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MEDICAL SURGICAL NURSING- II
UNIT – II
NURSING MANAGEMENT OF PATIENTS WITH
DISORDERS OF EYE
TOPIC : TUMORS OF THE EYE
PRESENTED BY
Mrs. SOUMYA SUBRAMANI, M.Sc.(N)
LECTURER, MSN DEPARTMENT
CON- SRIPMS, COIMBATORE.
Tumors of the Eye
What are ocular tumors ?
Ocular tumors can appear on the eyelids, in the eye
(conjunctiva, choroid or retina) and in the orbit (the cavity
that houses the eyeball).
• Eye neoplasms can affect all parts of the eye, and can be a benign
tumor or a malignant tumor (cancer).
• Eye cancers can be primary (starts within the eye) or metastatic
cancer (spread to the eye from another organ).
• The two most common cancers that spread to the eye from
another organ are breast cancer and lung cancer.
• Other less common sites of origin include the prostate, kidney,
thyroid, skin, colon and blood or bone marrow.
Early diagnosis and treatment is necessary. Time is
of the essence to save vision, the eye and even the
life of the patient in the most serious cases.
There are several types of benign and malignant tumors
that affect the eye and its different structures
Include :
1Tumors of the Orbit
2Tumors of the Lacrimal Gland
3Tumors of the Eyelid
4Tumors of the Conjunctiva
5 Tumors of the Uveal tract
6 Tumors of the Retina
Orbital Tumors
A lesion in the orbit?
Decide whether it is an ocular lesion OR
a non-ocular lesion, i.e. is it involving the globe or
involving the structures outside the globe.
If it is a non-ocular lesion, see the lesion is involving
which space.
Intraconal space tumors:
 Benign
 Dermoid cyst
 Capillary and cavernous hemangioma
 Optic nerve lesions
◦ Optic neuritis
◦ Optic nerve glioma
◦ Optic nerve meningioma
 Schwannoma of 3rd and 6th cranial nerve
 malignant
 Lymphoma- more prevalent neoplasm in adults
 Rhabdomyosarcoma- children
Extraconal space tumors : Etiology
 Benign
 Dermoid cyst
 Schwannoma of the trochlear nerve
 Metastatic
 Lacrimal gland tumors
DERMOID CYSTS
usually occur in children and make up 4% to 6%
of orbital tumors.
Painless mass, free from the skin, with variable
ocular displacement.
Mostly located near the lacrimal fossa or nasal bone.
Grow slowly, remodeling adjacent bones or stuctures.
RHABDOMYOSARCOMA
Most common primary malignant tumor of the
orbit in children.
A highly malignant tumor.
Av age of presentation:7 yrs.
M>F
presents with rapidly progressive exophthalmos. •
Originates from extra-ocular muscles, •
nasopharynx, or paranasal sinuses.
Usually present in the superomedial orbit and •
may produce bone destruction.
Lacrimal Gland Tumors
Benign
‐ Pleomorphic adenoma
‐ Schwannoma
‐ Dermoid
‐Neurofibroma
Malignant
‐ Adenoid Cystic Carcinoma
‐ Malignant Mixed Tumour of the Lacrimal Gland
‐Adenocarcinoma
‐Metastatic Tumor
Pleomorphic adenoma
• Most common epithelial tumor‐benign mixed‐
cell tumor.
• Presentation‐adult life with painless, smooth,
firm non‐tender, slowly growing in the upper
outer quadrant
• Examination‐ most cases, arising from the
orbital portion of the lacrimal gland. CT scan
bony excavation of the lacrimal gland fossa.
• Treatment‐‐ Lateral orbitotomy
TUMOURS OF EYELIDS
LidTumors
Benign tumours:
– Simplepapilloma
–Naevus
–Angioma
–Haemangioma
–Neurofibroma
–Sebaceousadenoma
Pre‐cancerous conditions.
