Intraocular tumors can cause secondary glaucoma through several mechanisms. The most common intraocular tumors that cause glaucoma are iris and ciliary body melanomas. These tumors can lead to glaucoma by directly invading the anterior chamber angle, releasing pigment that obstructs the angle, or causing inflammation and neovascularization of the angle. Diagnosis involves a thorough exam including gonioscopy and imaging like ultrasound to identify the location and characteristics of the tumor. The document focuses on the various pathophysiological mechanisms by which different types of intraocular tumors can cause a rise in intraocular pressure and secondary glaucoma.

1. GLAUCOMA IN INTRAOCULAR
TUMOUR
By- Shweta .S. Maurya
3nd year B. Optometry
Institute For Technology And Management

2. GLAUCOMA
 Glaucoma is the
second leading cause
of blindness.
 Glaucoma is actually
a group of diseases.
The most common
type is hereditary.

3. INTRAOCULAR TUMORS
 Intraocular tumors are rare causes of glaucoma.
 The epidemiology, prognosis, and mechanism of
action depend on the specific tumor type.
 The tumor may not be directly visible in some
instances, and different methods of indirect
visualization may need to be used.

4. PATHOPHYSIOLOGY
 There are a number of different mechanisms by
which ocular tumors can cause glaucoma:
(1) Pigment dispersion,
(2) Secondary angle closure
(3) Uveitis,
(4) Iris neovascularization,

5. (5) Choroidal detachment,
(6) Suprachoroidal hemorrhage, and
(7) Anterior displacement of lens-iris diaphragm.

6. THE MOST COMMON INTRAOCULAR TUMOR
CAUSING SECONDARY GLAUCOMA
 Melanoma arising within the iris.
 Melanoma arising within the
ciliary body.
 Direct growth of the tumor into
the angle.
 Tumor seeding into the angle.

7.  Neovascularization of the angle,
 Hemorrhage with volume effect
or angle closure,
 Pigment release into the aqueous, the surface of
the iris, and the chamber angle.

8. PATHOPHYSIOLOGY

9. I. OPEN ANGLE GLAUCOMA
1. Direct Invasion of Anterior Chamber Angle.
mechanically
Invasion of the tumor cells in AC angle
Blocks aqueous humor from leaving the eye
Rise in IOP

10. 2. Secondary Pigmentary Glaucoma.
 Massive dispersion of pigment displaced from tumor
cells (i.e.- melanoma and melanocytoma) can lead to
obstruction of the AC angle.
 In ciliary body melanomas this is the most common
cause of secondary glaucoma.

11. 3. Melanomalytic Glaucoma.
 Melanomalytic glaucoma is secondary to the
release of pigment from tumor cells which
subsequently get taken up by macrophages.
 Based on studies , these pigment-laden
macrophages cause blockage of the trabecular
meshwork which leads to increased IOP

12.  Trabecular meshwork seeding (melanomalytic). Slit-
lamp photography shows collections of episcleral
perilimbal pigment deposition (arrows).
 NEXT
 Episcleral venous hypertension due to traumatic
carotid-cavernous fistula ”

13. 4. Increased Episcleral Venous Pressure.
 Aqueous humor outflow is dependent on the pressure
gradient between IOP and episcleral venous pressure.
 Orbital tumors or extraocular extension of an intraocular
tumor can cause an increase in episcleral venous pressure
through direct compression.
 Due to this pressure gradient
reduction from the elevated
episcleral pressure, there is a
rise in IOP often causing a
secondary glaucoma.

14. II. CLOSED ANGLE GLAUCOMA
1. Uveitic.
 Inflammation secondary to intraocular
tumors can cause peripheral anterior synechiae, which
can lead to closure of the angle and elevation in IOP.
 In patients with uveal melanomas and uveitis, secondary
angle closure is common from peripheral anterior
synechiae.
 Metastatic tumors also have been shown to have
significant inflammation leading to peripheral anterior
synechiae.

15. 2. Secondary Neovascularization.
 Patients with intraocular tumors can often develop
neovascularization of the iris and angle, which can lead to
angle closure glaucoma.
 This is commonly true in tumors which cause a chronic
RD ,leading to ischemia of the tissue and resultant
secondary neovascularization.
 Metastatic cutaneous melanomas
also are highly associated with
neovascularization.
 Neovascularization can also
present as a complication of
tumor treatment.

