TUMOR

EYELID
1. EPIDERMAL
A. ACTINIC KERATOSIS
- lesi skuamosa prekanker
- pada middle age
- klinis : eritema, scaly macules or papules on sun-exposed skin (face, dorsal surface of the hands)
- histopato : epidermis with hyperkeratosis and parakeratosis

B. BASAL CELL CARCINOMA

- The most common eyelid malignancy 90-95%.
- medial canthus (48.3%), lower eyelid (47.5%), and upper eyelid (3.9%).
- RIsks : fair-skinned, blue-eyed, red- haired or blond, and middle-aged and elderly persons with English, Irish, Scottish, or
Scandinavian ancestry, prolonged sun exposured, smoking.
- Associated : basal cell nevus (Gorlin syndrome) or xeroderma pigmentosum
Types Clinis Histopatology

Nodular (most firm, raised, pearly nodule that may be associated with
common) telangiectasia and central ulceration nests of basal cells that originate from the
basal cell layer of the epithelium and may
show peripheral palisading

Morpheaform (less
common, but more thin cords that radiate peripherally rather
aggressive) than peripheral palisading. The surrounding
stroma may show fibrotic proliferation of

firm and flat or slightly elevated pale yellow indurated plaque,

with margins that may be indeterminate on clinical examination

connective tissue.
Ulcerative

Pigmented

Management :
1. Biopsy : bisa insisi or excisi (kalau kecil and do not involved the eyelid margin, canthus, punctum)
2. Surgical excision with frozen section techniques atau untuk konfirmasi complete removal, bisa dilakukan Mohs
micrographic surgery.
3. Reconstructions dilakukan setelah dipastikan complete excision
4. Radiation → untuk palliative treatment

C. SQUAMOUS CELL CARCINOMA, KERATOACANTOMA

- less common than BCC
- lebih sering di lower eyelid, tapi lebih sering melibatkan upper eyelid dibanding BCC
- terapi SCC : frozen section (conventional or Mohs), terapi keratoacanthoma : jika tidak involusi spontan, eksisi
- prognosis buruk jika ada perineural dan lymphatic invasion

types klinis histopatologi

SCC ranging from ulcer to plaque to nodular growth atypical squamous cells that form nests and strands,
keratin pearl
 madarosis extend beyond epidermal basement membrane, infiltrate
scaly appearance dermis, incite fibrotic tissue reaction

keratoacantoma rapidly growing invaginasi berbentuk cup dengan well differentiated

potential for spontaneous involution → skar squamous cell (squamous eddies)
dome shaped nodule, dengan keratin-filled central acanthotic projections
craters
RF : imunosupresi
 2. DERMAL
A. HEMANGIOMA, SYRINGOMA, MCC

types klinis histopatologi

hemangioma muncul shortly after birth early : solid nest of plump endothelial cells, little vascular lumen
bright red lesion, growth over weeks to months, formation
involute by school age lanjutan : well-developed capillary channels lined with plump
endothelial cells in a lobular configuration
involusi : fibrosis dan hialinisasi dinding kapiler dengan oklusi lumen

terapi :
- beta-blocker (1st line)
eksisi
merkel cell ca
 berasal dari
neuroendocrine
RF : infeksi merkel cell
polyomavirus + UV +
imunosupresi
monotonous round, blue tumor cells, with scant eosinophilic
red/violaceous nodule
cytoplasm, vesicular nuclei with finely granular chromatin, and
dengan permukaan telengiektasia
multiple nucleoli
terapi :
- wide local excision (minimal 5mm dari
margin)
- biopsi regional lymph node
- resection dengan frozen section atau
Mohs
- radiation / and chemothrapy
prognosis : HIGH MORTALITY
- risiko metastasis 30%, rekurensi (+)

B. SEBACEOUS CGLAND ARCINOMA

Nama lain Sebaceous gland carcinoma, sebaceous cell carcinoma, sebaceous adenocarcinoma, meibomian gland carcinoma

Origins - meibomian glands of the tarsal plate;

- glands of Zeis associated with the eyelashes;
- sebaceous glands of the caruncle, eyebrow, or facial skin.

