TUMOR

GANAS MATA
ALFA SYLVESTRIS, MD
RETINOBLASTOMA

• “Retinoblastoma is a disease
that causes the growth of
malignant tumors in the retinal
cell layer of the eye.”
• It is the most common eye
tumor in children (<5 yo)

2
THE PREVALENCE OF
RETINOBLASTOMA
• Retinoblastoma is a childhood disease.
• There is no known sex or racial
predilection.
• “Retinoblastoma affects 1 child for every
15,000 live births.”
• 250-350 new cases are diagnosed each
year in the U.S., 90% of which occur
before the age of five.
• Retinoblastoma can be either a
hereditary or non-hereditary disease
• unilateral / bilateral

• extraocular:

- through sclera 
orbital cavity  proptosis
- through N.II  intra cranial
WHAT CAUSES RETINOBLASTOMA?
• Retinoblastoma is caused by a mutation
on the 13th chromosome.
• “Possibly environmental factors increase
mutational events at the retinoblastoma
gene locus.”
SYMPTOMS OF RETINOBLASTOMA
• Problems with eye • “A white spot on
movements the pupil of the
(crossed eyes). eye.” cat’s eye
• A persistent red • Visual disturbance
irritation in the eye.
• Differences in pupil
size, iris color,
abnormal eye
movements,
bulging forward of
the eyes, tearing,
and cataract.
SIGN
• VA decreased
• Leucocoria
• Strabismus
• Glaucoma
• Tumor mass in the vitreous with
calcification
ADDITIONAL EXAM
• X Photo Ro
• USG B scan
• CT Scan
• PA durante op
• Lactic Dehidrogenase enzyme (LDH) in
humour aqeous paracintesa (compared
with level serum)
• Specific skin test for retinoblastoma using
crude membrane extracts of
retinoblastoma cells developed in tissue
culture.
• Tapping paracintesa for tumour cells in
anterior chamber
HISTOPATHOLOGY

Flexner-Wintersteinerrosettes Homer-Wright rosette

HISTOPATHOLOGY

Fleurettes
DIAGNOSIS
• Untreated, Retinoblastoma is
almost always fatal.
• Early examination is key to
survival.
STAGING SYSTEMS REESE-ELLSWORTH CLINICAL
GROUPING

• Stage I - Very Favorable · Solitary tumor, less than 4

disk diameters (DD)* in size, at or beyond equator. ·
Multiple tumors, none over 4 DD in size, all at or
behind equator.
• Stage II - Favorable · Solitary tumor, 4 to 10 DD in size,
at or behind equator. · Multiple tumors, 4 to 10 DD in
size, at or behind equator.
• Stage III - Doubtful · Any lesion anterior to equator. ·
Solitary tumors larger than 10 DD behind equator.
• Stage IV - Unfavorable · Multiple tumors, some larger
than 10 DD. · Any lesion extending anteriorly to ora
serrata. Stage V - Very Unfavorable · Massive tumors
involving over half the retina. · Vitreous
seeding.
* 1 Disk Diameter (DD)= 1,6 mm.
TREATMENTS
• Chemotherapy
• Cryotherapy (freezing treatment)
• Enucleation ( removal of the eye)
• External beam radiation therapy
(radiation treatment)
• Localized plaque radiation therapy
(radiation therapy)
• Photocoagulation (laser treatment)
PROGNOSIS
• The survival rate for Retinoblastoma
patients is more than 90%.
• This is attributed to earlier diagnosis
and improved methods of treatment.
SQUAMOUS CELL CARCINOMA
• SCC  keganasan ke-2 terbanyak pd
kelopak mata (9%)
• US  105 kasus tiap 100.000
penduduk
• Australia  166 kasus tiap 100.000
penduduk
• SCC kelopak mata > conjungtiva
• Invasi ke bola mata, struktur orbita,
lymphe node regional, metastese
jauh  diagnosa awal sangat
penting !
ETIOLOGI
• Paparan sinar matahari kronis
• Usia tua (60-70 th)
• Orang muda dengan imunitas 
(radioterapi, HIV)
• Human papilloma virus pd pasien HIV
• Zat kimia (minyak, tar, asap rokok,
arsenik, parafin)
• Dermatosis prekanker
• Pria (75%) > wanita (25%)
DIAGNOSIS KLINIS
• Riwayat kelainan dan keganasan
kulit
• Status imun penderita
• Riwayat paparan UV
• Riwayat paparan zat kimia
• Riwayat lesi jinak kelopak mata
• Kelopak mata bawah > sering drpd
atas (1,4:1)
• Tidak khas  nodule, plak dg tepi
ireguler, luka kronis, fissura kulit, tepi
mengkilat, telengiektasis, ulserasi,
papiloma, cutaneous horn, lesi kistik
• sering didaerah
limbus, pada fissura
interpalpebra
• sering mirip
pterygium