16. 3. Anterior Displacement of Lens-Iris Diaphragm.
 The most common mechanism of angle closure in
patients with intraocular tumor is forward displacement
of the lens-iris diaphragm.
 The displacement is usually from a large mass in the
posterior segment of the eye causing forward pressure of
iris and lens structures leading to pupillary block, C iris
bombé and finally peripheral anterior synechiae closing
the AC angle.

17. DIAGNOSIS

18. HISTORY
 The workup begins with a thorough history, which should
be performed in all patients with elevated IOP.
A thorough history includes all of the following:
• Complete history of present illness (including current and
past symptoms)
• Past medical and ocular histories
• Past surgical history (including radiation treatments)
• Medication list (including eye drops)
• Family history (especially cancer history)
• Complete review of systems
• Social history (including drugs, tobacco use, alcohol and
occupation)

19. SYMPTOMS
 The symptoms that the patient experience depend greatly
on the type and location of the malignancy as well as the
amount of ocular involvement.
 The most frequently recorded symptoms are blurred
vision, pain, redness and floaters.
 Some patients, however, will not complain of any
symptoms.
 Glaucoma symptoms are dependent on the speed at which
the pressure rises.

20.  With acute glaucoma from angle closure, decreased
vision, halos around lights, ocular pain, and nausea may
be present.
 With chronic glaucoma with progressive angle closure
or open angles, no ocular symptoms related to the
elevation of IOP may be present.
 With iris melanoma, a hyperchromic heterochromia
may be present.
 With a ciliary body melanoma, a distorted pupil or
vision change from lenticular astigmatism may occur.
 With choroid melanoma, the visual symptoms may be
decreased vision or a change in the peripheral vision,
depending on the location of the tumor.

21. PHYSICAL EXAMINATION
 Slit lamp biomicroscopy
 Gonioscopy of the angle.
 A complete dilated exam, as long as the AC angle is
not too narrow.
 The following findings are essential to note for any
patient suspected of having tumor induced secondary
glaucoma.

22. ANTERIOR SEGMENT
EXAM FINDINGS:
 Pigment dispersion in AC or angle,
 Dilated episcleral vessels,
 Hyphema,
 Neovascularization of Iris or Angle,

23. o Intraocular inflammation noting
cell/flare, hypopyon,
o Mass lesions of the iris/ciliary
body/angle,
o Transillumination of globe
(tumors create shadows)
o Open versus closed angle

24. POSTERIOR SEGMENT FINDINGS:
 Retinal detachments,
 Posterior segment masses,
 Vitritis,
 Vitreous hemorrhage.

25. PHYSICAL (DIAGNOSIS)
 Diagnosis is assisted by Gonioscopy, indirect
ophthalmoscopy, and ultrasonography. Clinical scenarios
depend on tumor location.
 Iris melanoma can occur as either diffuse or well-
circumscribed forms, with the former being less common
and more malignant.

26.  Ciliary body melanomas tend to be larger at presentation
due to their obscure location and usual lack of visual
symptoms.
 They may present as a ring melanoma, which completely
encircles the ciliary body. Proportion of epithelioid cells
is higher in ciliary body melanoma.
 Choroidal tumors associated with glaucoma typically
have broken through the Bruch membrane and are
mushroom shaped.

27.  Diagnosis of melanocytoma is through direct
visualization using slit lamp, Gonioscopy, or indirect
ophthalmoscopy.
 Melanocytomas can occur anywhere along the uveal
tract, although the optic nerve is the most common site.
 These appear as darkly
pigmented, isolated
lesions, which have a
strong tendency to
undergo necrosis and
fragmentation, leading to
pigment release.

28. • Consequently, trabecular meshwork becomes
obstructed, leading to IOP elevation.
•Treatment of melanocytoma consists of observation
with photographic documentation in smaller lesions.

29.  Benign iris melanocytic lesions (Iris nevi) are difficult
to grossly distinguish from malignant lesions of the iris.
 These occur as small, discrete, flat, or slightly raised
nodules with variable pigmentation.
 Another study identified 5 factors associated with
higher risk of malignancy, as follows:
 (1) Diameter greater than 3 mm,
 (2)Pigment dispersion,
 (3) Prominent tumor vascularity,
 (4) Increased intraocular pressure, and
 (5) Tumor-related ocular symptoms.

30.  Although iris nevi rarely cause a pathologic process,
diffuse growth can cause elevated IOP by direct extension
into the trabecular meshwork.
 The treatment of an iris nevus consists of observation with
photographic documentation in smaller lesions.

