orbital lymphoma,

1. Management of Optic Nerve
Glioma and Primary Orbital
Lymphoma
By
Dr Parneet Singh
Max Hospital,Saket

2. OPTIC NERVE TUMOURS
• Rare neoplasm involving optic nerve
• Tumor of children(<15yrs), females, NF and genetic factors
• Slow growing
• Histologically low grade gliomas- Gr I, II
• Both Pilocytic and ODG seen
• 75 % cases occur in 1st decade of life
• Can involve optic nerve, chiasma, adjacent tract

3. CLINICAL FEATURES
• Depends on the site & nature of disease process
• M/C -Ocular / orbital swelling, proptosis
• Assymetry on movement of eyeball
• Changes in vision, diplopia
• Eyelid drooping/ fullness
• Associated pain , photophobia
• Vision defects,
• Nystagmus
• Endocrine effects
• Raised ICP

4. EXAMINATION
CLINICAL EXAMINATION
• GPE: LNs, thyrotoxicosis
• External ocular examination: conjunctiva, sclera, cornea, pupils and lacrimal
system
• Eyelids: Lid lag, lid edema (thyroid ophthalmopathy)
• Proptosis measurement (>21mm or 2mm asymmetry)
• Ocular motility
• Optic nerve: Visual Acuity, perimetry
• IOP
• Slit lamp microscope,
• Opththalmoscopy

5. • Xray orbit, whole skull: Waters (occipitomental),
Caldwell (occipitofrontal)
Rhese view - optic foramen
• Bony destruction, metastatic deposit, sutural separation,
calcification
• CT/MRI can yield diagnostic accuracies 95%.
Confirmation of diagnosis
• FNAC
• Incision biopsy
• Excision biopsy

8. USG
Nature of lesion-
cystic, solid,
angiomatous,
infiltrative

9. CT Scan
• Coronal & axial – help to determine relationship of an
orbital tumour to optic nerve, extraocular ms, globe,
sinuses , brain
• Bony landmarks-Imp for surgical / RT planning

10. Optic Glioma on CT
• Fusiform thickening
• Thickening of same density as the nerve.
• Kinking of the nerve.
• Smooth edges.
• Optic canal enlarged.
• No bone erosion/hyperostosis.
• Calcifications rare.

11. Coronal noncontrast T1-weighted MRI reveals a
large intraorbital mass centered on the optic
nerve.
Enlargement, often iso to hypointense compared to the contralateral
side

12. Coronal postgadolinium T1-weighted MRI with fat
saturation reveals diffuse, intense enhancement of the
intraorbital mass
Homogenous Enhancement

13. Axial postgadolinium T1-weighted MRI with fat
saturation reveals diffuse, intense enhancement of the
intraorbital mass. The lesion is confined to the orbit.

14. Axial postgadolinium T1-weighted shows enhancement
of the intracranial optic nerve slightly expanded.

15. Axial noncontrast T1-weighted MRI reveals
bilateral, fusiform enlargement of the optic nerves
with bilateral optic nerve gliomas.

16. T2 Images-Hyperintense centrally

17. Treatment Strategies
• Surveillance
• Radiation therapy
• Chemotherapy
• Surgery
• Combination of Therapies

18. ROLE OF SURGERY
• Role Limited
• For Diagnosis: - Biopsy(not necessary)
• In cases of progressive loss of vision
• No useful vision in eye
• Debulking: Large unresectable tumor
• To relieve hydrocephalus
• Transcranial orbital resection
• Although completeness of Sx (rare) correlates with survival, leads to
significant vision loss

19. Chemotherapy
• Used to delay radiation upto 5 years of age or till progression
• To achieve delay in radiation as long term sequel is neuro-cognitive dysfunction
• Vincristine ,carboplatin
• Cisplatin or CCNU based
• French society of pediatric oncology 5 year progression free survival of 34% and 5
year radiation free survival of 61% and 5 year overall survival 89%
• Cog trial A9952 compared VCR and carbo vs procarbazine,ccnu and VCR in <10 year
children with OPG
• COG trial ,ACNS0223 is evaluating vcr,carbo and TMZ in chiuldern <10years with
optic pathway glioma.results are pending

