Rianti Soediro Suryo

Tumor & Reconstruction Unit

Cicendo Eye Hospital Bandung
 Introduction
• Eye Tumors can :

* Be live threatening

* Reduce vision

* Cause cosmetic problems

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 Eye Tumor Classification
• External : on palpebra, conjunctiva, cornea
and lacrimal sac.

• Intraocular

• Orbital

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 Benign External Eye Tumor

• Dermoid cyst and dermoid tumor

• Xanthelasma
• Millium
• Papilloma
• Nevus
• Verruca vulgaris = Common wart
• Molluscum contagiosum
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 Malignant External Tumor

• Carsinoma in situ
• Basal cell carcinoma
• Squamous cell carcinoma
• Aquired melanosis
• Lacrimal sac tumor

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 Intraocular Eye Tumor

• 1. Malignant melanoma.

• 2. Retinoblastoma.

• 3. Uveal metastatic tumor.

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 Orbital Tumor (1)

• Hemangioma
• Neurogenic Tumor :
* Optical nerve glioma
* Meningioma
* Neurofibroma
• Rhabdomysarcoma

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 Orbital Tumor (2)
• Lacrimal gland tumor :
* Benign mixed tumor
* Adenoid cystic carcinoma
• Metastatic tumor
• Malignant lymphoma
• Idiopathic orbital inflammatory desease
• Dysthyroid ophthalmopathy

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 Dermoid Cysts
• Congenital tumor
• The tumor wall consists of dermis and
epidermis layer
• Location : supero-temporal
• Tumor palpation : firm, round, and smooth,
freely mobile under the skin.
• T/ : In toto excision

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 Dermoid Cysts

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 Dermoid Tumor
• Congenital, located at the limbus
• Consists of connective tissue with hair
follicles & sebaceus gland, walled by stra-
tified squamous epithelium
• Signs : solid, smooth, round, protruded
mass, whitish in colour
• T/ : excision

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 Dermoid Tumor

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 Nevus = Benign Melanoma(1)
• Consists of pigment stained cells
• Observed in children, grows during puberty
with increase pigmentation
• Nevus cells can be found in the :
* epidermis : Junctional nevus
* dermis : Compound nevus
* whole dermic layers : Intradermal nevus

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 Nevus = Benign Melanoma(2)
• Malignant degeneration can happen to
junctional & intradermal nevus
• Signs : slightly elevated, pigment stained,
clearly defined lesion
• T/ : Excision ; with following indications :
* cosmetic
* irritation
* rapid tumor’s growth

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 Nevus = Benign Melanoma

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 Papilloma
• Location : margo palpebra and limbus
• Signs : tumor mass is cauliflower-like and
pedunculated
• Large tumor size can resembles malignancy
• Has regrowth tendency
• Related to viral infection.
• T/ : In toto excision.

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 Verruca vulgaris
• Not a true tumor

• Etiology : viral

• Shape : various

• T/ : excision

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 Molluscum Contagiosum
• Etiology : a pox viral
• Signs : small, pale, waxy,
umbilicated nodule
• T/ : excision

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 Intraepithelial epithelioma =
Carsinoma in situ =
Bowen Desease
• Location :
cornea, conjungtiva, palpebral skin
• Age 60, particularly men
• Signs : Diffuse lesion, elevated, hyper-
keratotic nodule
• T/ : In toto excision

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 Carsinoma in situ

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 Basal Cell Carsinoma
• Known as Basal cell epithelioma
• 90% on eyelids
• Age 50-55 y, particularly men
• Signs : ulcerative small node, with elevated
border
• Rarely cause metastasis

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 Basal Cell Carsinoma: Therapy
• Wide excision with 2 mm safety margin,
controlled by frozen section
• Radiotherapy
• Cryotherapy
• Mohs’ Chemosurgery
• Curetage and electrodessication

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 Basal Cell Carsinoma

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 Squamous Cell Carsinoma
• 2 - 9 % of eyelid malignancies
• More aggressive tumor than BCC
• Slow growth
• Metastasis to regional lymph nodes
• Sign : plaque-like, nodular, ulcerating tumor
• T/ : similar to basal cell carcinoma

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 Acquired Melanosis
• Manifestation : Precancerous or cancerous
• Precancerous : at age 40-60 y, becomes
malignant after 5-10 y
• Signs : diffuse pigmentation
• Treatment : wide conjunctivectomy with
adjuvant irradiation
• Cancerous : excenteration

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 Lacrimal Sac Tumor

• Rarely found
• Benign : papiloma
• Malignant : carcinoma
• Sign : tumor mass + epiphora
• Treatment : total excision and irradiation

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 Intraocular Malignant Melanoma

• Iris Melanoma : 6-8%

• Uveal melanoma : 9%

• Choroidal Melanoma : 85%

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 Iris Melanoma
• Sign : pigmented or nonpigmented nodule,
>3mm in diameter and >1mm in thickness
• Angle involvement, if extensive, may give
rise to secondary glaucoma
• T/ :
* Small tumors : iridectomy
* Diffusely growing tumors :
enucleation

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 Ciliary Body Melanoma
• Often recognized clinically after it pushes
the iris anteriorly
• Sign: depend on the size and location of
the tumor
• Extraocular extension through the scleral
emissary vessels may produce a dark
epibulbar mass
• T/ : iridosiclectomy or enucleation

