This presentation contains information about tumours of eyelids and orbits.

1. TUMOURS OF EYELID AND ORBIT
Presented by:
Rojita Bajracharya
Msc. Optometry, 1st sem
Himalaya Eye Institute

2. DEFINITION OF TUMOUR
 A mass of abnormal tissue that arises without obvious cause from pre-existing
body cells, has no purposeful function, and is characterized by a tendency to
independent and unrestrained growth

3. TUMOURS OF EYELIDS
Benign tumours Pre-malignant
tumours
Malignant tumours
• Simple papilloma
• Naevus
• Angioma
• Xanthelasma
• Keratoacanthomas
• Haemangioma
• Neurofibroma
• Sebaceous adenoma
• Solar keratosis
• Carcinoma in situ
• Xeroderma pigmentosa
• Squamous cell carcinoma
• Basal cell carcinoma
• Malignant melanoma
• Sebaceous adenocarcinoma

4. CLINICAL EVALUATION OF EYELID TUMOURS
 History taking
-Prior skin cancer or conditions
-Excessive sun exposure, sunburn
-Previous radiation therapy
-Smoking
-Ancestry (Fair skin, red hair, blue eyes)
-Immunosuppression

5.  Physical examination
-Notice painless growth of a lesion
-Palpable induration extending well beyond visibly apparent margins suggests tumour infiltration into the dermis
and subcutaneous tissue.
-Lesions near puncta should be evaluated for punctal or canalicular involvement. Probing and irrigation may be
required to exclude lacrimal system involvement or to prepare for surgery.
-Large lesions should be palpated for evidence of fixation to deeper tissues or bones.
-Regional lymph nodes should be palpated for evidence of metastases in case of suspected malignant tumours.
Rubbery swelling of lymph nodes may be noted in case of metastasis.
-Restriction of ocular motility and proptosis suggest orbital extension.

6. BENIGN TUMOURS
1. Papillomas
• Most common benign tumours arising from surface epithelium
• Occur in two forms viz:
a. Squamous papillomas:
 Derived from squamous cells
 Very slow growing/stationary raspberry-like growths or pedunculated lesion in lid margin (generally)
 Non-specific or related to human papilloma virus (verruca vulgaris/ viral wart)
 Treatment: Simple excision
b. Seborrhoeic papillomas:
 Derived from basal cells; in middle aged and older persons
 Surface is friable, verrucous and slightly pigmented

7. BENIGN TUMOURS
2. Naevus
 Common cutaneous lesions that arise from the arrested epidermal melanocytes
 Depending upon the depth of involvement, naevi are of three types:
a. Junctional naevi: These are located at the epidermis/dermis and are flat and brown
in appearance.
b. Dermal naevi: These are located within the dermis, are elevated lesions which may
not be visibly pigmented.
c. Compound naevi: These are slightly elevated and share features of junctional and
dermal naevi.

8. BENIGN TUMOURS
3. Haemangioma: These are common eyelid tumours that occur in three forms viz:
i. Capillary haemangioma: Most common variety that occurs at or shortly after birth
-Grows rapidly but also resolves by self by the age of 7 years
-Superficial
-Bright red in color (strawberry naevus) or deep bluish or violet in
colour
-Consists of proliferating capillaries and endothelial cells
-Treatment: Only if it doesn’t resolve on its own. Excision,
intralesional steroid (triamcinolone), high dose oral steroid therapy
alternate days regimen or superficial radiotherapy

9. ii. Naevus flammeus (port wine stain)
 It may occur pari passu or more commonly as a part of Sturge-Weber syndrome.
 It consists of dilated vascular channels
 It doesn’t grow or regress like the capillary haemangioma
iii. Cavernous haemangioma
 Developmental venous anomaly and usually occur after first decade of life
 It consists of large endothelium-lined vascular channels.
 Shows no regression
 Treatment: Excision, Steroid therapy, Superficial radiotherapy

10. BENIGN TUMOURS
4. Xanthelasma
 Creamy yellow plague like lesions which frequently
involve the skin of upper and lower lids near the inner
canthus
 Common in middle aged women
 Lipid deposits in dermis of lids
 Possible associations: DM or high cholesterol levels
 Treatment: Excision, recurrences are common

