6. Epidermal Tumors : Basal Cell Carcinoma
- Its four morphological types are the Nodular, Ulcerative, Pigmented &
Morpheaform tumor. Rarely, Cystic or Multicenteric.
- It mostly involves the lower eyelid. More likely to affect fair skinned persons
with high solar exposure.
- Tumors at the medial canthus warrant a special attention.
Rodent Ulcer Pigmented Nodular Morpheaform BCC
BCC
7. Epidermal Tumors : BCC Syndromes
Gorlin- Goltz Syndrome: A.D., multiple BCC, palmer dyskeratosis, bifid ribs,
odontogenic keratotic cysts and ectopic calcifications.
Xerodermia Pigmentosa: A.R., defective DNA repair mechanisms multiple
cancers in areas exposed to normal dose of UV rays.
Organoid Nevus Syndrome: Oculo-Neuro-Cutaneous syndrome, skin nodules,
parietal alopecia, linear pigmentation, multiple BCC .
Bazex Syndrome: A.D., Multiple facial BCCs with typical atrophic dermal
changes on the extremities “Ice-pick marks”.
Gorlin-Goltz Xerodermia Organoid Nevus
Syndrome Pigmentosa Syndrome
16. Neural Tumors : Neurofibroma
It is proliferation of Axons, Schwann cells & endoneural fibroblasts.
1- Plexiform Neurofibroma : Pathognomonic NF1, tends to recur.
2- Solitary Neurofibroma : Not associated with NF1
3- Multiple Neurofibromata : Mostly associated with NF1
Plexiform Café au lait Absent greater wing
Neurofibroma Patches of Sphenoid
17. Neural Tumors : Schwannoma
- It is proliferation of Schwann cells )Neurilemoma(.
- It can be multiple in association with NF1 or a solitary lesion.
- Encapsulted with perineural capsule.
- Aggressive recurrence if incompletely excised.
19. Vascular Tumors: Congenital Capillary Hemangioma
- Cutaneous, Subcutaneous, Diffuse or Orbital.
- Involution in 50% by fifth year, 70% by seventh year.
- Early interference if strabismus or occlusion – induced amblyopia.
- Associations: . Sublingual or nasopharyngeal hemangioma.
. Kassabach – Meritt Syndrome.
Cutaneous Subcutaneous Diffuse
Capillary Hemangioma Capillary Hemangioma Capillary Hemangioma
20. Vascular Tumors: Lymphangioma
- Multiple small cysts filled with clear fluid
and blood.
- Eyelid affection is mostly associated with
conjunctival lymphangioma.
- Bouts of engorgement with hemorrhage or
local infections.
- Associated with buccal or labial
lymphangioma.
- Shows no involution with age and Poor
response to steroids.
39. II- Conjunctiva: Benign Tumors
Benign amelanotic masses include squamous
cell papilloma, conjunctival granuloma and
amelanotic nevus.
Clinical experience with their various
presentations helps to avoid unnecessary
aggressive interference.
Limbal dermoid is a choriostoma.
If it has visual or cosmetic symptoms, excision
with partial lamellar corneal grafting then
may be needed.
40. II- Conjunctiva: Melanocytic Lesions
Pigmented melanocytic lesions involving the conjunctiva include
melanocytic nevus that mostly does not show malignant changes. Also, primary
acquired melanosis )PAM( with possible malignant potentials and conjunctival
malignant melanoma.
Knowing of the malignant potentials or metastases, and the final cosmetic
outcome, determines the extent of surgical management.
41. II- Conjunctiva: Malignant Tumors
Squamous cell carcinoma is the most
common primary malignant tumor of
conjunctiva, manifests usually as a fleshy
vascularized mass at the limbus.
Lymphocytic proliferative disorders can
involve the conjunctiva as a “salmon patch”.
It is essential here to exclude other systemic
or ocular tissue involvement before deciding
the full treatment plan.
42. II- Conjunctiva: Surgical Management
Alcohol epitheliectomy with lamellar
keratosclerectomy is the standard procedure
for malignant tumors at the limbus.