31.  Tumors of the retina (retinoblastoma)
 The tumor may assume an endophytic or exophytic
configuration. In the former configuration, tumor cells
may invade into the vitreous and anterior chambers.
 Neovascularization is associated with angiogenic factor
production from the tumor or ischemia due to large
retinal vasculature involvement.
 Subsequent neovascularization of the iris with angle
closure accounts for 73% of glaucoma.
 Pupillary block leading to angle closure from large
tumors and infiltration of the trabecular meshwork
account for the remaining cases.

32. RETINOBLASTOMA

33. OPHTHALMIC IMAGING

34.  1. B-Scan Ultrasonography (B-Scan)
 If there is opacification of the media obstructing the
examiner’s view.
 It is helpful in taking measurements of the posterior
segment tumors like uveal melanomas and looking for
concurrent RD , vitreous hemorrhage or vitritis.

35.  2. Ultrasound Biomicroscopy (UBM)
 To further characterize tumors in the anterior segment,
ultrasound biomicroscopy can be performed.
 UBM uses a high frequency wavelength which allows for
accurate measurements of anterior lesions which is
helpful for diagnosis (solid versus cystic masses).

36. CAUSES
Intraocular tumors that may cause elevated
IOP may be of 3 types
1. Melanocytoma(benign)
2. Malignant melanoma
3. Metastatic (spreading )

37. IT CAN ARISE FROM
IRIS

38. CILIARY BODY

39. CHOROID

40. OPTIC NERVE

41. RETINA
 Carcinoma
 Cutaneous melanoma

42. METASTATIC TUMORS
 The 2 most common sites of primary tumor metastatic
to the eye are breast and lung cancers
 The posterior uvea is the most common site of
metastases, but glaucoma more often is associated with
anterior metastases.
 Mechanism of glaucoma includes direct invasion of the
trabecular meshwork by tumor cells, secondary angle
closure by anterior displacement of the lens-iris
diaphragm, and neovascularization of AC angle.
 Squamous cell carcinoma may produce a sheet of tumor
cells.

43.  Glaucoma may be the initial presentation in some
patients.
 Medical management of IOP coupled with radiation
or chemotherapy may be useful.
 Enucleation is reserved for blind, painful eyes.

44. PHAKOMATOSES
 Phakomatoses are a group of hamartomatous disorders
in which abnormal proliferation of tissues occurs in their
normal locations.
 These tumors primarily affect the eye, skin, and nervous
system, although other systems may be affected to a
lesser degree.

45.  Neurofibromatosis is a common autosomal dominant
systemic disorder divided into types 1 and 2
 Glaucoma usually is unilateral and associated with
eyelid thickening and contour abnormalities.
 Different mechanisms for elevated IOP have been
proposed, as follows:
(1) Direct infiltration of the angle tissue,
(2)Secondary angle closure due to ciliary body and
choroid thickening,
(3)Fibrovascular membrane
formation over the angle.

46. MISCELLANEOUS TUMORS

47. MISCELLANEOUS TUMORS
 The eye may become involved in acute and chronic
lymphocytic leukemia and affect the anterior and posterior
segment.
 Elevated IOP results from outflow obstruction secondary to
iritis, hyphema, hypopyon, or leukemic infiltration of the
trabecular meshwork and Schlemm canal, as well as episcleral
tissue, which may involve aqueous veins.
 Management is
primarily through
medical means to
control IOP and to
address the underlying
leukemia through
appropriate means.

48.  Multiple myeloma may cause ciliary body cysts,
which may lead to secondary glaucoma arising from
lens dislocation or anterior displacement of the iris
root.
 Large cell lymphoma may mimic uveitis and may
infiltrate the uveal tract, leading to secondary angle
closure.
 Juvenile xanthogranuloma is a benign histiocytic
tumor affecting the skin and eye in young children.
• A salmon colored iris tumor is associated with
spontaneous hyphema in children .
• Radiation therapy to the iris tissue has helped to
resolve the iris tumor and glaucoma.