11. BENIGN TUMOURS
5. Keratoacanthomas
 Nonpigmented protrusions with a keratin filled central crater
 Uncommon
 Rapid growth for 2 to 6 weeks and then spontaneously involute over a
few months
 May appear similar to squamous cell carcinoma
 Treatment: Complete excision and biopsy

12. BENIGN TUMOURS
6. Neurofibroma
 Lids and orbits are commonly affected in neurofibromatosis (von
Recklinghausen’s disease)
 Von Recklinghausen’s disease or NF1 is a genetic disorder characterized by
increased risk of developing benign and malignant tumours as well as other
physical and neurological manifestations
 Usually of plexiform type

13. BENIGN TUMOURS
7. Sebaceous adenoma
 Rare tumour
 Yellowish papule on the face, scalp, or trunk
 May mimic a basal cell carcinoma or seborrheic
keratosis

14. PREMALIGNANT TUMOURS
1. Actinic(Solar) Keratosis
 Commonly due to sun exposure
 Uncommon in eyelids
 Flat, scaly lesion with hyperkeratosis with or
without keratin horn

15. PREMALIGNANT TUMOURS
2. Xeroderma pigmentosa
 Autosomal recessive disease
 Progressive cutaneous pigmentation resulting from damage on exposure to
natural sunlight
 Bird like facies
 Predisposition to develop lid tumours (basal cell carcinoma, squamous cell
carcinoma and melanoma) and conjunctival malignancies

16. MALIGNANT TUMOURS
1. Squamous cell carcinoma
 Second common malignant tumour of lids
 Commonly arises from the lid margin (mucocutaneous junction) in elderly patients
 Occurs fresh or from pre-existing lesion such as actinic keratosis, Bowen’s disease and radiation dermatosis
 Affects lower lids more frequently and commonly in males
 Risk factors: Sun exposure, radiation, fair skin, injury, irritative insults
 Clinical presentation: Either ulcerated, scaly, erythematous plaque like growth with elevated margins or
fungating or polypoid verrucous lesion without ulceration.
 Metastasis: Preauricular and submandibular lymph nodes
 Treatment: Surgery/Radiotherapy/Cryotherapy

17. MALIGNANT TUMOURS
2. Basal cell carcinoma
 Most malignant tumour of the lids (90%)
 Usually seen in elderly people
 Locally malignant and involves most commonly lower lid (50%) followed by medial canthus (25%), upper lid (25%) and outer
canthus (5-10%)
 Predisposing factors: Increasing age, white skin, sun exposure, xeroderma pigmentosa and basal cell naevus syndrome
 Clinical features: It may present in four forms viz
i. Non-ulcerated nodular form
ii. Sclerosing or morphae type
iii. Pigmented basal cell carcinoma
iv. Noduloulcerative basal cell carcinoma
 Small nodule—Central ulceration with pearly rolled margins—Burrowing and destroying of tissues locally like a rodent---hence also
known as RODENT ULCER
 Treatment: Surgical excision of tumour alongwith 3 mm surrounding area of normal skin with primary repair, radiotherapy.
cryotherapy

18. MALIGNANT TUMOURS
3. Malignant Melanoma (Melanocarcinoma)
 Rare (less than 1% of all eyelid lesions)
 May arise from a pre-existing naevus, but usually arises de novo from the melanocytes present in the skin
 Clinical features:
-Present in three forms viz lentigo maligna type, superficial spreading type and nodular type
 Metastases via bloodstream and lymphatics
 Treatment: As it is radio-resistant tumour, treatment of choice is surgical excision with 10 mm margins with lid
reconstruction.

19. MALIGNANT TUMOURS
4. Sebaceous gland carcinoma
 Rare tumour arising from meibomian glands (western literature)
 Commonest malignancy of eyelid followed by basal and squamous cell carcinoma
(Indian literature)
 Clinical feature: Usually presents as a nodule (mistaken for chalazion) more
frequently on the upper eyelid which then grows to form a big growth. Rarely
diffused along the lid margin (may be mistaken as chronic blepharitis)
 Treatment: surgical excision with reconstruction of lids. Recurrences are common.