• Menjadi tumor
ganas kornea
bila
menginvasi
daerah kornea
PEMERIKSAAN
• Hertel exophthalmometri
• Palpasi lymphe node regional
• Tes faal hepar
• Analisa genetis untuk xeroderma
pigmentosum
• Tes HIV
• CTscan
POLA INVASI DAN METASTASIS
• Dermis  otot orbicularis superficial
• Wajah, periosteum, pembuluh lymphe, pembuluh
darah, selubung syaraf
• Klasifikasi BRODERS’  grade I,II,III,IV
 Grade 1: 75% keratinocytes are well differentiated
 Grade 2: >50% keratinocytes are well differentiated
 Grade 3: >25% keratinocytes are well differentiated
 Grade 4: <25% keratinocytes are well differentiated
• Penyebaran lymphatic  lnn. Preauriculer, lnn.
Submandibular,
• Infiltrasi perineural  n.trigeminus, syaraf motoris
ekstraokuli, n. fascialis
HISTOPATOLOGI
• Sarang-sarang sel masuk ke dermis
disertai reaksi inflamasi kronis
• Keratinisasi pada well differentiated
• Undifferentiated  sitoplasma eosinofilik,
mutiara keratin, jembatan interseluler
STADIUM KLINIS
T0 lesi in situ
T1 diameter < 2 cm
T2 diameter 2-4 cm
T3 diameter > 4 cm
T4 invasif pada tulang dan otot
TERAPI
PEMBEDAHAN
Bedah eksisi dengan :
• Tehnik Mohs’
• Vriescope
Diikuti dengan bedah rekonstruksi

Kadang diperlukan eksenterasi orbita dan eksisi

en bloc bila telah melibatkan tulang
RADIOTERAPI
Bila ada KI bedah atau menolak operasi
SCC lebih resisten  dosis lebih 
Kelemahan :
• Tepi tidak terkontrol
• Komplikasi post radiasi
• Kunjungan berulang kali
•Tidak dapat : area yang pernah diradiasi, tumor
di tengah kelopak, usia < 40 th, xeroderma
pigmentosum
CRYOTERAPI
• Kelebihan : biaya, nyaman, potensiasi
kataraktogenik , dapat diulang
• Tidak dapat : tumor terfiksasi di
periosteum, pada canthus medialis,
diameter >10 mm, lesi tidak jelas
tepinya, lesi melebihi conjungtiva
fornix
• Nitrogen cair semprot dengan
melindungi bola mata
• ES  depigmentasi, bulu mata hilang,
hipertrofi scar, ektropion, epifora,
hiperplasia pseudoepitelomatous
KEMOTERAPI
• Sebagai terapi tambahan untuk SCC
kelopak mata lanjut
• Cisplatin, atau kombinasi dengan
doxorubicin, bleomycin, isotretinoin, -
interferon
FOLLOW UP
• Curiga SCC kelopak mata  evaluasi
6-12 bulan
• Telah mengenai lymph node  2-3
bulan selama 2 th pertama
PENCEGAHAN
• Sunscreen SPF 15 wajah dan kelopak
mata bawah, dan dioleskan tipis-tipis
pada kelopak mata atas dan dahi
• Pakaian dan topi pelindung
• Menghindari paparan sinar matahari
pk. 10.00-15.00 WIB sore
 Menurunkan 78%
BASAL CELL CARCINOMA
• Most common human malignancy
• Elderly patients
• Chronic exposure to sunlight
• Slow growing, locally invasive, non
metastating
NODULO-ULCERATIVE BCC
 SHINNY, FIRM, PEARLY NODULE,
SMALL DILLATED BLOOD VESSEL
ON ITS SURFACE
 GROWTH SLOW  CENTRAL
ULCERATION, RAISED ROLLED
EDGES (RODENT ULCER)