49. DIFFERENTIAL DIAGNOSES
1. Glaucoma, Angle Closure, Acute
2. Primary Angle-Closure Glaucoma
3. Hyphema Glaucoma
4. Lens-Particle Glaucoma
5. Neovascular Glaucoma
6. Pigmentary Glaucoma
7. Primary Open-Angle Glaucoma
8. Pseudo exfoliation Glaucoma
9. Unilateral Glaucoma
10. Uveitic Glaucoma

50. Leukemia
 Involvement of the eye can occur in just under one-
third of cases of systemic leukemia. Secondary
glaucoma is generally caused by AC angle obstruction
from leukemic cells
Retinoblastoma
 Retinoblastoma is the most common primary
intraocular tumor in children and most commonly
presents with strabismus and leukocoria. Patients also
rarely can have a red, painful eye either from
inflammation or elevated IOP.

51. DIAGNOSTIC PROCEDURES
 If there is still uncertainty with the diagnosis after a full
history, physical exam and imaging then diagnostic
sampling of the lesion can be performed for most tumors.
 The use of fine needle aspiration biopsy with either a
25-guage needle or vitrector can be performed .

52. PROCEDURES
 For tissue diagnosis, fine-needle biopsy, aqueous
aspiration, or excisional biopsy is needed.
 Diagnosis of iris nevus is aided by fluorescein
angiography of iris, aqueous aspiration, or biopsy.

53. MANAGEMENT

54. MANAGEMENT
 For intraocular tumors with secondary glaucoma
elimination of the viable tumor cells is of primary
importance.
 Control of IOP control is secondary and can be
done either medically or surgically.

55. MEDICAL THERAPY
 Treatment of the secondary glaucoma should begin with
topical eye drops (alpha-agonists, beta-blockers and
carbonic anhydrase inhibitors) which decrease aqueous
humor production.
 If the pressure remains uncontrolled on topical medication
then oral hypotensive agents like acetazolamide or
methazolamide should be tried.
 For metastases with multiple lesions in the eye,
systemically chemotherapy can play a large role. In eyes
that have poor visual potential and elevated pressure,
treatment for the secondary glaucoma should only be given
if pain is present.

56. MEDICAL CARE
 Glaucoma management itself begins with medical therapy,
concurrent with treatment of the intraocular tumor through
surgery, radiation, chemotherapy, or a combination of these
treatments.
1. Standard methods of IOP control should be used, although
success rates with these topical medications will necessarily
be low.
2. Anti-vascular endothelial growth factor (anti-VEGF) therapy
may be helpful in those cases where neovascularization plays
a role in the pathogenesis of the glaucoma.
3. In systemic lymphoma, leukemia, and metastatic
malignancies, treatment will often include systemic
chemotherapy and radiation, with current regimens favoring
the use of chemotherapy first.

57. SURGERY
 Sometimes treatment of the underlying tumors with
plaque brachytherapy, external beam radiation,
chemotherapy or radiation can improve the IOP.
 For certain tumors like large uveal melanomas,
retinoblastoma or if the eye is painful with no visual
potential then primary enucleation may be the
appropriate option.

58. SURGICAL CARE
 Surgical options to control intraocular pressure must be
tempered by the need for preventing extraocular tumor
spread.
 For smaller tumors, observation is warranted until
growth is documented.
 Iridectomy are options for removing smaller tumors.
 For anterior tumors, argon laser to tumor-free areas is
an option.
 More posterior tumors may require local resection,
photocoagulation, or episcleral radiopaque therapy.
 Enucleation is also an option.

59.  For melanocytoma, laser or surgical excision of the
lesion may decrease the pigment load and decrease the
IOP.
 Glaucoma usually is associated with advanced stages of
Retinoblastoma, and enucleation may need to be
discussed.
 Any surgical intervention must be tempered by the risk
of extra ocular spread.
 Blind eyes should not undergo incisional glaucoma
surgery because of the risk of spreading the tumor to
outside the globe.

60. PROGNOSIS
 With early detection, the outlook is improved.
 The prognosis for the patient depends upon the type
of tumor, the amount of involvement within the eye,
as well as the treatment received.
 The primary goal for any treatment is to control the
tumor first and then control the IOP.
 Management of any intraocular tumor must be done
concurrently with an oncologist to account for
systemic disease.

61.  Complications
Even with early detection and aggressive therapy for
intraocular tumors, loss of the eye or death can occur.
A significant number of patients will develop side effects
from treatment of the original tumor with brachytherapy
which can lead to neovascularization and secondary angle-
closure glaucoma.

62.  Consultations
Glaucoma specialist or oncologist
 Patient Education
Follow-up care is important.

63. THANK YOU FOR LISTENING,
HOPE YOU LEARNED
SOMETHING NEW.