ORBITAL TUMOR AND IT'S MANAGEMENT

1. ORBITAL TUMORS
DR. HARSH GOYAL
PG JR- 3
SAIMS ,INDORE
DEPT OF RADIOTHERAPY

2. INCIDENCE & EPIDEMOLOGY
 Tumors of the eye and orbit are rare.
 Male to female incidence is similar.
 In adults, Melanoma is the most common
primary intraocular cancer, followed by
Lymphoma
 In children Retinoblastoma is the most common
tumor, followed by Medulloepithelioma
 Metastases, or secondary intraocular tumors, are
more common than primary tumors and typically
come from breast or lung cancers.

3. ORBIT
 The orbit is the cavity or socket of the skull in which
the eye and its appendages are situated.
 In the adult human, the volume of the orbit is 30 mL,
of which the eye occupies 6.5 ml.
 The orbits are conical or four-sided pyramidal cavities,
which open into the midline of the face and point back
into the head.

5. CLASSIFICATION
BY ORIGIN
 Primary - lesions originating from the orbital
tissues
 Secondary - lesions originating from the neighboring
cavities and tissues
 Metastatic - lesions reach the orbit via
hematogenous or lymphatic spread

6. BENIGN ORBITAL TUMORS
a. Pterygium
b. Chorodial Hemangiomas
c. Orbital Pseudotumors
d. Thyroid associated orbitopathy

7. MALIGNANT ORBITAL TUMORS
 Metastatic Carcinoma to the uvea
 Malignant Melenoma of Uvea
 Retinoblastoma

8. MALIGNANT ORBITAL TUMORS
The most common malignant orbital tumors in adults
include:
LYMPHOMA - It is the most common type of malignant
orbital tumor in the adults.
 Occurs mainly in the lacrimal gland, but can occur in any
other orbital structure.
OPTIC NERVE GLIOMA - It is extremely rare tumor that
begins in the optic nerve and spreads to the orbit

9.  SARCOMA –
almost any type of sarcoma can involve
the orbit
 Angiosarcoma
 Fibrosarcoma
 Osteosarcoma
 Chondrosarcoma
 Liposarcoma
 Malignant fibrous histiocytoma

10. • Cancers from other parts of the body, such as the
breast, lung, prostate, brain and kidney, can also
spread (metastasize) to the orbit.
• Metastasis from melanoma can also occur but it is
uncommon.
• SECONDARY CARCINOMAS - they can also be
secondary cancers that have spread to the orbit from
nearby structures, such as the eyeball (intraocular
tumors), eyelid, conjunctiva, sinuses or nasal cavity.

11. TNM STAGING
 TX - Primary tumor cannot be assessed
 T0 -No evidence of primary tumor
 T1 - Tumor is 15 mm (0.6 inch) or less in size.
 T2 - Tumor is more than 15 mm in size. It has not spread into
the globe of the eye or the bony wall of the orbit.
 T3 -Tumor is any size and has spread into the orbital tissues or
bony walls of the orbit.

12. T4 - Tumour has spread into one or more of the following:
 the globe
 periorbital structures, such as the eyelids or temporal fossa
 nasal cavity and paranasal sinuses
 central nervous system (the brain and spinal cord)

13.  NX - Regional lymph nodes cannot be assessed
 N0 -No regional lymph node metastasis
 N1 - Regional lymph node metastasis
 M0:No distant metastasis
 M1 :Distant metastasis

14. SIGNS & SYMPTOMS
 Proptosis – the most important sign
 Blurred vision,
 Diplopia,
 Strabismus ,
 Whitish or yellowish glow through the pupil,
 Decreasing/loss of vision,
 Red and painful Eye
 Pain

15. MANAGEMENT
 Systemic examination ,
 Opthalmic examination,
 Vascular study-orbital venography,
carotid angiography, MR angiography,
 Routine blood investigation
 Imaging of bony structure-Digital X ray
 Ocular ultrasonography
 Ct scan & MRI.
 Biopsy.

16. TREATMENT
Orbital tumor may be treated by:
 Surgery
 Chemotherapy
 External beam radiotherapy
 Plaque brachytherapy

17. LACRIMAL GLAND TUMORS
 Lacrimal gland tumors are seen more frequently in the third
decade of life, and the second bimodal peak is in the teenage
years.
 The lacrimal gland is a bilobed eccrine secretory gland, which
is situated in the Superotemporal orbit.
 The 2 lobes of the lacrimal gland
- Orbital lobe
- Palpebral lobe,

18. LACRIMAL GLAND APPARATUS

19.  Lacrimal gland swelling can be classified broadly into
- Inflammatory
- Neoplastic subtypes.
 Inflammatory etiologies, include
- Dacryoadenitis,
- Sarcoidosis .
 Most of the neoplastic lesions in the lacrimal gland are
epithelial in origin,
 50% classified as
- BENIGN and 50% as MALIGNANT PSEUDO TUMOR.

20.  BENIGN LESIONS -
- Pleomorphic adenomas ,
- Reactive lymphoid hyperplasia,
- Oncocytomas.
These lesions are slowly growing masses more
commonly found in adults in their forth to fifth decades of
life.

21.  MALIGNANT LESIONS :
- Adenoid cystic carcinoma- comprising 50% of
malignant tumors of lacrimal gland and 25% of all
lacrimal gland tumors
- Adenocarcinoma,
- Squamous cell carcinoma,
- Mucoepidermoid carcinoma,
- Lymphomas

22. TNM STAGING
 TX: The primary tumor cannot be assesed.
 T0 : no evidence of primary tumor.
 T1: The tumor is 2 centimeters (cm) or smaller and may or may
not extend outside of the lacrimal gland to the orbital soft tissue.
 T2: The tumor is between 2 cm and 4 cm and likely extends to
the orbital soft tissue.
 T3: The tumor is greater than 4 cm and likely extends to the
orbital soft tissue.
 T4: The tumor has invaded the periosteum (the membrane of
connective tissue that covers the bone) or the orbital bone.
 T4a: The tumor has invaded the periosteum.
 T4b: The tumor has invaded the orbital bone.
 T4c: The tumor has extended beyond the orbit to adjacent
structures, including the brain and sinuses.