Conjunctival tumors are treated by complete
excision biopsy with frozen section control of
tumor margins and cryotherapy of the tumor
bed.
Reconstruction can be done then by simple
closure, conjunctival grafting or amniotic
membrane transplant.
43. II- Conjunctiva: Non-Surgical Options
Management of conjunctival neoplasia may
require the primary or adjunctive use of local
treatment with some chemotherapeutic agents
such as Mitomycin-C, 5- Flourouracil and
Interferon alpha 2-b.
In some diffuse radiosensitive tumors as
lymphoma, fractionated external beam
radiotherapy or application of a radioactive
plaque may be employed.
45. III- Intraocular: Iris Tumors
Iris masses could be melanocytic as
nevus and melanoma.
They also include inflammatory masses
as granuloma, vascular as hemangioma,
muscular as leiomyoma, infiltrative
such as lymphoma, metastases, and
extension from a ciliary body tumor.
46. III- Intraocular: Pigmented Tumors
Choroidal melanoma is the most common intraocualr tumor in adults.
Clinical examination is the method of choice to diagnose choroidal melanoma
and to differentiate a choroidal nevus from a small choroidal melanoma.
Other common intraocular pigmented tumors include optic nerve head
melanocytoma, retinal pigment epithelium adenoma and combined
hamartoma of retina and retinal pigment epithelium.
47. III- Intraocular: Non-pigmented Tumors
Choroidal non-pigmented masses include amelanotic melanoma, uveal
granuloma, lymphoma, osteoma and choroidal metastases.
Choroidal hemangioma has a characteristic orange color and is usually
associated with sub-retinal fluid transudation.
Retinal non-pigmented masses include retinoblastoma, astrocytoma, and
toxocara granuloma.
48. III- Intraocular: Retinoblastoma
Retinoblastoma is the most common
pediatric intraocular malignancy,
presents mostly with leukocoria or
strabismus.
Retinoblastoma is bilateral in 33% of
patient and can be multifocal. It may show
tendency to vitreous seeding and
extraocular spread along the optic nerve.
Management requires a great deal of
experience with the potentials of the new
treatment options available and with
accurate regimen for follow-up.
49. III- Intraocular: Investigations
Help to differentiate some
tumors from other tumor-
simulating masses.
Basic for precise measurement of
tumor dimension, and accurate
follow-up.
They include Intraocular
Transillumination, Retcam
Ultrasonography, Fundus
Photography and Angiography
and Doppler Ultrasonography .
51. Treatment : I- Tumor Resection
Local resection of unifocal intraocular tumors, with preservation of the eye, is
advocated for small to medium sized tumors of the iris, ciliary body and
peripheral choroid.
53. Treatment : II- Radioactive Plaque
The treatment of choice for most medium sized intraocular neoplasms. The
radioactive isotopes currently used in manufacturing plaques are
Ruthenium106 and Iodine125.
Dosimetry, is calculated by a sophisticated computer software.
64. Treatment: III- Transpupillary Thermotherapy
Transpupillary Thermotherapy is using a modified large spot infrared laser to
elevate temperature of tumor cells up to 60 o C, enough to cause damage to
malignant cells.
It is useful for small central tumors in the fundus with the least damage to
surrounding tissues
65. Combined Ruthenium Plaque Irradiation with
Transpupillary Thermotherapy
“ Sandwich Technique !”
Transpupillary
Thermotherapy
3.9 mm
Melanoma
5 mm
Ru Plaque
106
67. Treatment: IV- Cryotherapy
To ablate small peripheral fundus tumor, by performing triple freeze-thaw
cycles technique.
Useful in unresectable peripheral retinal tumors as retinal angiomas, and is
very effective in multifocal peripheral retinoblastoma.
68. Treatment: V- Chemotherapy
It has no role for melanoma treatment.
Initial treatment for medium to large, and multifocal Retinoblastoma, and in
some choroidal metastases.
For retinoblastoma: 6 cycles of Vincristine + Etoposide + Carboplatin +
Cyclosproin A.
Adjunctive Cryotherpay and Thermotherapy with Chemotherapy .