20. ROLE OF RADIOTHERAPY
ADVANTAGES & OBJECTIVES:
1. Vision preservation
2. To cure the disease
3. Cosmesis

21. Local radiotherapy-
• In optic gliomas of post location (post optic tract)
• Intracranial chiasmatic location
• Recc after sx
• Optic nerve gliomas long term local control is 100% with RT
• Chiasmal gliomas have a poorer prognosis than optic nerve tumours
Recurrence rate – 56 % vs 22 %
• Overall survival for chiasmatic tumors in 90-100%
• For posterior gliomas progression free survival at 5 years is 20-60%
• Radiation therapy beneficial to chiasmal gliomas – improving vision
&decreasing recurrence rates by 41 %

22. Conventional Radiation portals
• Ant + lat 45 wedges-2 field technique
• 3 field in case of intracranial location -1vertex + 2 lateral
• B/l lateral fields
• Superior and inferior oblique
• Newer Techniques-Conformal Radiation, IMRT and FSRT and
Proton therapy

23. Ant & Lateral Wedge pair technique
• Anterior field:-
• Superior : supraorbital ridge
• Inferior: infraorbital ridge
• Medial: C/L medial canthus
• Lateral: Lateral canthus
• Lateral field:-
• Anterior: lateral canthus
• Posterior: front of tragus
• Superior & inferior same

25. Superior and inferior oblique fields
• More role in anteriorly lying tumors
• Proptosis
Advantages:
• Avoids C/L orbit with uniform irradiation of the entire orbital
contents.

26. Lateral fields
• Parallel opposed with anterior half beam block
• B/l eye disease
• ON glioma involving optic chiasma
RESULTS
Long term survival 80-100%, Relapse free survival(6yrs) 75-
80%.

27. 3 Field Technique
• Three field technique
– 2 lateral opposed and 1 vertex field -
– 15-30 °wedge
– Typically 5x5 cm field used,
• centered on sella
• Position - supine
• Head and neck flexed
• Head typically held at 45°
– Tilting –head base plate immobilization system

28. • All patients were treated in a
supine position with flexion of the
head so that base of the skull was
in right angle to the couch and
parallel to the central plane
• Dose: Adults: 50 Gy@ 2Gy/#
Children: 45Gy@ 1.6-1.8Gy/#

30. Orbital lymphoma
• Lymphoid tumors -common despite the orbit not containing lymph nodes
or a well defined lymphatic vasculature.
• Incidence 13% of all orbital tumors.
• Primary or associated with systemic disease.
• Represents 8 % of all extranodal NHL & 1 % of all NHL
• Approx 35% of patients with lymphoid tumors of orbital tumors will
eventually develop systemic lymphoma
• Presenting age- 15- 70 yrs
• Majority are low grade- 84 %

31. • Most lesions are in the superior orbit.
• 20 % bilateral
• Majority of primary ocular lymphomas are large cell type and a CNS
LYMPHOMA should be excluded
• 85-90% diffuse low grade, B cell
• 15% follicular or nodular characterstics
• Progressive painless proptosis over several wks to months.
• The classic lesion is a smooth, pink-orange mass ("salmon patch")
under an intact conjunctiva.

32. • Biopsy
• Worked up for systemic disease
• Including a complete physical exam, a complete blood count,
bone marrow biopsy and CT scans of the thorax, abdomen and
pelvis.
• CSF examination
• CT scan -homogeneous mass with well defined borders that
does not destroy surrounding structures or bone.
• MRI – to assess extent of disease
• PET CT

33. Staging

35. T1 axial
Hypointense on T1

36. T1 Coronal Fat Sat

37. T1 Axial

38. T1 c
Homogenous Enhancement

39. T2 Coronal
Hyperintense on T2

40. Radiotherapy
• Stage I, II- RT main t/t
• - Whole orbit is to be
treated
• Energy- 1.25 Mv / 6 Mv
• Supine
• Immobilise
• Ant & lat field using 45
wedges
• Looks into beam,
• dose- 25- 30 Gy@ 1.8- 2
Gy/#
• Bilateral disease- parallel
opposed fields