–Solar keratosis,
– Carcinoma‐in‐situ
– xerodermapigmentosa
Malignant tumours
• Squamous cell carcinoma
• Basal cell carcinoma
Nonmelanoma skin cancers account for more than 1/3rd of all cancers; Basal cell
carcinoma (BCC) accounts for 92.5% of all eyelid tumours; Squamous cell
carcinoma (SCC) accounts for 5% and Sebaceous gland carcinoma (SGC) for
around 1%
BCC SCC SGC
Incidence Most common 2nd most common Least common
Origin Basal cells of the
epidermis, cells fail to
mature and keratinise
Arise from the
differentiated cells of
the epidermis, mature
and keratinise
Arise from the
meibomian glands or
the glands of Zeis
Site Lower lid, medial
canthus, upper lid and
lateral canthus
Lower lid Upper eyelid
Predisposing factors UV light UV light, burn scars,
traumatic
ulcers,immunocom
promised, irradiation
Recurrent chalazion
and irradiation
BCC
Clinical characteristics:
Present as small, firm, upraised
nodules with umbilicated or
ulcerated centre;
Raised, rolled, pearly translucent
margins with fine telangiectasias
Minimally invasive Management:
•Surgery, Fresh tissue
micrographic surgical approach
(Moh’s surgery)
•Cryosurgery, electrodessication
and curettage : small tumors
•Radiotherapy and chemotherapy
: Inoperable tumors
SCC
Clinical characteristics:
Indurated, scaly, elevated
plaque,with a punched out
ulcerated area with
serosanguienous and
indurated edges, surface
crusting
Highly invasive and
metastatic
Management:
•Wide surgical excision
with frozen section
control and immediate
reconstruction
•Moh’ s surgery
•Exenteration : orbital
invasion
•Radiotherapy and
chemotherapy
SGC
Clinical characteristics:
Elderly female with
persistent unilateral
blepharoconjunctivitis or
painless upper lid nodule
(recurrent chalazion)
Invasive and metastatic
Management:
•Complete wide surgical
excision –treatment of
choice
•Preferably with frozen
section control or adjunctive
radio and chemotherapy
•Exenteration in invasive
tumors
Conjunctival tumors
Conjunctival tumors
Non‐pigmented tumours
I.Congenital: dermoid and lipodermoid
(choristomas).
II.Benign: simple granuloma, papilloma,
adenoma, fibroma and angiomas.
III.Premalignant: intraepithelial epithelioma
(Bowen's disease).
IV.Malignant: epithelioma or squamous cell
carcinoma, basal cell carcinoma.
Pigmented tumours
I.Benign: naevi or congenital moles.
II.Precancerous melanosis: superficial
spreading melanoma and lentigo maligna
(Hutchinson's freckle).
III.Malignant: primary melanoma
(malignant melanoma).
Uveal Tract Tumors
TUMOURS OF CHOROID
Benign
– Naevus
– Haemangioma
– Melanocytoma
– Choroidal osteoma
Malignant
– Melanoma
TUMOURS OF CILIARY BODY
Benign
– Hyperplasia
– Benign cyst
– Meduloepithelioma
Malignant
– Melanoma
TUMOURS OF IRIS
Benign
– Naevus
– Benign cyst
– Naevoxanthoendothelioma
Malignant
– Melanoma
Iris melanoma
Iris naevus
Choroidal Melanoma
• Typically begins asymptomatic, however, blurred, VF
loss and flashes/floaters may all be symptoms
• 2 varieties: circumscribed and diffuse
• Elevated, mottled lesion
• Most melanomas arise from pre‐existing, benign
choroidal nevi.
• Exudative retinal detachments are seen with tumors
more that 4 mm thick.
• Lipofuscin, an orange pigment, is characteristically
seen at the level of the retinal pigment epithelium
(RPE).
• Some large melanomas, especially those involving the
ciliary body, have prominent episcleral feeder vessels.
• Distant metastasis
• Radiation most common treatment modality
• Enucleation treatment of choice for high risk
melanomas
Ciliary body melanomas
• 10% of uveal melanomas – only visualised when pupil is
widely dilated.
• Presentation depends on size and location – lens
• subluxation or localised lens opacities, sentinal vessels,
erosion into anterior chamber, posterior extension
retinal detachment.