23. TNM STAGING
 NX: The regional lymph nodes cannot be evaluated.
 N0 : There is no regional lymph node metastasis.
 N1: There is regional lymph node metastasis.
 MX: Distant metastasis cannot be evaluated.
 M0 : There is no distant metastasis.
 M1: There is metastasis to other parts of the bod

24. SIGNS AND SYMPTOMS
 Malignant lesions characteristically present with a
subacute course of proptosis and temporal sensory
loss in the distribution of the lacrimal nerve in one
third of patients.
 Diplopia and diminished visual acuity can be seen in
rapid progressive lesions.
 Benign lesions commonly present with painless
inferonasal globe displacement and fullness of the
superotemporal lid and orbit.

25. DIAGNOSIS
 CT scan of BENIGN epithelial lesions, such as
pleomorphic adenomas, reveals a well-circumscribed,
pseudoencapsulated lesion in the superotemporal
fossa.
 In contrast, MALIGNANT epithelial lesions, such as
adenoid cystic carcinoma, usually present as an
irregular mass, producing bony erosion (70%) and
occasional calcification (20%).

26. TREATMENT
 SURGERY : Surgery is the mainstay treatment of lacrimal
gland tumors.
 RADIOTHERAPY:
 It is most often used for lacrimal gland lymphoma.
 The dosage of radiation used and the site and type of the
tumor significantly affect the risks of side effects.

27.  Cataracts are a very common side effect of radiation
therapy to the eye area.’
 Also, loss of eyelashes and/or a dry eye can occur with
external-beam radiation therapy.
 Other side effects include radiation retinopathy.
 Radiation optic neuropathy involves nerve damage in the
eye.
SIDE EFFECTS

28.  CHEMOTHERAPY -
 IMMUNOTHERAPY - Rituximab (Rituxan) is the
most common targeted therapy used in the treatment
of a lacrimal gland tumor

29. MALIGNANT MELANOMA OF THE UVEA
UVEA - It is the primary matrix of eye. It is continuous with
in the iris, ciliarybody, and choroid .
UVEAL MELANOMA are the most common primary
intraocular malignancy in adults and account for 5% of all
melanomas.
ETIOLOGY- 1) lower socio economic status,
2) UV light exposure,

30.  CHOROIDAL MELANOMAS are located posterior to
the ciliary body ,
 IRIS MELANOMA are the most easily visible,They
tend to be smallest at diagnosis,and lest likely to
metastasis.
 CILIARYBODY MELANOMA are least visible as they
are hidden behind iris ,and more likely to
metastasized.

31. HISTOLOGY
 CALLENDAR classified UVEAL melanoma into
3 categories:
1) SPINDLECELL MELANOMAS
2)EPITHELOID CELL MELANOMAS
3)MIXED CELL MELANOMAS

32. SYMPTOMS
 Decreased or blurry vision,
 Flashes of light,
 Distortion or loss of vision.
 Dark spot on the colored part of the eye or a distorted
pupil.

33. DIAGNOSIS
 Ocular ultrasonography
 Fine needle aspiration biopsy,
 Fluorescein angiography and photography(by slit lamp,
gonioscopy for iris and ciliary body tumors)

34. MANAGEMENT
 ENUCLEATION -
Enucleation is required in a subset of patients, because
of complications of conservative therapy.
 ENDORESECTION -
Transretinal endoresection is controversial, mainly
because of fear of seeding of tumor cells.

35.  TRANSSCLERAL RESECTION -
Transscleral local resection has been promoted for
tumors >6-mm thick in patients highly motivated to
retain vision.
 TRANSPUPILLARY THERMOTHERAPY -
In this technique 1-minute applications of 3-mm
spots of low-energy diode laser are administered to the
tumor and the surrounding choroid.

36. PLAQUE BRACHYTHERAPY
 Plaque brachytherapy is the mainstay of treatment in
many centers, with iodine-125 and ruthenium-106
being the most common isotopes, although
palladium-103 has also been used effectively.
 91 Iodine emits γ-rays, which have a range sufficient for
tumors up to 8- to 10-mm thick, while ruthenium
delivers beta particles that have a more limited range,
which is suitable for tumors upto 5 mm.

37.  The general objective with all plaques is to deliver
approximately 80 Gy to the tumor apex by fixing the
plaque in the exact location of the tumor.
 Best results were obtained in eyes with small tumors
outside a radius of 5 mm from the optic disc and foveola.

38. PLAQUE BRACHYTHERAPY

39. PROTON BEAM THERAPY
 Proton beam therapy may be used to treat uveal
melanoma and is usually indicated for tumors that
extend close to the optic disc,
 ADVANTAGE - Proton beam delivers a homogeneous
dose to tumor and has a sharp edge, a high tumor dose
can be delivered with relative sparing of the optic
nerve.

40. STEREOTACTIC RADIOTHERAPY
 Stereotactic radiation can be delivered by
LINEAR ACCELERATOR (LINAC)
 By specialized devices -
GAMMA KNIFE which provides focused radiation with a
multitude of sources.

41. COMPLICATIONS
The most frequently encountered complications are
 Exudative retinopathy
 Neovascular glaucoma
 Vitreous hemorrhage
 Radiation retinopathy
 Cataract