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 Choroidal Melanoma
• Signs : elevated, subretinal, oval-shaped
mass
• Frequently brown, may be amelanotic
• Mushroom-shaped appearance as it breaks
through the Bruch membrane
• T/ Small tumors : observation
Very large tumors : enucleation
Extensive extraocular extension :
excenteration
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 Metastatic tumor to Uvea
• The most frequent primary-tumor site is :
* The breast in women
* The bronchus in men
• Signs : fast growing, creamy-white, placoid
or oval lesion most frequently at macula
• T/ : Radiotherapy, Systemic therapy
Enucleation for a painful blind eye

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 Metastatic tumor to Uvea

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 Retinoblastoma
• The most common primary, malignant,
intraocular tumor of childhood
• Occurring in about 1:20.000 live birth
• No sexual predilection
• The vast majority become clinically
apparent before the age of 3 years

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 Genetic Aspects of
Retinoblastoma
• Non heritable account for 60% of cases
• The tumors arises at the somatic level in a
single retinal cell
• Heritable account for 40 % of cases.
• The primitive retinal cells are predisposed
to malignant transformation
• Only 6% have a positive family history
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 Presentation of Retinoblastoma
• Leukocoria (white • Orbital inflamation
pupillary reflex), in • Proptosis
about 60% cases. • Metastasis (regn. LN)
• Strabismus, in 20% of • Routine exam to risk
cases patients
• Secondary glaucoma
• Anterior segment
invasion

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 Signs of Retinoblastoma
• Early intraretinal tumor : a flat or round
white lesion
• Endophytic tumor : white mass, secondary
calcification
• Exophytic tumor :
* Multiglobulated white mass with
retinal detachment
* Vitreus hemorrhage

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 Retinoblastoma
Special Investigations

• Ultrasonography

• CT-Scan : to detect calsification

• MRI : to detect optic nerve infiltration

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 Retinoblastoma Treatment
• Unilateral cases : enucleation, including
about 10 mm of optic nerve
• Bilateral cases : enucleation of the worse
eye, irradiation of the fellow eye
• Extraocular growth : excenteration
• Metastasis : palliative radiotherapy or
chemotherapy

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 Retinoblastoma Treatment

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 Retinoblastoma Prognosis

• Optic nerve :
* uninvolved : 8% mortality rate
* involved : 65 % mortality rate

• The tumor involves the lamina cribosa :

15% mortality rate

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 Capillary Hemangioma
• Appear at the first month after birth
• Strawberry birthmark on palpebral skin
• 1st year enlargement followed by regression
• T/ : * Steroid, low dosage of irradiation
* Dysfunction or marked deformity is
an indication of excision

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 Capillary Hemangioma

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 Cavernous Hemangioma
• Benign orbital tumor most frequently found
in adults
• Signs : slow progressive exophthalmos
• Age : 20-40 year
• D/ : USG and CT-Scan
• T/ : Extirpation

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 Rhabdomyosarcoma
• The most common primary orbital malignant
tumor in children.
• Presentation : rapidly progressive proptosis on
children 7 year of age
• Sign : A palpable mass at nasal superior orbit
• Swelling and injection of overlying skin but the
skin is not hot
• D/ : USG and CT, biopsy
• T/ : Irradiation and chemotherapy

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 Rhabdomyosarcoma

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 Pleomorphic Lacrimal Gland
Adenoma (Benign Mixed Tumor)
• Lacrimal gld. most common epithelial
tumor
• The fifth decade of life
• Painless, slowly progressive swelling in the
upper outer quadrant
• D/ : X-ray, CT
• T/ : In toto surgical excision without prior
biopsy

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 Lacrimal gland carsinoma
(Adenoid cystic carsinoma)
• A high morbidity and mortality rare tumor
• Presentation : 40-60years of life
• Tends to spread perineurally with bone
destruction
• T/: * Excenteration and mid-facial resection
* Radiotherapy + local resection

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 Metastatic Tumor in Children

• The most common metastatic tumor in

children is :
* Neuroblastoma
* Ewing Sarcoma
* Leukemia
• T/ : Palliative local irradiation

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 Malignant Lymphoma

• Location : subconjunctiva and lacrimal gland

• The tumor maybe primary or disseminated
• Signs : exphthalmos and vision impairment
• D/ : X- ray, USG, CT, Biopsy
• T/ : * Irradiation for localized lesions
* Chemotherapy for disseminated disease

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 Idiopathic Orbital Inflammatory
Disease (Orbital Pseudotumor)
• Non-neoplastic and non-infectious,
space-occupying orbital lesions
• Abrupt painful onset in 20-50y of age
• Signs : periorbital swelling, chemosis
and conjunctival inflammation
• Proptosis, ophthalmoplegia, optic nerve
dysfunction may be present
• T/: systemic steroids
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 Dysthyroid Ophthalmopathy
• Female : Male = 8 : 1
• Patients’ age : 20-45 years
• Clinical features :
* Darymple’s sign * Von Graefe’s sign
* Exophthalmos * Diplopia
* Periorbital and lid swelling
* Conjuctival hyperaemia

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 Dysthyroid Ophthalmopathy

• D/ : Blood T4,T3

• USG and CT

• T/ : High dose of steroid

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