TUMOR

GANAS MATA
ALFA SYLVESTRIS, MD

KULIAH PAKAR dr. ALFA, SpM

 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)

KULIAH PAKAR dr. ALFA, SpM

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

KULIAH PAKAR dr. ALFA, SpM

 • unilateral / bilateral

• extraocular:
- through sclera 
orbital cavity  proptosis
- through N.II
 intra cranial

KULIAH PAKAR dr. ALFA, SpM

WHAT CAUSES RETINOBLASTOMA?
• Retinoblastoma is caused by a mutation
on the 13th chromosome.
• “Possibly environmental factors increase
mutational events at the retinoblastoma
gene locus.”

KULIAH PAKAR dr. ALFA, SpM

SYMPTOMS OF
RETINOBLASTOMA
• Problems with eye • “A white spot on
movements (crossed the pupil of the
eyes).
eye.” cat’s eye
• A persistent red
irritation in the eye. • Visual
• Differences in pupil disturbance
size, iris color,
abnormal eye
movements, bulging
forward of the eyes,
tearing, and cataract.

KULIAH PAKAR dr. ALFA, SpM

SIGN
• VA decreased
• Leucocoria
• Strabismus
• Glaucoma
• Tumor mass in the vitreous with
calcification

KULIAH PAKAR dr. ALFA, SpM

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
KULIAH PAKAR dr. ALFA, SpM
HISTOPATHOLOGY

Flexner-Wintersteinerrosettes Homer-Wright rosette

KULIAH PAKAR dr. ALFA, SpM

HISTOPATHOLOGY

Fleurettes

KULIAH PAKAR dr. ALFA, SpM

DIAGNOSIS
• Untreated, Retinoblastoma is
almost always fatal.
• Early examination is key to
survival.

KULIAH PAKAR dr. ALFA, SpM

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
KULIAH retina.
PAKAR dr. ALFA, SpM · Vitreous seeding.
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)

KULIAH PAKAR dr. ALFA, SpM

PROGNOSIS
• The survival rate for Retinoblastoma
patients is more than 90%.
• This is attributed to earlier
diagnosis and improved methods of
treatment.

KULIAH PAKAR dr. ALFA, SpM

KULIAH PAKAR dr. ALFA, SpM
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 !

KULIAH PAKAR dr. ALFA, SpM

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%)

KULIAH PAKAR dr. ALFA, SpM

DIAGNOSIS KLINIS
• Riwayat kelainan dan keganasan
kulit
• Status imun penderita
• Riwayat paparan UV
• Riwayat paparan zat kimia
• Riwayat lesi jinak kelopak mata

KULIAH PAKAR dr. ALFA, SpM

 • 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

KULIAH PAKAR dr. ALFA, SpM

 • sering didaerah
limbus, pada
fissura
interpalpebra
• sering mirip
pterygium
• Menjadi tumor
ganas kornea
bila
menginvasi
daerah kornea

KULIAH PAKAR dr. ALFA, SpM

PEMERIKSAAN
• Hertel exophthalmometri
• Palpasi lymphe node regional
• Tes faal hepar
• Analisa genetis untuk xeroderma
pigmentosum
• Tes HIV
• CTscan

KULIAH PAKAR dr. ALFA, SpM

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

KULIAH PAKAR dr. ALFA, SpM

HISTOPATOLOGI
• Sarang-sarang sel masuk ke dermis
disertai reaksi inflamasi kronis
• Keratinisasi pada well differentiated
• Undifferentiated  sitoplasma
eosinofilik, mutiara keratin, jembatan
interseluler

KULIAH PAKAR dr. ALFA, SpM

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

KULIAH PAKAR dr. ALFA, SpM

TERAPI
PEMBEDAHAN
Bedah eksisi dengan :
• Tehnik Mohs’
• Vriescope
Diikuti dengan bedah rekonstruksi

Kadang diperlukan eksenterasi orbita dan eksisi

en bloc bila telah melibatkan tulang

KULIAH PAKAR dr. ALFA, SpM

 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

KULIAH PAKAR dr. ALFA, SpM

 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

KULIAH PAKAR dr. ALFA, SpM

 KEMOTERAPI
• Sebagai terapi tambahan untuk SCC
kelopak mata lanjut
• Cisplatin, atau kombinasi dengan
doxorubicin, bleomycin, isotretinoin,
-interferon

KULIAH PAKAR dr. ALFA, SpM

FOLLOW UP
• Curiga SCC kelopak mata  evaluasi
6-12 bulan
• Telah mengenai lymph node  2-3
bulan selama 2 th pertama

KULIAH PAKAR dr. ALFA, SpM

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%

KULIAH PAKAR dr. ALFA, SpM

KULIAH PAKAR dr. ALFA, SpM
BASAL CELL
CARCINOMA
• Most common human malignancy
• Elderly patients
• Chronic exposure to sunlight
• Slow growing, locally invasive, non
metastating

KULIAH PAKAR dr. ALFA, SpM

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.