• Ultrasound may be necessary
• Treatment – enucleation, local resection, radiotherapy
• Prognosis is poor as presentation is usually late
Ciliary body melanoma
Picture on left showing
black mass in red reflex
Picture on right
showing tumour
pushing on and
displacing the lens
Choroidal melanoma/ Malignantmelanoma
85% of uveal melanomas, most common during
sixth decade of life
Raised pigmented oval shaped
mass(occasionally amelanotic)
Commonly asymptomatic – found on routine
fundal examination – may cause decreased visual
acuity or defect in visual field – can cause an
exudative retinal detachment, secondary glaucoma,
cataract or uveitis
Choroidal melanoma/MM
Peripheral MM MM at macula
Diagnosis of choroidalmelanoma
Ocular ultrasound – gives a measurement of size of •
tumour particularly the height, also differentiates
between a normal retinal detachment (RD) and RD
caused by tumour
MRI of orbits and optic nerves to check for extra scleral
•
spread
Fluorescein angiography, shows increased vascularity
•
and leakage from tumour
Management of choroidal
melanoma
Consider visual acuity of involved eye •
Size, location, extent and apparent •
activity of involved eye
State of fellow eye •
General health and age of patient •
Treatment of choroidalmelanoma
Radioactive plaques •
Enucleation •
Cyclotron – generated charged
particle radiation •
Photocoagulation •
Trans pupillary thermotherapy •
Localised resection •
Exenteration •
Palliation with chemotherapy •
TUMORS OF RETINA
Primary tumours
Neuroblastic tumours.
–Sensory retina (retinoblastoma and astrocytoma)
–Pigment epithelium (benign epithelioma; melanotic malignant
tumours).
Mesodermal angiomata
–Cavernous haemangioma
Phakomatoses
–Angiomatosis retinae (von hippel‐lindau disease)
–Tuberous sclerosis (bourneville’s disease),
–Neurofibromatosis (von recklinghausen’s disease)
–Encephalo‐trigeminal angiomatosis (Sturge‐weber Syndrome).
Secondary tumours
•Direct extension e.g., from malignant melanoma of the
choroid.
•Metastatic carcinomas from the gastrointestinal tract,
genitourinary tract, lungs, and pancreas.
•Metastatic sarcomas.
•Metastatic malignant melanoma from the skin.
Retinoblastoma
• Incidence ‐ 1 in 18,000 to 1 in 20,000 live birth
• Av. age of diagnosis – 18 months (B/l 12mths,U/L
24mths)
• Unilateral (2/3rd) and Bilateral (1/3rd) .
• Heritable (Germline mutation) and non‐heritable
(Somatic mutation).
• Approximately 4% of all Pediatric tumors.
• 250‐300 new cases/yr in US, about 5000 new cases of
retinoblastoma are found worldwide each year
Indirect ophthalmoscopy
- Solitary/ Multiple yellow- white retinal tumors
with prominent retinal feeder vessels
USG - masses with high
reflectivity that block sound and
calcification
Needle biopsy - rarely done
(tumor seeding and orbital
invasion)
CT scan/MRI - extraocular and
intracranial extension
International Classification Of Retinoblastoma
(ICRB)
Risk of loss of Eye
Very low risk Low
risk
Moderate risk
High risk
Very high risk
CHEMOTHERAPY
• Purpose of chemotherapy is chemoreduction.
• Chemoreduction – use of chemotherapy to reduce the
tumour size so that local treatment can be applied for
ultimate tumour control.
• Chemotherapy alone is never a cure of retinoblastoma
and almost always requires intensive local therapy.
OPECRegimen
Oncovin : alkaloids, interferes with mitosis , blocking metaphase, neurotoxic.
Cisplatin: Heavy metal, penetrates cns & bone. S/E highly emetic, ototoxic, renal
toxicity, neuropathy.
Etoposide: semi synthetic derivative of podophyllotoxin,a plant glycoside. Arrests
cell cycle in G2 phase ,causes DNA breaks by affecting topoisimerase II function.
S/E G I disturbance, alopecia, leucopenia.
Cyclophosphamide: Alkylating agent, cross linkages with DNA strand thereby
impairing repair, single most effective drug. S/E alopecia, hge cystitis.