20. ORBITAL TUMOURS
Primary tumours
Secondary tumours
Metastatic tumours

21. PRIMARY TUMOURS
1. Developmental tumours
2. Vascular tumours
3. Adipose tissue tumours
4. Fibrous tissue tumours
5. Osseous and cartilaginous tumours
6. Mesenchymal tumours
7. Neural tumours
8. Tumours of lacrimal gland
9. Lymphoproliferative disorders

22. 1. DEVELOPMENTAL TUMOURS
DERMOIDS EPIDERMOIDS LIPODERMOIDS TERATOMAS

23. 1. DEVELOPMENTAL TUMOURS
A. Dermoids
 Common
 Arise from an embryonic displacement of the epidermis to a subcutaneous location
 Lined with keratinizing epithelium
 Probable presence of one or more dermal adnexal structures such as hair follicles and sebaceous glands
 Two types viz:
a. Superficial dermoids: infancy/not associated with bony defects/no proptosis
b. Deep dermoids: adolescence/associated with bony defects/proptosis
 Treatment: Surgical excision

24. 1. DEVELOPMENTAL TUMOURS
B. Epidermoid
 Composed of epodermis
 No epidermal appendages in the wall of the cyst
 Almost always cystic
 Cyst wall contains keratin debris
 Treatment: Surgical excision

25. 1. DEVELOPMENTAL TUMOURS
C. Lipodermoids
 Solid tumours usually seen beneath the conjunctiva
 Generally located adjacent to the superior temporal quadrant of the globe
 No surgical intervention is required unless significant enlargement

26. 1. DEVELOPMENTAL TUMOURS
D. Teratomas
 Composed of ectoderm, mesoderm and endoderm
 Solid, cystic or mixture of both; however cystic form is more prevalent
 Mostly benign but some solid tumours in newborns are malignant
 Treatment: Exenteration for solid tumours
Excision for cystic tumours without removing the eyeball

27. 2.VASCULAR TUMOURS
HAEMANGIOMAS
CAPILLARY CAVERNOUS
LYMPHANGIOMAS
Most common primary benign
tumours of the orbit

28. 2. VASCULAR TUMOURS
A. Haemangiomas
 Abnormal growth of blood vessels
i. Capillary haemangioma:
 Commonly seen at birth or during the first month
 Periocular swelling in the anterior part of the orbit
 Increases in size on straining or crying
 Initial growth—Stabilization—Regression—Disapperance
 Treatment: Not required as it’s self resolving
 Indications for treatment: Optic nerve compression, exposure keratitis, ocular
dysfunction or cosmetic blemish
 Modes of therapy: steroids (systemic/intralesional), radiations, surgery, cryotherapy, systemic beta
blockers like propranolol

29. ii. Cavernous haemangioma:
 Commonest benign orbital tumour among adults with female
preponderance (70%)
 Usually located in the retrobulbar muscle cone
 Unilateral axial proptosis in the second to fourth decade
 May cause optic nerve compression without causing proptosis
 Treatment: Surgical excision (Lateral orbitotomy)

30. 2. VASCULAR TUMOURS
B. Lymphangiomas
 Uncommon tumour, young person
 Slowly progressive proptosis
 Often enlarges due to spontaneous bleed within
the vascular spaces, forming ‘chocolate cysts’
which may regress spontaneously

31. 4. FIBROUS TISSUE
TUMOURS
FIBROMA FIBROSARCOMA FIBROMATOSIS

32. 5. OSSEUS AND CARTILAGINOUS TUMOURS
OSTEOMA
CHONDROMA
OSTEOBLASTOMA
OSTEOGENIC SARCOMA AFTER IRRADIATION
FIBROUS DYSPLASIA OF BONE
EWING’S SARCOMA