Most frequent Women, upper eyelid > lower eyelid (6-8%)

>50 years old (tapi ada juga di younger patients)

Klinis - Menyerupai chalazia, chronic blepharitis, basal cell or squamous cell carcinoma, mucous membrane (ocular
cicatricial) pemphigoid, superior limbic keratoconjunctivitis, or pannus associated with adult inclusion
conjunctivitis
- Yellow (krn ada lipid material within the neoplasic cell)
- Madarosis!

Histopatologi - Intraepidermal : extend over the palpebral and bulbar conjunctiva → PAGETOID SPREAD
- Intraepithelial : replace corneal epithelium → Marked conjunctival inflammation and hyperemia may be
present

DD Muir-Torre syndrome (MTS) → autosomal dominant condition of sebaceous tumors (including sebaceous carcinoma, sebaceous
adenoma, and basal cell epithelioma with sebaceous differentiation) involving the gastrointestinal, endometrial, or urologic
systems.

Diagnostic Full thickness biopsy with permanent sections or full-thickness punch biopsy of the tarsal plate

Management
- Wide surgical excision
 - Mohs micrographic surgery → lower local recurrence rates for periorbital sebaceous carcinoma
- Map biopsies (untuk lihat pagetoid spread), kalau sudah pagetoid → cryotherapy
- Orbital exenteration (recurrent or large tumors invading through the orbital septum)
- Sentinel lymph node biopsy → kalau dengan high risk features sbb recurrent lesions, or extensive involvement
of the eyelid [>10 mm] or orbit
-

Gambar

3. MELANOCYTIC NEOPLASMS
A. MELANOCYTIC NEVUS
B. MELANOMA
types klinis histopatologi

melanocytic nevus - terlihat sejak lahir atau baru terlihat

(common) saat dewasa nevus cells, specialized melanocytes that have a round rather than
- smooth dome shaped, dendritic shape and tend to cluster together in nests.
hyperpigmented/ blue nevi/ spitz
nevi/ dysplastic nevi
- macular lesions, evolve with age tipe lain
- junctional nevus : mirip freckle
1. combined nevus : combination of round and spindle-shaped
cells is seen in a lesion
Junctional 2. junctional nevus : nests of nevus cells in the epidermis, along
the dermal–epidermal junction
3. compound nevus : both junctional and intradermal components
are present
4. intradermal/ dermal nevus : nevus cells only within the dermis
tipe sel nevus :
1. tipe A : round to ovoid nuclei
(kissing nevus) 2. tipe B : cells are smaller, have less cytoplasm, and resemble
Compound
lymphocytes
3. tipe C : similar to Schwann cells of peripheral nerves with a
spindle configuration

Intradermal

melanoma - asimetris
(rare) - batas iregular pagetoid intraepidermal spread of atypical melanocytic nests and
- terjadi dari nevus - color variation single cells, nuclear abnormalities, lack of maturation in the deeper
- de novo - diameter > 6mm portions of the mass, and atypical mitotic figures
- extensi dari tumor lain - bisa metastasis ke regional LN dahulu
di wajah tipe :
1. lentigo maligna
- most common type occurring on eyelid
- irregular pigmented macular lesion
- atypical melanocytes proliferate along the dermal-epidermal
junction

2. superficial spreading
- most common type of cutaneous melanoma
- radial (intraepidermal) growth pattern

3. nodular
- significant vertical growth phase that results in a raised or
indurated mass
ORBITAL
1. LACRIMAL GLAND NEOPLASM

Epithelial tumor

Pleomorphic adenoma / benign mixed tumor Adenoid cystic carcinoma Malignant mixed tumor

Epidemiology 50% of epithelial tumors Most common malignant epithelial tumor Arise from long standing pleomorphic adenoma /
has recurred after initial incomplete excision of
pseudocapsule

Risk factors - Adults 4th-5th decade of life -woman>man

- Men>women

Characteristic - Progressive -Rapid course < 1 year

- Axial proptosis -early onset of pain
- Painless inferior & medial displacement of the globe
- Firm, lobular mass near superolateral
 Imaging Well circumscribed, slightly nodular. -Tumor usually extend into posterior orbit
No bone destruction -Its lack of true encapsulation
-Bone destruction

microscopically Circumscribed by fibrous pseudocapsule Benign-apperaring cells that grow in tubules Same with pleomorphic adenoma
Proliferation of benign epithelial cell & stroma Increase in growth rate is hallmark of malignant
composed of spindle-shaped cells with occasional degeneration
cartilaginous, mucinous, osteoid degeneration /