•D0‐Vincristine 1.5mg/m2 Cyclophosphamide‐600mg/m2
•D1 –Cisplatin 60 mg/m2
•D2 –NIL
•D3 –Etoposide 120mg/m2
•Repeated every 21 day
External beam radiotherapy
• Fallen out of favour in many centers
• risk of dev of nonocular cancers in survivor of germinal RB
• Pt. radiated during first year of life are 2‐8 times more likely to
develop second cancer than those radiated after the age of
one year
Abramson et al ophthalmic Genet 2001;108:1868‐1876
• The 30 year cumulative incidence for a second cancer in
bilateral retinoblastoma is 35% for patients who received
EBR compared with 6% for those who did not
Roarty et al. Ophthalmology 1988;95:1583‐87
Indications of EBRT
– Tumours located within the macula
– Multifocal tumours, ineffective to local
therapy
– Bilateral advanced intraocular disease
– Failure of focal therapy
– Extraocular or metastatic disease along
with chemotherapy
40-45 Gy, # 150-200 cGy 4-5 wks
Local treatment options for RB
• Laser photocoagulation
• Cryotherapy
• Thermotherapy
• Plaque radiotherapy
Laser photocoagulation
• Laser‐ diode ,argon, or xenon arc photocoagulation
• Used as a primary therapy or recurrent/new tumour
• For tumor 4.5 mm or less in basal diameter and 2.5
mm or less in thickness with no vitreous seeds.
• Tx is directed to delimit the tumor and coagulate all
blood supply to the tumor
• Two or three session at 1 month interval are usually
adequate to control most tumor
• It offers a 70% tumor control rate and a 30% recurrence
rate
• Not used with chemoreduction b/s it causes vascular
coag. and minimizes chemotherapy delivery to tumours
• Complication‐ transient serous RD, retinal vascular
occlusion, retinal traction, retinal hole, preretinal fibrosis
CRYOTHERAPY
• Useful method for managing equatorial and peripheral small
retinoblastoma(3.5 mm/2.0mm thickness without vitreous
seeding)
• May be used as a primary t/t or as a sec. t/t for recurrent
tumor
• Tumor destruction is usually achieved with one or two
session of triple freeze thaw cryotherapy delivered at 1
month interval
• 90% of tumor <3mm D are cured permanently with
cryotherapy
• Cryotherapy administered 24 hour before chemotherapy
significantly increased the intravitreal penetration of
carboplatin
CRYOTHERAPY
THERMOTHERAPY
• Method of delivering heat to the eye using ultrasound, microwaves, or
infrared radiation
• Applies focused heat directly to tumour at sub photocoagulation levels
(42‐60ºc) → apoptosis of tumor cells
• Peripapillary tumour can be treated effectively with thermotherapy
• Tumour < 3 mm in base & < 2mm in thickness can be treated with
thermotherapy alone
• Done by either a transpupillary route (T T T) through operating microscope
or an indirect ophthalmoscope system or trans scleral route
• 810 nm infrared laser – 1.6 ‐ 3.0 mm spot x 350‐1500 mw x 1‐5 minute
ENUCLEATION
• Indications
– Advanced intraocular disease (stage v)
– Active tumour in a blind eye, eg. long standing RD
– Glaucoma from tumour invasion
– Failure of other treatment
– anterior chamber involvement or choroid,
– optic nerve or orbital tumor extension
– No expectation for useful vision eg. Vit hge
PLAQUE RADIOTHERAPY
• A method of brachytherapy in which
a radioactive implant is placed on
the sclera over the base of
retinoblastoma to irradiate the
tumor trans‐scleral
• I125 is most commonly used
• It requires an average of 2‐4 days
of treatment to deliver the total
dose of 4000 cGy to the apex of the
tumor
Management of Advanced Retinoblastoma
• Following Categories are included under the definition of
advanced retinoblastoma:
• Group E tumors (International Classification Of
Retinoblastoma)
• Tumors with high risk histopathologic characteristics
following enucleation.
• Orbital Retinoblastoma.
• Metastatic Retinoblastoma.