69. Treatment: VI- External Beam Irradiation
For intraocular lymphoma, metastatic carcinoma and vitreous seeds in
.retinoblastoma
.secondary cancers in irradiated region 35%
.Orbital bones deformity
70. Treatment: VII- Enucleation
In advanced melanoma, unilateral large retinblastoma with no visual
potential, failed treatment, or tumor-induced glaucoma.
Primary orbital implant gives excellent cosmetic outcome.
76. IV- Orbit: Circumscribed Tumors in Adults
The most common circumscribed orbital
tumors in adults include Cavernous
Hemangioma, Neurofibroma, orbital
dermoid, Fibrous Histiocytoma, and
Hemangiopericytoma.
The most common lacrimal gland tumors
include Pleomorphic adenoma and
Adenoid Cystic Carcinoma.
CT scans are preferred here, to study
orbital bone affection and to decide the
most suitable surgical approach.
77. IV- Orbit: Diffuse Tumors in Adults
The most common lesions that show as
diffuse masses, molding around orbital
structures, on orbital imaging include
Orbital Pseudotumor, Lymphoproliferative
tumors, Orbital Metastases,
Granulomatous inflammations and
Cellulitis.
Assessment for the possible systemic
associations in these cases can give clues to
the natural history of the suspected mass
prior to confirmation by incision biopsy.
78. IV- Orbit: Pediatric Tumors
Pediatric primary orbital masses include
cystic lesions as dermoid cyst, vascular
tumors as capillary hemangioma and
lymphangioma, Inflammatory lesions,
lymphocytic and leukemic infiltrates, and
Juvenile pilocytic astrocytoma of the optic
nerve.
Rhabdomyosarcoma may show rapid
progression as orbital Cellulitis, but has an
excellent cure rate with combined
radiotherapy and chemotherapy.
145. What can be done, if you want to deliver a large mass
??!from a small opening
146. !!Think like an obstetrician
A large tumor can be delivered through a small opening, if the
!tumor is compressible, and surrounding soft tissues are stretchable
147. Bone cutting surgery is usually indicated for deep tumors, for optic nerve tumors,
.and in tight orbits
151. Ocular History
Total Excision of a left( 1992- )
.orbital mass
Incision Biopsy of a right( 1993- )
.orbital mass
(Progressive Proptosis)
Histopathology
Angiolymphoid
Hyperplasia
152. Previous Management History
Two more debulking surgeries were-
.followed by Recurrence
Several 2-weeks courses of full dose-
of systemic steroids were followed
.by Recurrence
Gys of fractionated stereotactic 40-
radiotherapy ended by
.Recurrence
153. Imaging at initial visit
Diffuse orbital mass involving lateral rectus muscle
154. Management: Step 1
months course of Combination of 3
Prednisolone 30 mg/day
+
Azathioprine 50 mg/ day
160. Three Weeks After Surgery
No recurrence after 38 months of follow-up
161. Diffuse angiolymphoid hyperplasia of the
orbit can be controlled by:
- Long term course of low dose of
systemic steroids + immunosuppressant
- Followed by
Surgical excision of the shrunken tumor
remnants.
- Patient is maintained on 5 mg/day of
systemic predinisolone, without recurrence
for 3 years
173. Lid sparing” exenteration gives rapid rehabilitation, but may be“
.not be suitable if the patient to receive adjuvant radiotherapy
174. No bone surgery is needed in most- 1
acrimal gland and extraconal
umors
hortest route for anterior intraconal- 2
umors is by transconjunctival
approach
Tissue laxity tested before attempting- 3
elivery of a large tumor without
bone cutting
Tranosseous approach for deep- 4
umors, tight orbits and optic
nerve tumors
Lid sparing exenteration gives faster- 5
rehabilitation
176. Hope for Ocular Tumors Patients
Clinical experience and modern
investigative and treatment
instruments, enable us to design
accurate plans for management and
follow-up for each case.
The objective is both total
elimination of tumor dangers with
the maximal possible preservation
of ocular structures and functions.
177. Recent Advances
in
Ocular Oncology
Hatem Krema, FRCS
Consultant in Ocular Oncologist
Cairo – Egypt
