed MassA Large Intraconal Circumscribed Mass

Initial PresentationInitial Presentation

Lateral CanthotomyLateral Canthotomy

Limbal based Periotomy and hooking of musclesLimbal based Periotomy and hooking of muscles

Disinsertion of two recti musclesDisinsertion of two recti muscles

Retraction of the globe and orbital fatRetraction of the globe and orbital fat

Cryoextraction of the tumor out of orbitCryoextraction of the tumor out of orbit

Tumor delivered completely out of orbitTumor delivered completely out of orbit

Muscles are sutured in PlaceMuscles are sutured in Place

Lateral canthotomy is re-suturedLateral canthotomy is re-sutured

Conjunctiva is re-sutured in PlaceConjunctiva is re-sutured in Place

One Week After SurgeryOne Week After Surgery

Preoperative and Postoperative ComparedPreoperative and Postoperative Compared

What can be done, if you want to deliver a large mass What can be done, if you want to deliver a large mass from from aa small openingsmall opening ??! ??!

Think like an obstetricianThink like an obstetrician!!!!

A large tumor can be delivered through a small opening, if the A large tumor can be delivered through a small opening, if the tumor is tumor is compressible,compressible, and surrounding soft tissues are and surrounding soft tissues are stretchablestretchable!!

Bone cutting surgery is usually indicated for Bone cutting surgery is usually indicated for deepdeep tumors, for tumors, for optic nerveoptic nerve tumors, tumors,

and in and in tight orbitstight orbits..

Video: Modified Khronlein Lateral OrbitotomyVideo: Modified Khronlein Lateral Orbitotomy

77- - Stepwise approach in management Stepwise approach in management of a diffuse orbital tumorof a diffuse orbital tumor

Initial PresentationInitial Presentation

Right Proptosis after orbital biopsy 9 years agoRight Proptosis after orbital biopsy 9 years ago

Ocular HistoryOcular History

- )- )19921992( ( Total ExcisionTotal Excision of a of a leftleft orbital massorbital mass..

- )- )19931993( ( Incision BiopsyIncision Biopsy of a of a rightright

orbital massorbital mass..

HistopathologyHistopathology

Angiolymphoid Angiolymphoid HyperplasiaHyperplasia

))Progressive ProptosisProgressive Proptosis((

Previous Management HistoryPrevious Management History

- - Two more Two more debulking surgeriesdebulking surgeries were were followed by followed by RecurrenceRecurrence..

- - Several Several 2-weeks courses of full dose 2-weeks courses of full dose of systemic steroids were of systemic steroids were followed followed byby RecurrenceRecurrence..

- - 4040 Gys of Gys of fractionatedfractionated stereotactic stereotactic radiotherapyradiotherapy ended byended by RecurrenceRecurrence..

Imaging at initial visitImaging at initial visit

Diffuse orbital mass involving lateral rectus muscleDiffuse orbital mass involving lateral rectus muscle

Management: Management: Step 1Step 1

33 months course of Combination ofmonths course of Combination of

Prednisolone 30 mg/dayPrednisolone 30 mg/day

++

Azathioprine 50 mg/ dayAzathioprine 50 mg/ day

Before Medical TherapyBefore Medical Therapy

After 3 months of medical treatmentAfter 3 months of medical treatment

No further proptosis regression after 8 weeks of treatmentNo further proptosis regression after 8 weeks of treatment

MRI after Medical TherapyMRI after Medical Therapy

MRI T1 with contrast enhancement and fat MRI T1 with contrast enhancement and fat suppressionsuppression

MRI T1 Axial ViewMRI T1 Axial View

Management: Management: Step 2Step 2

Total Surgical Excision of tumor remnantsTotal Surgical Excision of tumor remnants

Before SurgeryBefore Surgery

Three Weeks After SurgeryThree Weeks After Surgery

No recurrence after 38 months of follow-upNo recurrence after 38 months of follow-up

Diffuse angiolymphoid hyperplasia of the Diffuse angiolymphoid hyperplasia of the orbit can be controlled by:orbit can be controlled by: - - Long termLong term course of course of low doselow dose of ofsystemic steroids + immunosuppressantsystemic steroids + immunosuppressant

- - Followed byFollowed bySurgical excision of the shrunken tumor Surgical excision of the shrunken tumor remnants.remnants.

- Patient is maintained on 5 mg/day of - Patient is maintained on 5 mg/day of systemic predinisolone, without recurrence systemic predinisolone, without recurrence for 3 years for 3 years

88- - Lid-Sparing Orbital Exenteration Lid-Sparing Orbital Exenteration for for

Invasive Conjunctival or Invasive Conjunctival or Orbital MalignancyOrbital Malignancy

Initial PresentationInitial Presentation

Previous BiopsyPrevious BiopsyConjunctival Mucoepidermoid CarcinomaConjunctival Mucoepidermoid Carcinoma

En- block Excision of all orbital contentsEn- block Excision of all orbital contents

Dissecting both eyelids into Dissecting both eyelids into anterior and posterior lamellaeanterior and posterior lamellae

En- block Excision of all orbital contentsEn- block Excision of all orbital contents

Sparing anterior lamellae of both eyelidsSparing anterior lamellae of both eyelids

Suturing anterior lamellae of both eyelidsSuturing anterior lamellae of both eyelids

Video: Lid-sparing Orbital ExenterationVideo: Lid-sparing Orbital Exenteration

One week after surgeryOne week after surgery

Patient fitted with orbital prosthesisPatient fitted with orbital prosthesis

““Lid sparing” exenteration gives Lid sparing” exenteration gives rapid rehabilitation,rapid rehabilitation, but may be but may be not be suitable if the patient to receive adjuvant radiotherapynot be suitable if the patient to receive adjuvant radiotherapy..

11- - No bone surgery is needed in most No bone surgery is needed in most lacrimal gland and extraconal lacrimal gland and extraconal tumorstumors..

22- - Shortest route for anterior intraconal Shortest route for anterior intraconal tumors is by transconjunctival tumors is by transconjunctival approachapproach..

33- - Tissue laxity tested before attempting Tissue laxity tested before attempting delivery of a large tumor without delivery of a large tumor without bone cuttingbone cutting..

44- - Tranosseous approach for deep Tranosseous approach for deep tumors, tight orbits and optic tumors, tight orbits and optic

nerve tumorsnerve tumors. .

55- - Lid sparing exenteration gives faster Lid sparing exenteration gives faster rehabilitationrehabilitation..

ConclusionConclusion

Hope for Ocular Tumors PatientsHope for Ocular Tumors Patients

Clinical experienceClinical experience and modern and modern

investigative and treatment investigative and treatment

instruments,instruments, enable us to design enable us to design

accurateaccurate plans for management and plans for management and

follow-upfollow-up for each case. for each case.

The objective is both The objective is both totaltotal

elimination of tumor dangerselimination of tumor dangers with with

the maximal possible the maximal possible preservation preservation

of ocular structures and functionsof ocular structures and functions. .

Recent AdvancesRecent Advances in in

Ocular OncologyOcular Oncology

Hatem Krema, FRCSHatem Krema, FRCSConsultant in Ocular OncologistConsultant in Ocular Oncologist

Cairo – EgyptCairo – Egypt