Tumors of the eye

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Tumors of the eye

  • 1. MEDICAL SURGICAL NURSING- II UNIT – II NURSING MANAGEMENT OF PATIENTS WITH DISORDERS OF EYE TOPIC : TUMORS OF THE EYE PRESENTED BY Mrs. SOUMYA SUBRAMANI, M.Sc.(N) LECTURER, MSN DEPARTMENT CON- SRIPMS, COIMBATORE.
  • 3. What are ocular tumors ? Ocular tumors can appear on the eyelids, in the eye (conjunctiva, choroid or retina) and in the orbit (the cavity that houses the eyeball).
  • 4. • Eye neoplasms can affect all parts of the eye, and can be a benign tumor or a malignant tumor (cancer). • Eye cancers can be primary (starts within the eye) or metastatic cancer (spread to the eye from another organ). • The two most common cancers that spread to the eye from another organ are breast cancer and lung cancer. • Other less common sites of origin include the prostate, kidney, thyroid, skin, colon and blood or bone marrow.
  • 5. Early diagnosis and treatment is necessary. Time is of the essence to save vision, the eye and even the life of the patient in the most serious cases.
  • 6. There are several types of benign and malignant tumors that affect the eye and its different structures Include : 1Tumors of the Orbit 2Tumors of the Lacrimal Gland 3Tumors of the Eyelid 4Tumors of the Conjunctiva 5 Tumors of the Uveal tract 6 Tumors of the Retina
  • 8. A lesion in the orbit? Decide whether it is an ocular lesion OR a non-ocular lesion, i.e. is it involving the globe or involving the structures outside the globe. If it is a non-ocular lesion, see the lesion is involving which space.
  • 9.
  • 10.
  • 11. Intraconal space tumors:  Benign  Dermoid cyst  Capillary and cavernous hemangioma  Optic nerve lesions ◦ Optic neuritis ◦ Optic nerve glioma ◦ Optic nerve meningioma  Schwannoma of 3rd and 6th cranial nerve  malignant  Lymphoma- more prevalent neoplasm in adults  Rhabdomyosarcoma- children
  • 12. Extraconal space tumors : Etiology  Benign  Dermoid cyst  Schwannoma of the trochlear nerve  Metastatic  Lacrimal gland tumors
  • 13. DERMOID CYSTS usually occur in children and make up 4% to 6% of orbital tumors. Painless mass, free from the skin, with variable ocular displacement. Mostly located near the lacrimal fossa or nasal bone. Grow slowly, remodeling adjacent bones or stuctures.
  • 14.
  • 15. RHABDOMYOSARCOMA Most common primary malignant tumor of the orbit in children. A highly malignant tumor. Av age of presentation:7 yrs. M>F
  • 16. presents with rapidly progressive exophthalmos. • Originates from extra-ocular muscles, • nasopharynx, or paranasal sinuses. Usually present in the superomedial orbit and • may produce bone destruction.
  • 17. Lacrimal Gland Tumors Benign ‐ Pleomorphic adenoma ‐ Schwannoma ‐ Dermoid ‐Neurofibroma Malignant ‐ Adenoid Cystic Carcinoma ‐ Malignant Mixed Tumour of the Lacrimal Gland ‐Adenocarcinoma ‐Metastatic Tumor
  • 18. Pleomorphic adenoma • Most common epithelial tumor‐benign mixed‐ cell tumor. • Presentation‐adult life with painless, smooth, firm non‐tender, slowly growing in the upper outer quadrant • Examination‐ most cases, arising from the orbital portion of the lacrimal gland. CT scan bony excavation of the lacrimal gland fossa. • Treatment‐‐ Lateral orbitotomy
  • 19.