KULIAH PAKAR dr. ALFA, SpM

KULIAH PAKAR dr. ALFA, SpM
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

KULIAH PAKAR dr. ALFA, SpM

TREATMENT
Cryotheraphy  small superficial BCC
Laser microsurgery  well-circumscribed BCC of
the lid margin without conjunctival extention

KULIAH PAKAR dr. ALFA, SpM

KULIAH PAKAR dr. ALFA, SpM
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

KULIAH PAKAR dr. ALFA, SpM

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)

KULIAH PAKAR dr. ALFA, SpM

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
•KULIAH
Palliation
PAKAR dr. ALFA, SpM
 chemotherapy for metastatic disease
PROGNOSIS
• 5 ysr 12 %
• 10 ysr 25 %
• Metastase : regional lymph nodes,
lung, brain, liver

KULIAH PAKAR dr. ALFA, SpM

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)

KULIAH PAKAR dr. ALFA, SpM

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

KULIAH PAKAR dr. ALFA, SpM

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
KULIAH PAKAR dr. ALFA, SpM
DIAGNOSTIC
• Triple mirror contact lens
• Transillumination  for amelanotic
melanoma
• USG
• Biopsy

KULIAH PAKAR dr. ALFA, SpM

THERAPY
• Enucleation  large tumour and
affecting the anterior choroid,
secondary glaucoma
• Iridocyclectomy  small medium
tumours involving less than one
third of the angle
• Radiotherapy

KULIAH PAKAR dr. ALFA, SpM

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

KULIAH PAKAR dr. ALFA, SpM

COMPLICATION
• Secondary exudative RD
• Choroidal folds, haemmorrhage,
secondary glaucoma, cataract, and
uveitis

KULIAH PAKAR dr. ALFA, SpM

DIAGNOSIS
• Binocular indirect
ophthalmoscopy
• Indirect slit lamp biomicroscopy
• USG
• FFA
• ICG
• MRI
• FNAB

METASTASIS  lung, mammae, GIT

KULIAH PAKAR dr. ALFA, SpM
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.

KULIAH PAKAR dr. ALFA, SpM

 • 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

KULIAH PAKAR dr. ALFA, SpM

KULIAH PAKAR dr. ALFA, SpM
CARCINOMA KELENJAR
LACRIMAL
• Upper eyelid fullness,
• alteration of the upper eyelid contour,
• downward and nasal displacement of the globe

• 50% benign mixed tumors and 50% carcinomas.

• About half of the malignancies are adenoid cystic
carcinoma
• The incidence of epithelial tumors of the lacrimal gland
ranges from 5 to 8% of orbital neoplasia

KULIAH PAKAR dr. ALFA, SpM

DIAGNOSIS
• duration of symptoms  acute /chronic
• the presence of pain  painfull swelling upper eyelid
• radiologic findings  CT scan (elongated mass extending
along the lateral orbital wall with expansion of the lacrimal
fossa with bone invasion and calcification)
• failure to resolve over a few weeks  biopsy (transeptal
incision)
• painless swelling in the upper lid without inflammatory
symptoms, presents >12 months benign mixed tumor
(pleomorphic adenoma)
• CT scans  round to oval, wellcircumscribed mass and
enlargement of the lacrimal fossa without invasion of
overlying bone. Biopsy of pleomorphic adenoma should be
avoided.
• completely remove all encapsulated or well circumscribed
masses
KULIAH without
PAKAR dr. ALFA, SpM incisional biopsy.
KULIAH PAKAR dr. ALFA, SpM
DIAGNOSIS
• Dermoid cysts are not true
lacrimal gland tumors 
originate from epithelial rests
located in the orbit, particularly
in the superolateral quadrant.
• present as painless proptosis in a
younger age group.
• Bony change in the superolateral
wall is common.
• diagnosed easily by CT or MRI 
images of a lesion of fatty
density with no or only faint
enhancement and smoothly
outlined osseous changes.

KULIAH PAKAR dr. ALFA, SpM

MALIGNANT MIXED TUMOR

• 4 to 15% of the epithelial tumors of the lacrimal gland

• a pleomorphic adenoma that has undergone malignant
degeneration
• Men are more often affected by an adenocarcinoma
arising from pleomorphic adenoma, whereas women
are more often affected by an adenoid cystic
carcinoma arising from pleomorphic adenoma.
• Older age.

KULIAH PAKAR dr. ALFA, SpM

MALIGNANT MIXED TUMOR
Malignant mixed tumors usually present in three clinical ways.
• First, the patient whose benign mixed tu-mor was not removed
totally may develop a sudden recurrence several years later
• Second, the patient with indolent long-standing lacrimal tu-mor
history presents with sudden expansion of the mass, as well as
pain and swelling of the upper eyelid.
• Third, the patient has rapidly developing symptoms of pain and
bone destruction, and the tumor is diagnosed as malignant at
the first presentation. De novo.
CT  an enlarged lacrimal fossa surrounded by bone destruction
means malignant tumors.
Pathology  Malignant mixed tumors have the histologic features
of a benign mixed tumor with areas of malignant change. In most
cases the malignant elements are poorly differentiated
adenocarcinoma.

