This document discusses various ocular and orbital tumours. It begins by providing an overview of orbital anatomy and the effects of space-occupying lesions. It then discusses the most common paediatric and adult orbital tumours. Specific tumour types are examined in more detail, including retinoblastoma, rhabdomyosarcoma, lacrimal gland tumours, squamous cell carcinoma, and orbital metastases. For each tumour, the document outlines epidemiology, clinical presentation, investigations, differential diagnosis, and treatment approaches.

1. Ocular and Orbital Tumours
Imo Eustace
Moradeyo Oyindamola

2. Introduction
• The orbit is an anatomically complex structure containing the globe;
extraocular muscles; fat; and vascular, nerve, glandular, and
connective tissues.
• The orbit describes the cavity containing structures essential for
ocular function and the bony architecture that encases them.
• Since the orbit is a relatively small anatomical area with little wasted
space, space-occupying lesions that increase orbital volume may
result in proptosis of the globe.
• May adversely affect visual and extraocular muscle function.

5. Epidemiology
• Dermoid cysts, capillary hemangiomas, and rhabdomyosarcoma are
the most common paediatric orbital tumours.
• Retinoblastoma is the most common malignant ocular tumour in
children.
• Neuroblastoma can involve the orbit via metastases and is the most
common metastatic tumor to the orbit in children.
• Lymphoid tumors, cavernous hemangiomas, and meningiomas are
the most common orbital tumours in adults.
• Other tumors include those of the lacrimal gland, tumors from the
surrounding sinuses, metastatic tumors such as breast cancer in
women, and neural-based tumors.

6. Ocular Tumour - Retinoblastoma
• Retinoblastoma is a malignant tumor of early childhood that develops
from immature retinal cells.
• It generally arises from a multipotential precursor cell (mutation in
the long arm of chromosome 13 band 13q14) that could develop into
almost any type of inner or outer retinal cell.
• Intraocular growth patterns – endophytic, exophytic, diffuse
infiltrating growth

7. Epidemiology
• Retinoblastoma is most common malignant ocular tumor in children.
• Occurring in approximately one of 20000 births. In 30% of all cases, it
is bilateral.
• Third most common intraocular tumour after melanoma and
metastasis.
• 90% of patient present before 3years of age.
• Occurs equally in males and females.
• Occurs equally in left and right eyes.

8. Epidemiology
• No racial predilection
• 60-70% are unilateral (mean age at diagnosis, 24months)
• 30-40% are bilateral ( mean age at diagnosis, 14months)
• Hereditary form is autosomal dominant with 90% penetrance.
• 60% non-hereditary and unilateral.
• 15% hereditary and unilateral
• 25% hereditary and bilateral

9. Clinical Presentation
• Cat’s eye reflex- leukocoria
• Strabismus
• Mass protruding through the eye
• Proptosis
• Loss of vision
• Secondary changes in the eye, including glaucoma, retinal
detachment, and inflammation secondary to tumor necrosis.

11. Investigations
• Ultrasound- size, calcification
• CT scan- calcification
• MRI- optic nerve, extraocular extension, pinealoblastoma
• X-ray- calcification
• Genetic studies- enucleated tissue, blood, sperm

12. Treatment
Early retinoblastoma can be treated with:
• Chemotherapy – Vincristine, Etoposide, Carboplatin
• Photocoagulation
• Cryotherapy
• Thermotherapy
• Brachytherapy
Late presentation:
• Enucleation
• Exenteration

13. Differential Diagnosis
• Congenital Cataract
• Exudative Retinal Detachment
• Pediatric Tuberculosis
• Retinopathy of Prematurity
• Vitreous Hemorrhage

14. Orbital Rhabdomyosarcoma
• Rhabdomyosarcoma (RMS) is a rare tumor, with an annual incidence
of 4.3 cases per million children.
• The orbit is the primary site in approximately 10% of these tumors.
• It is the most common malignant orbital tumor of childhood.
• Rhabdomyosarcoma of the orbit usually appears in the first decade of
life.
• The mean age at diagnosis is 8 years.
• Boys are affected more often than girls.
• A history of trauma may be associated with the clinical presentation
of this tumor

15. Rhabdomyosarcoma
• Orbital RMS develops from undifferentiated mesenchymal cells that
have the capacity to differentiate into striated muscle.
• The histopathologic types of RMS include embryonal, alveolar, and
pleomorphic.
• The embryonal form is the most common
• The alveolar variety is less common and carries the worst prognosis

16. Clinical Features
• Can present insidiously, clinically and radiologically mimicking other
space-occupying lesions.
• Most characteristic presentations, however, are the rapid onset and
progression of unilateral proptosis and displacement of the globe.
• Ptosis, strabismus, superonasal mass
• May also present as a palpable subconjunctival or eyelid nodule with
edema of the lids and/or chemosis
• Symptoms depend on the origin and site of the lesion.