, solid nests / Swiss-cheese pattern

metaplasia
-Positive with IHC for keratin, keratin, actin, myosin,
fibronectin, S-100 protein in myoepithelial areas
(cribiform)
Basaloid
 Infiltration of orbital tissue (perineural)

-Positive IHC for S-100 protein, keratin, actin

within area of myoepithelial diff

Management Complete removal with its pseudocapsule and -biopsy

surrounding margin of orbital tissue -Traditional : orbital exenteration / radial orbitectomy + radiotherapy
Without preliminary biopsy - other : neoadjuvant intra-arterial chemotherapy à exenteration or eye-sparing surgery à radiation
(typically proton) therapy.

Prognosis Risk become malignant 10% per decade Morphology :

-Basaloid à poor prognosis (survival rate 20%)
-Cribiform (most common)
-Comedo
Sclerosing
Tubular / ductal
Expression of p53 à poor prognosis
Bcl-2 & BAX à good prognosis

2. LYMPHOPROLIFERATIVE LESION

Ocular adnexal lymphoproliferativelesions :

- Reactive lymphoid hyperplasia (RLH)
- Atypical lymphoid hyperplasia (ALH)
- Ocular adnexal lymphoma (OAL)
Reactive lymphoid
hyperplasia (RLH)
manifestation Slow expansion
Painless
anteriorly in the orbit /
beneath the conjunctiva
Imaging
Histopatology Prominent lymphoid follicles
with germinal centers,
tingible body macrophages
(containing apoptotic debris),
and polymorphous population
of mature lymphocytes
Atypical lymphoid Lymphoma maligna
hyperplasia (ALH)
Gradual Gradual
Painless Painless
anteriorly in the orbit / Systemic lymphoma
beneath the conjunctiva Non Hodgkin low-grade B cell
tumors
Types :
-extranodal marginal (EMZL)
-Follicular (FL)
-Diffuse large B-Cell (DLBCL)
-Mantle cell (MCL)
admixture of small, Monomorphic sheet of
mature-appearing lymphocytes and are
lymphocytes and larger composed of B cells with
lymphoid cells of unknown immunopositivity for CD19
maturity and CD20.
 Treatments
NON EMZL :
- Chemotherapy, immunomodulation, radiotherapy

Prognosis EMZL & FL good outcomes

DLBCL & MCL poor outcomes

3. SOFT TISSUE TUMOR

histopatologi : round cell, spindle cell, myxoid, epithelioid, pericytomatous, and pleomorphic

4. VASCULAR TUMOR

type klinis PA
lymphangioma - in childhood
- recurrent and fluctuating proptosis unencapsulated, diffusely infiltrating proliferations of vascular
- enlarging saat URTI channels with lymphatic differentiation and scattered lymphoid
aggregates with a fibrotic interstitium

hemangioma - in adults
- terletak intraconal cavernous : encapsulated, consisting of large, irregular cavernous spaces
with relatively thin, fibromuscular walls

infantile : unencapsulated and more cellular, often with a cutaneous

component, and are composed of capillary-sized vessels
5. FIBROUS DIFFERENTIATION : solitary fibrous tumor
- most common mesenchymal tumor of the orbit in adults
- common site : superonasal orbit
- 5th decade of life
- manifestasi : proptosis, pain, diplopia, blurred vision, epiphora
- PA : staghorn vessels, spindle shaped, plump histiocyte-like cells with a focal storiform architecture. giant cell angiofibroma was
distinguished by the presence of multinucle- ated floret-type giant cells and ectatic vascular spaces.