33. 6. MESENCHYMAL
TUMOURS
RHABDOMYOSARCOMA
FIBROUS
HISTIOCYTOMA

34. 6. MESENCHYMAL TUMOURS
A. Rhabdomyosarcoma
 Highly malignant tumour
 Arises from the pluripotent mesenchymal cells—striated muscles
 Most common primary orbital tumour among children; 90% occurrence below 15 years
 Slightly common in males
 Clinical feature:
Classically presents as rapidly progressive proptosis of sudden onset
Mimics an acute inflammatory process
Commonly involves superonasal quadrant but may invade any part of the orbit

35. RHABDOMYOSARCOMA
Diagnosis:
i. X-rays: Shows bone destruction
ii. CT/MRI scan: Irregular but well defined tumour with adjacent bone destruction
iii. Biopsy: Confirmational test
Treatment:
Surgical excision biopsy
Chemotherapy
High dose radiation therapy (5000 rads in 5 weeks)
Exenteration

36. 7. NEURAL TUMOURS
OPTIC NERVE GLIOMA
OPTIC NERVE SHEATH MENONGIOMA
NEUROFIBROMA
NEUROFIBROMATOSIS
SCHWANNOMA

37. Optic nerve glioma
 Slow growing tumour arising from the astrocytes.
 Occurs in first decade of life
 May present as solitary or as a part of von Recklinghausen’s neurofibromatosis (30%)
 Involvement of only optic nerve (28%), optic chiasma (72%), often with mid brain and hypothalamic involvement
 Clinical feature: Gradual, painless, unilateral axial proptosis associated with vision loss and an afferent pupillary
defect. Other ocular findings may include optic atrophy, disc swelling, nystagmus and strabismus. Intracranial
extension of glioma through optic canal is not uncommon.
 Diagnosis: X-ray shows uniform rounded enlargement of optic foramen in 90% of cases.
CT and MRI shows fusiform enlargement of optic nerve often with kinking of the nerve
MRI may also show cystic degeneration, if present
 Treatment: Surgical excision or Radiotherapy

38. Optic Nerve sheath meningioma
 Primary intraorbital meningiomas
 Rare benign tumour of meningothelial cells of the meninges
 Occurs in mid age usually
 Slight female preponderance
 Early visual loss associated with limitation of ocular movements, optic disc oedema or atrophy
and a slowly progressive unilateral proptosis
 Presence of optocilliary shunt is pathognomic
 Diagnosis: CT shows distinct fusiform thickening of optic nerve
 Treatment: Observation only if vision is good
 Surgical excision for severe proptosis with blind eye or threat to chiasma

39. 8. TUMOURS OF LACRIMAL GLAND
A. EPITHELIAL TUMOURS
B. NON-EPITHELIAL TUMOURS

40. 9. LYMPHOPROLIFERATIVE DISORDERS
A. BENIGN REACTIVE LYMPHOID HYPERPLASIA
B. MALIGNANT ORBITAL LYMPHOMAS
C. LANGERHAN CELL HISTIOCYTOSIS
D. PLASMA CELL TUMOURS
E. XANTHOGRANULOMA

41. SECONDARY TUMOURS
1. Tumours of eyeball: RETINOBLASTOMA, malignant melanoma
2. Tumours of the eyelids: Squamous cell carcinoma, basal cell carcinoma
3. Tumours of nose and paranasal sinuses: Carcinomas, Sarcomas, osteomas
4. Tumours of nasopharynx: Carcinoma of nasopharynx
5. Tumours of cranial cavity invading orbit: Glioma, Meningioma

42. METASTATIC ORBITAL TUMOURS
 Haematogenous spread from a distant primary focus
Children Adults
Neuroblastoma Carcinoma
Nephroblastoma Malignant melanoma
Ewing’s sarcoma
Leukaemia infiltration
Testicular embroynal or
ovarian sarcoma

43. Most common orbital tumours
Tumour Children Adult
Primary benign Dermoid cyst Cavernous haemangioma
Primary malignant Rhabdomyosarcoma Lymphoma
Secondary tumour Retinoblastoma Squamous cell carcinoma
Metastatic tumour Neuroblastoma Carcinoma breast (females)
Carcinoma lungs (males)

44. References
 Comprehensive Ophthamology by A. K Khurana
 American Academy of Ophthalmology
 Internet

45. THANK YOU