  • 22. Malignant tumours • Squamous cell carcinoma • Basal cell carcinoma
  • 23. Nonmelanoma skin cancers account for more than 1/3rd of all cancers; Basal cell carcinoma (BCC) accounts for 92.5% of all eyelid tumours; Squamous cell carcinoma (SCC) accounts for 5% and Sebaceous gland carcinoma (SGC) for around 1% BCC SCC SGC Incidence Most common 2nd most common Least common Origin Basal cells of the epidermis, cells fail to mature and keratinise Arise from the differentiated cells of the epidermis, mature and keratinise Arise from the meibomian glands or the glands of Zeis Site Lower lid, medial canthus, upper lid and lateral canthus Lower lid Upper eyelid Predisposing factors UV light UV light, burn scars, traumatic ulcers,immunocom promised, irradiation Recurrent chalazion and irradiation
  • 24. BCC Clinical characteristics: Present as small, firm, upraised nodules with umbilicated or ulcerated centre; Raised, rolled, pearly translucent margins with fine telangiectasias Minimally invasive Management: •Surgery, Fresh tissue micrographic surgical approach (Moh’s surgery) •Cryosurgery, electrodessication and curettage : small tumors •Radiotherapy and chemotherapy : Inoperable tumors SCC Clinical characteristics: Indurated, scaly, elevated plaque,with a punched out ulcerated area with serosanguienous and indurated edges, surface crusting Highly invasive and metastatic Management: •Wide surgical excision with frozen section control and immediate reconstruction •Moh’ s surgery •Exenteration : orbital invasion •Radiotherapy and chemotherapy SGC Clinical characteristics: Elderly female with persistent unilateral blepharoconjunctivitis or painless upper lid nodule (recurrent chalazion) Invasive and metastatic Management: •Complete wide surgical excision –treatment of choice •Preferably with frozen section control or adjunctive radio and chemotherapy •Exenteration in invasive tumors
  • 26. Conjunctival tumors Non‐pigmented tumours I.Congenital: dermoid and lipodermoid (choristomas). II.Benign: simple granuloma, papilloma, adenoma, fibroma and angiomas. III.Premalignant: intraepithelial epithelioma (Bowen's disease). IV.Malignant: epithelioma or squamous cell carcinoma, basal cell carcinoma.
  • 27. Pigmented tumours I.Benign: naevi or congenital moles. II.Precancerous melanosis: superficial spreading melanoma and lentigo maligna (Hutchinson's freckle). III.Malignant: primary melanoma (malignant melanoma).
  • 28. Uveal Tract Tumors TUMOURS OF CHOROID Benign – Naevus – Haemangioma – Melanocytoma – Choroidal osteoma Malignant – Melanoma TUMOURS OF CILIARY BODY Benign – Hyperplasia – Benign cyst – Meduloepithelioma Malignant – Melanoma
  • 29. TUMOURS OF IRIS Benign – Naevus – Benign cyst – Naevoxanthoendothelioma Malignant – Melanoma Iris melanoma Iris naevus
  • 30. Choroidal Melanoma • Typically begins asymptomatic, however, blurred, VF loss and flashes/floaters may all be symptoms • 2 varieties: circumscribed and diffuse • Elevated, mottled lesion • Most melanomas arise from pre‐existing, benign choroidal nevi.
  • 31. • Exudative retinal detachments are seen with tumors more that 4 mm thick. • Lipofuscin, an orange pigment, is characteristically seen at the level of the retinal pigment epithelium (RPE). • Some large melanomas, especially those involving the ciliary body, have prominent episcleral feeder vessels. • Distant metastasis • Radiation most common treatment modality • Enucleation treatment of choice for high risk melanomas
  • 32.
  • 33. Ciliary body melanomas • 10% of uveal melanomas – only visualised when pupil is widely dilated. • Presentation depends on size and location – lens • subluxation or localised lens opacities, sentinal vessels, erosion into anterior chamber, posterior extension retinal detachment. • Ultrasound may be necessary • Treatment – enucleation, local resection, radiotherapy • Prognosis is poor as presentation is usually late
  • 34. Ciliary body melanoma Picture on left showing black mass in red reflex Picture on right showing tumour pushing on and displacing the lens
  • 35. Choroidal melanoma/ Malignantmelanoma 85% of uveal melanomas, most common during sixth decade of life Raised pigmented oval shaped mass(occasionally amelanotic) Commonly asymptomatic – found on routine fundal examination – may cause decreased visual acuity or defect in visual field – can cause an exudative retinal detachment, secondary glaucoma, cataract or uveitis
  • 37. Diagnosis of choroidalmelanoma Ocular ultrasound – gives a measurement of size of • tumour particularly the height, also differentiates between a normal retinal detachment (RD) and RD caused by tumour MRI of orbits and optic nerves to check for extra scleral • spread Fluorescein angiography, shows increased vascularity • and leakage from tumour
  • 38. Management of choroidal melanoma Consider visual acuity of involved eye • Size, location, extent and apparent • activity of involved eye State of fellow eye • General health and age of patient •
  • 39. Treatment of choroidalmelanoma Radioactive plaques • Enucleation • Cyclotron – generated charged particle radiation • Photocoagulation • Trans pupillary thermotherapy • Localised resection • Exenteration • Palliation with chemotherapy •
  • 40. TUMORS OF RETINA Primary tumours Neuroblastic tumours. –Sensory retina (retinoblastoma and astrocytoma) –Pigment epithelium (benign epithelioma; melanotic malignant tumours). Mesodermal angiomata –Cavernous haemangioma Phakomatoses –Angiomatosis retinae (von hippel‐lindau disease) –Tuberous sclerosis (bourneville’s disease), –Neurofibromatosis (von recklinghausen’s disease) –Encephalo‐trigeminal angiomatosis (Sturge‐weber Syndrome).