-
- prognosis : benign, rekuren (+)
 6. MUSCLE DIFFERENTIATION

type klinis histopatologi

rhabdomyosarcoma -most common primary malignant tumor of berasal dari sel mesenkia yang diferensiasi menjadi otot skeletal. tipe :
childhood -embryonal (most common) : grapelike submucosal clusters (ie, botryoid
-onset 5-8 tahun variant), spindle cells are arranged in a loose syncytium with occasional
-rapid onset elongated cells demonstrating cytoplasmic cross-striations (strap cells)
-progressive unilateral proptosis
-reddish discoloration of the eyelid, no local
heat or fever (beda dg cellulitis)

- -alveolar (worst prognosis) : Poorly cohesive rhabdomyoblasts separated by fibrous

septa (arrows) into “alveoli”

-pleomorphic (best prognosis, least common)

leiomyoma -benign, slowly progressive unilateral -diferensiasi jadi smooth muscle
proptosis -spindle cell tumors show slender blunt-ended, cigar-shaped nuclei and
-dekade 4-5 trichrome-positive filamentous cytoplasm

leiomyosarcoma -dekade 7 -more cellularity, necrosis, and nuclear pleomorphism, mitotic figure

7. PERIPHERAL NERVE SHEATH AND CNS TUMOR

Neurofibroma Neurilemoma/Schwannoma Meningioma

Epidemiology Most common peripheral nerve most common nerve sheath tumors found in the
sheath tumor orbit of adults

Source and - mixture of endoneurial
histology fibroblasts, Schwann cells, and
axons
- spindle-shaped cells have
slender, wavy nuclei.
- From Schwann cell of peripheral nerves
- Antony A pattern
slender spindle cells in which the nuclei are
arranged in palisades that may form intervening
Verocay bodies
- Derived from meninges
- primary tumors of the
intracranial cavity that invade
through the orbital bones, typically
the sphenoid bone

- Antony B pattern
stellate cells set in a mucoid stroma

Clinical Circumscribed, not capsulated, - Slow growing, encapsulated, yellowish Usually invade bone esp shenoid
appearance firm, and rubbery - Show cystic spaces and hemorrhagic bone
necrosis
 Soliatary / associated with neurofibromatosis or
Schwannomatosis

Type Plexiform
- Pathognomonic for NF1
- Intricate network -> “Bag
of worms”

S-shaped deformity of upper

eyelid
 Tortuous nerves with
proliferation of fibroblast and
Schwann cells

ADIPOSE TUMOR BONY LESIONS LACRIMAL SAC

NEOPLASIA
 Fibrous dysplasia - rare
Lipoma - in orbit usually monostotic (1 bone), but may cross suture lines involving - may mimic
multiple orbital bone dacryocystitis
- encapsulation and
- Narrowing of the optic canal and lacrimal drainage system can occur. - Most lacrimal sac
a distinctive lobular
appearance - Radiographic ground-glass appearance with lytic foci tumors are benign
- Histology: papillomatous neo-
- difficult to Trabeculae composed of of woven bone with a fibrous stroma that is highly plasms that arise from
distinguish from vascularized (C-shaped) the epithelial lining
normal tissue

Liposarcoma

- presence of
lipoblasts

Rare in orbit

Fibroosseous dysplasia
(juvenile ossifying fibroma)

Histology : spicules of bone rimmed by osteoblasts

Osteoma

- Composed of mature bone, slow growing, well circumscribed

- Mostly arises from frontal sinus

- most common osseous tumor in orbit

Osteoblastoma -> bening

Osteosarcoma -> malignant

Secondary Tumors

Secondary malignant orbital tumor

lesions that invade the orbit by direct extension from adjacent structures, such as the
paranasal sinus, intracranial cavity, eye, eyelids, or ocular surface
 Metastatic tumor

malignant lesions that have spread from a distant primary site, usually by a
hematogenous route

- breast in women, prostate in men, neuroblastoma in children

OPTIC NERVE
1. MELANOCYTOMA OF ONH
2. GLIOMA
3. MENINGIOMA

klinis histopatologi

melanocytoma of ONH -benign, grow slowly -magnocellular nevus, plump polyhedral

-heavily pigmented melanocytic tumor eccentrically on ONH melanocytes
adjacent choroid
glioma (astrocytoma) -bisa di sepanjang visual pathway
-berhubungan dengan NF1
-muncul di dekade pertama kehidupan
-fusiform or sausage-shaped enlargement within sheath
-duramater intak
-proliferasi spindle-shaped astrocytes
-hairlike cytoplasmic processes
-rosenthall fibers

meningioma -berasal dari lapisan arachnoid ON sheath -meningothelial dengan syncytial growth pattern
-bisa juga berhubungan dengan NF arranged in whorl
-bisa invasi dura ke orbit dan EOM -psamoma bodies
-tram track sign

