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
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