KULIAH PAKAR dr. ALFA, SpM

 MALIGNANT MIXED TUMOR
• When preoperative evaluation indicates a malignant
Tumor  transseptal biopsy followed by complete
removal of the tumor  a one-stage procedure for the
surgical removal of the tumor and adjacent adnexa:
an en bloc resection of the neoplasm, its peri-orbital
base, and surrounding bone.
• A radical orbitec-tomy with regional and cervical lymph
node dissec-tion has been advised because an
adenocarcinoma arising in a benign mixed tumor may
disseminate early via lymphatics.
• Postoperative radiotherapy
• If metastases have occurred, treatment is often limited
to surgical debulk-ing followed by postoperative
radiotherapy.

KULIAH PAKAR dr. ALFA, SpM

MALIGNANT MIXED TUMOR
• Even with extensive surgery, patient mortality re-
mains high. 30% of patients died of a tumor at 5 years,
45% at 11 years, and 50% by 12 years.

KULIAH PAKAR dr. ALFA, SpM

ADENOID CYSTIC CARCINOMA
• the second most common epithelial tumor of the
lacrimal gland and the most common malignant
epithelial tumor of the lacrimal gland
• 1.6% of all or-bital tumors and 3.8% of all primary
orbital tumors
• indicated for any unilateral mass in the upper temporal
quadrant
• rapid temporal sequence usually under 1 year, pain,
globe displace-ment, mass or swelling, numbness,
diplopia, visual change, lacrimation, and ptosis

KULIAH PAKAR dr. ALFA, SpM

ADENOID CYSTIC CARCINOMA
• High-resolution CT  elongated mass extending along
the lateral orbital wall, with expansion of the lacrimal
fossa with bone invasion.
• High-resolution CT with bone windows is
recommended.
• Contrast enhancement helps to reveal involvement of
the dura and intracranial extension.
• MRI with enhancement is best for assessing the
invasion of the tumor into the cavernous sinus, brain,
and bone marrow.

KULIAH PAKAR dr. ALFA, SpM

ADENOID CYSTIC CARCINOMA
• Tumor re-moval and postoperative radiotherapy
comprise the most common treatment
• Surgical strategy  local resection, en bloc re-moval,
exenteration, and radical exenteration (radical
orbitectomy)
• Radical exenteration with re-moval of the orbital roof,
the lateral wall, and the an-terior portion of the
temporalis muscle where the zy-gomaticofrontal and
zygomaticotemporal nerves
• Patients usually die of intracra-nial spread as a result
of perineural invasion and pulmonary metastasis

KULIAH PAKAR dr. ALFA, SpM

ADENOID CYSTIC CARCINOMA
• Radiation therapy in the dose of 50 to 60 Gy after local
resection of adenoid cystic carcinoma significantly
delays the onset of tumor recurrence and prolongs
survival
• Implanted sources of radiation (brachytherapy)  no
evidence for long time results
• Intra-arterial chemotherapy for supplemental
management
• Intracarotid cisplatin and intravenous doxorubicin

KULIAH PAKAR dr. ALFA, SpM

KULIAH PAKAR dr. ALFA, SpM
‫ن‬‫م ن‬ ‫م م‬ ‫جك ك ن‬ ‫خجر ج‬ ‫ه أج ن‬ ‫والل ل ك‬‫ج‬
‫م جل‬ ‫ك‬ ‫بك ك‬
‫هات مك ك ن‬ ‫م ج‬ ‫نأ ل‬ ‫طو م‬
‫ل‬ ‫ع ج‬ ‫ج ج‬ ‫و ج‬ ‫شي نئئا ج‬ ‫ن ج‬ ‫مو ج‬ ‫عل ج ك‬‫تج ن‬
‫ج‬
‫صاجر‬ ‫وانلب ن ج‬ ‫ع ج‬ ‫م ج‬ ‫س ن‬ ‫م ال ل‬ ‫ل جك ك ك‬
‫م‬ ‫عل لك ك ن‬ ‫فئ مدجةج ل ج ج‬ ‫وانل ج ن‬ ‫ج‬
( 78 ) ‫ن‬ ‫شك ككرو ج‬ ‫تج ن‬
 
Dan Allah
mengeluarkan
kamu dariperut
ibumu dalam
keadaan tidak
mengetahui
sesuatupun. Dan
Dia memberi kamu
pendengaran,
penglihatan dan
hati agar kamu
bersyukur. (An
KULIAH PAKAR dr. ALFA, SpM