INTRAOCULAR
1. UVEAL NEVI
2. UVEAL MELANOMA
3. UVEAL MELANOCYTOMA
 - Nama lain : Manocellular nevus
- Benign
- Muncul dimana saja pada uveal tract tapi paling sering di peripapillary region
- Histopatology : large polyhendral cells with small round to oval nuclei and abundant cytoplasm
- Pada kasus yang jarang uveal melanocytoma bisa berubah menjadi melanoma

4. EPITHELIAL TUMORS OF UVEA AND RETINA

A. ADENOMA, ADENOCARCINOMA
ADENOMA / Benign adenoma
- Bisa terjadi pada Siliary epitelium yang pigmented dan nonpigmented muncul pada ciliary body (jarang pada RPE)
- Sulit dibedakan dengan melanoma
- Jarang membesar dan menjadi malignant
ADENOCARCINOMA
- Jarang pada RPE → bila iya akan tampak feeder retinal vessel pada lesi exudate kekuningan
- dd/ Fuchs adenoma / pseudoadenomas hyperplasia
B. ACQUIRED HYPERPLASIA / Adenomatous hyperplasia

- Muncul sebagai respon dari post trauma, inflamasi, ocular insults

- muncul dan mimicing choroidal melanoma
 C. HAMARTOMA
Terdiri dari : Simple hamartoma dan combined hamartoma
SIMPLE HAMARTOMA

-> simple hamartoma

- muncul dengan gambaran lesi RPE kecil < 1 mm, sharply dermacated, pigmented trasnretinal lesion dekat dengan macula.
- Lesi muncul dari RPE dan tidak berubah seiring waktu

COMBINED HAMARTOMA

-> combined hamartoma of retina dan RPE

- Muncul dekat dengan optic nerve margin meskipun bisa muncul pada peripheral.
- Lesi pigmented dengan slightly elevated with vitreoretinal traction dan tortuous retinal vessel
- Complication : exudate

VASCULAR TUMORS
 1. CHOROIDAL HEMANGIOMA
2. RETINA
A. RETINAL CAVERNOUS HEMANGIOMA
B. RETINAL ARTERIOVENOUS MALFORMATION
C. RETINAL CAPILLARY HEMANGIOBLASTOMA
D. RETINAL VASOPROLIFERATIVE TUMOR

TUMOR METASTASIS
1. NEUROBLASTOMA
Terjadi 10-20% kasus
Bilateral
CT Scan → destrukti tulang, terutama dinding orbita lateral atau sphenoid marrow
Metastasis dapat terjadi jika tumor primer dideteksi di perut, mediastinum atau leher
Manajemen à kemoterapi, radioterapi (untuk kasus vision loss dengan compressive optic neuropaty).
Pasien yang didiagnosis < 1 tahun memiliki prognosis yang lebih baik (kelangsungan hidup 5 tahun/85%) dibandingkan anak yang
didiagnosis >1 tahun (kelangsungan hidup 5 tahun/36%). Rata-rata pasien dengan metastasis orbita usianya 2 tahun.
 2. METASTASIS DARI CA MAMMAE, BRONCHOGENIC, PROSTATE

Metastatic Breast carcinoma Bronchogenic Prostate carcinoma

Tumor in Adults carcinoma

Most common primary Most frequent

source orbital metastases in origin orbital tumor
woman in man

Waktu Terjadi tahunan setelah

metastasis breast lesion removed
(history cancer surgery
penting)
Manifestasi Menyebabkan fibrous: Primary lesion:
acute nonspecific
klinis enophthalmos, restriction quite small.
orbital
ocular motility
CT of suspicious lung inflammation
lesions may be
osteoblastic bone
performed in
lesion is identified
patients suspected of
on imaging
having orbital
metastases.

Terapi Respon terhadap hormone

terapi, berhubungan
dengan adanya estrogen
dan hormon lain di
jaringan tumor.

Management of - Usually palliative, consisting of local radiation therapy.

Orbital
Metastases
 - Consultation with the patient’s oncologist is important for
identifying candidates who might benefit from wide excision.

RETINOBLASTOMA