41. • If there is a forward displacement of eveball
• Involvement of post or anteromedial part of orbit
• Sup & inf oblique fields

42. • Stage III. IV- Combined CCT(3-4 Cycles) + RT
 In ocular lymphomas with involvement of brain & CSF
systemic chemotherapy with CHOP regime
 Intrathecal methotrexate biweekly
 Combined with WBRT and ORT

43. Results: All patients had a complete response to RT. Intraorbital recurrence
developed in previously uninvolved areas not included in the initial target
volume in 4 patients (33%) treated with partial orbit RT. All were salvaged by
repeat RT or surgery. No patient treated with whole orbit RT developed
intraorbital recurrence. The acute and long-term toxicity was similar in both
groups. All but 1 patient retained good vision.
Conclusion: Patients with orbital lymphoma should be treated to the entire
orbit.

44. Complication Post Radiotherapy
 Radiation induced Second malignant neoplasm within RT
• Osteosarcoma / fibrosarcoma / other spindle cell sarcoma / malignant
melanoma/ thyroid carcinoma
 Facial / orbital deformities
 Short strature
 Endocrinological disorders
 Moyamoya syndrome-veno-occlusion in circle of willis
 Opthalmic :
• Dry eye
• Corneal damage
• Cataract
• Retinal damage

45. MANAGING RADIATION REACTIONS
• Dryness of eye: Artificial tear drops
Antibiotic eye drops
Steroid eye drops (increased cataract)
• Epiphora: Rectify any ill fitting eye shield
• Lid deformities: managed surgically
• Cataract: IOL implantation

46. PRECAUTIONS AND
PREREQUISITES OF
RADIATION THERAPY

47. RADIATION TOLERANCE OF NORMAL
TISSUES
LENS
• TD5/5=10Gy, TD50/5= 18Gy
• Most radiosensitive structure in orbit.
• RT causes cell death in the germinative zone, which is the ring of mitotically
active cells at the periphery of the lens.
• Degenerated fibers and debris migrate to post pole of lens, resulting in
opacities (latency 2-3yrs).
Merrium and Focht observed lens opacities after as low as 3.6Gy and an
invariable cataract after 10.5Gy.
HOWEVER, the cataract if developed can be treated by IOL implantation.

48. CORNEA
• TD 5/5= 50Gy, TD 50/5= 60Gy
• Second most radiovunerable structure in the eye.
• Doses >30Gy @ 2Gy/# leads to acute keratitis
>50Gy leads to scarring, perforation
Initially pin head erosions
Punctate erosions leading to irritation and increased lacrimation
Edema and keratitis with decreased corneal sensation
ultimatly scarring and phthisis bulbi.
• Corneal blindness is an unacceptable side effect of RT .

49. RETINA
• TD 5/5=45Gy, TD 50/5=65Gy
• Injury mechanism: endothelial cell death in small vessels
leading to ischemic changes.
• Ophthalmoscopic findings: microaneurysms, new vessel
formation, hemorrhages, cotton wool exudates.
• Latent period 18-30 months for symptoms.
OPTIC NERVE
• TD 5/5=50Gy, TD50/5= 55Gy
• RT tolerance similar to brainstem, very low risk <50Gy
• Optic neuropathy can lead to blindness.

50. LACRIMAL GLANDS
• Dose <40Gy unlikely to cause severe dry eye syndrome.
• Dose >40Gy, leads to tear film # causing corneal damage.
• Shielding in the lateral aspect of superior orbit reduces
occurrence of dry eye syndrome.
BONY ANOMALIES
• RT usually avoided in children <3yr
• Mid facial anomalies: Hypotelorism, Enophthalmos, Depressed
temporal bone, Depressed nasion.

51. Conclusions
• Orbital tumours though rare very imp in field of oncology
• Neoplasms can arise from any structure
• Important prerequisite is not only diagnosis but also histopathological type
& extent of lesion
• Radiation is indicated in almost 70 % cases
• A major principle in management– to deliver therapeutic dose without
causing damage to critical neighboring structure
• Cure is the aim but vision preservation and cosmesis are important factors
to be considered while selecting treatment modality.
• Prevention of long term Radiation toxicity and management of acute
reactions are important issues