SCLEROSING BCC
 DIFFICULT TO DX  INFILTRATES
LATERALLY BENEATH THE EPIDERMIS
 PLAQUE
 PALPATION BETTER THAN
INSPECTION TO DETERMINE THE
TUMOR.
TREATMENT
• Surgical excision  remove the entire
tumour but preserve as much normal tissue
as possible
•  together with 4 mm margin tissue which
looks clinically normal
•  large tumour  frozen section or Moh
micrographic surgery
• Reconstruction the defects
• Radiotheraphy  for nodulo-ulcerative BCC
with no involvement of medial canthal area
and unsuitable for/refuse surgery
TREATMENT
Cryotheraphy  small superficial BCC
Laser microsurgery  well-circumscribed BCC of the lid
margin without conjunctival extention
MELANOMA MALIGNA
ORIGIN
• arising from PAM (primary acquired melanosis) with
atypia 75 %
• arising from a pre existing naevus 20 %
• primary melanomais the least common  6th
decade
MELLANOMA MALIGNA CONJUNCTIVA
• A solitary, black or grey nodule containing
dilated feeder vessels which may become
fixed to the sclera
• Amelanotic tumours are pink, smooth, fish-
flesh app.
• A common site is the limbus (may arise
everywhere)
THERAPY
• Circumscribe melanoma
 wide excision with clearence and cryotherapy to
prevent reccurence
 incomlete clearence +  re excision and
cryotherapy
 follow up every 6-12 monthly  suspicious area
 biopsy and impression citology
• Diffuse melanoma  excision and cryotherapy or
mitomicin C
• Orbital recurences  local resection and
raadiotherapy
• Lymph node involve  excision and radiotherapy
• Palliation  chemotherapy for metastatic disease
PROGNOSIS
• 5 ysr 12 %
• 10 ysr 25 %
• Metastase : regional lymph nodes,
lung, brain, liver
MELLANOMA MALIGNA EYELID
• Rare, but lethal
• Superficial spreading melanoma  plaque with
an irregular outline and variable pigmentation
• Nodular melanoma  blue-black nodule
surrounded by normal skin
• Melanoma arisin gfrom lentigo maligna (slowly
expanding pigmented macule in elderly 
Hutchinson freckle)
MELLANOMA MALIGNA CILLIARY BODY
• In the sixth decade with visual symptoms
• Discovered incidentally
• Pupillary dilatation and gonioscopy
Dilated episcleral blood vessels in the same
quadrant of tumour

Extraocular extension through sclera

Pressure to the lens  astigmatism,
subluxation

Erosion iris root

Retinal detachment caused by post
extension

Anterior uveitis
Annular/ circumferential growth 360˚-
 worst prognosis e.c difficulty to
diagnose
DIAGNOSTIC
• Triple mirror contact lens
• Transillumination  for amelanotic
melanoma
• USG
• Biopsy
THERAPY
• Enucleation  large tumour and
affecting the anterior choroid,
secondary glaucoma
• Iridocyclectomy  small medium
tumours involving less than one third
of the angle
• Radiotherapy
MELLANOMA MALIGNA CHOROID
• Sixth decade of life
• Decrease VA or VF defect
• Third patients  very brief ‘balls of light’
traveling across the visual field two-three
times a day in the subdued lighting
Elevated subretina, dome shaped, brown or
grey mass, mottled with dark brown/ black
pigment/ amelanotic.
Mushroom shape app if breaks through
Brunch membrane
COMPLICATION
• Secondary exudative RD
• Choroidal folds, haemmorrhage,
secondary glaucoma, cataract, and
uveitis
DIAGNOSIS
• Binocular indirect ophthalmoscopy
• Indirect slit lamp biomicroscopy
• USG
• FFA
• ICG
• MRI
• FNAB

METASTASIS  lung, mammae, GIT

TREATMENTS
• Brachytherapy  tumours < 10 mm in
elevation and < 20 mm in basal dia.
• External radiotherapy  more posterior
(> 4 mm of the disc)
• Transpupillary thermotherapy (w/ diode
laser)  small tumours, location near the
fovea or optic disc
• Trans scleral local resection = excision
tumours with the rim of healthy choroid
under a partial thickness scleral flap 
too thick for radiotherapy, < 16 mm in
dia.
 • Stereotatic radiosurgery  large tumours
with preservation of visual function in
selected case
• Enucleation  very large tumours, visual loss
• Exenteration  extensive extraocular
extension or orbital recurrences
• Palliation  chemo therapy /
immunotherapy  metastatic disease

PROGNOSIS
Lung metastatic < 1 year, Liver metastatic < 6
mo
SELAMAT BELAJAR