18. Clinical Features
• Posterior tumors tend to cause edema of the optic disk, choroidal
folds, and some degree of ophthalmoplegia
• When RMS is situated in the inferior and anterior portions of the
orbit, it often causes obvious chemosis and swelling of the eyelids.
• Intracranial extension and invasion of the paranasal sinuses are
uncommon at presentation
• Changes in the adjacent bone frequently occur.

19. Investigations
• Ultrasound scan
• CT scan
• MRI
• Biopsy

20. Treatment
• Radiotherapy + Chemotherapy after histological diagnosis
• Chemotherapy - vincristine, actinomycin D, and cyclophosphamide
(VAC)
• Surgery - tumorectomy, biopsy, and needle-aspiration biopsy

21. Lacrimal Gland Tumour
• The lacrimal gland is a bilobed eccrine secretory gland, which is
situated in the superotemporal orbit.
• The 2 lobes of the lacrimal gland - the orbital lobe and the much
smaller palpebral lobe
• The lobes are separated anatomically by the lateral horn of the
levator aponeurosis.
• Mass lesions of the lacrimal gland can be classified broadly into
inflammatory and neoplastic subtypes.

23. Lacrimal Gland Tumour
• Inflammatory etiologies include dacryoadenitis, sarcoidosis, and
orbital inflammatory pseudotumor.
• Most of the neoplastic lesions in the lacrimal gland are epithelial in
origin, with approximately 50% classified as benign and 50% as
malignant.
• Benign lesions are slowly growing masses more commonly found in
adults in their forth to fifth decades of life.
• Adenoid cystic carcinoma is the most common malignant lacrimal
gland tumor, comprising 50% of malignant tumors of lacrimal gland
and 25% of all lacrimal gland tumors.

24. Neoplastic Subtypes
Benign tumours
• pleomorphic adenomas (benign mixed cell tumors)
• benign reactive lymphoid hyperplasia
• oncocytomas
• Malignant tumours
• adenoid cystic carcinoma - third decade of life, bimodal peak in
teenage years
• adenocarcinoma
• squamous cell carcinoma
• mucoepidermoid carcinoma
• malignant lymphomas

25. Clinical Presentation
• Pain – commonly seen in inflammatory lesions
• Painless inferonasal globe displacement
• Fullness of the superotemporal lid and orbit.
• Diplopia
• Diminished visual acuity
• Proptosis
• Temporal sensory loss in the distribution of the lacrimal nerve

26. Treatment
• The treatment of lacrimal gland tumors is based on the duration of
symptoms, clinical evaluation, and radiographic features of the lesion.
• Pleomorphic adenoma – extirpation (lateral orbitotomy with
intracapsular removal of all lesional tissue)
• Adenoid cystic carcinoma - radical exenteration with frontal bone
excision, maxillectomy, and temporalis fossa excision
• Radiation therapy is the mainstay of treatment for lymphoid lesions

27. Squamous cell carcinoma
• Invasive epithelial malignancy
• Less common 2nd to BCC, but more aggressive
• 5-10% of eyelid malignancies
• Metastasis through lymph, blood, or direct extension
• Potentially lethal
• Most commonly in light skinned elderly individuals
• Majority over 60 years
• Males>Females - occupational sunlight exposure

29. Risk factors
• UV irradiation
• Actinic keratosis
• Immunodeficiency
• Genetic skin disorders
• Xeroderma pigmentosa,
• Albinism
• Exposure to arsenic, hydrocarbons, radiation

30. Presentation and Treatment
• Typical presentation - Painless, elevated, nodular or plaque-like lesion
• Lower lid followed by medial canthus, upper eyelid, lateral canthus
Treatment
• Wide surgical excision
• Radiation treatment
• Chemotherapy

31. Orbital Metastasis
• 6% of orbital tumours
• In children, the primary lesions are most commonly Ewing’s sarcoma
and neuroblastoma.
• In Ewing’s sarcoma, proptosis is usually unilateral with sudden onset
& accompanying haemorrhage.
• The presentation in neuroblastoma is similar; however, it is bilateral in
50% of cases.
• Other paediatric malignancies that metastasize to the orbit are
testicular tumours and leukaemia.

32. Orbital Metastasis
• In adults, the primary tumour is usually breast or lung carcinoma.
• Tumour metastasizes more frequently to the eye than the orbit
• Orbital metastases may be the initial manifestation of the lung, GI,
thyroid or renal carcinoma.
• In adults, an infiltrative retrobulbar mass and enophthalmos is
characteristic of carcinoma of the breast

33. Orbital Metastasis
• Metastasis often are diffusely infiltrating and have distinct margins.
Less frequently, they are well circumscribed.
• On CT, these lesions are isodense or hyperdense and enhanced

34. Metastatic Orbital Tumours
• Neuroblastoma
• Ewing sarcoma
• Acute myeloid leukemia
• Breast
• Bronchus
• Prostate
• Git
• Kidney
• Invasion from adjacent structures- sinonasal tumors

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