  • 41. Secondary tumours •Direct extension e.g., from malignant melanoma of the choroid. •Metastatic carcinomas from the gastrointestinal tract, genitourinary tract, lungs, and pancreas. •Metastatic sarcomas. •Metastatic malignant melanoma from the skin.
  • 42. Retinoblastoma • Incidence ‐ 1 in 18,000 to 1 in 20,000 live birth • Av. age of diagnosis – 18 months (B/l 12mths,U/L 24mths) • Unilateral (2/3rd) and Bilateral (1/3rd) . • Heritable (Germline mutation) and non‐heritable (Somatic mutation). • Approximately 4% of all Pediatric tumors. • 250‐300 new cases/yr in US, about 5000 new cases of retinoblastoma are found worldwide each year
  • 43. Indirect ophthalmoscopy - Solitary/ Multiple yellow- white retinal tumors with prominent retinal feeder vessels USG - masses with high reflectivity that block sound and calcification Needle biopsy - rarely done (tumor seeding and orbital invasion) CT scan/MRI - extraocular and intracranial extension
  • 44. International Classification Of Retinoblastoma (ICRB) Risk of loss of Eye Very low risk Low risk Moderate risk High risk Very high risk
  • 45. CHEMOTHERAPY • Purpose of chemotherapy is chemoreduction. • Chemoreduction – use of chemotherapy to reduce the tumour size so that local treatment can be applied for ultimate tumour control. • Chemotherapy alone is never a cure of retinoblastoma and almost always requires intensive local therapy.
  • 46. OPECRegimen Oncovin : alkaloids, interferes with mitosis , blocking metaphase, neurotoxic. Cisplatin: Heavy metal, penetrates cns & bone. S/E highly emetic, ototoxic, renal toxicity, neuropathy. Etoposide: semi synthetic derivative of podophyllotoxin,a plant glycoside. Arrests cell cycle in G2 phase ,causes DNA breaks by affecting topoisimerase II function. S/E G I disturbance, alopecia, leucopenia. Cyclophosphamide: Alkylating agent, cross linkages with DNA strand thereby impairing repair, single most effective drug. S/E alopecia, hge cystitis. •D0‐Vincristine 1.5mg/m2 Cyclophosphamide‐600mg/m2 •D1 –Cisplatin 60 mg/m2 •D2 –NIL •D3 –Etoposide 120mg/m2 •Repeated every 21 day
  • 47. External beam radiotherapy • Fallen out of favour in many centers • risk of dev of nonocular cancers in survivor of germinal RB • Pt. radiated during first year of life are 2‐8 times more likely to develop second cancer than those radiated after the age of one year Abramson et al ophthalmic Genet 2001;108:1868‐1876 • The 30 year cumulative incidence for a second cancer in bilateral retinoblastoma is 35% for patients who received EBR compared with 6% for those who did not Roarty et al. Ophthalmology 1988;95:1583‐87
  • 48. Indications of EBRT – Tumours located within the macula – Multifocal tumours, ineffective to local therapy – Bilateral advanced intraocular disease – Failure of focal therapy – Extraocular or metastatic disease along with chemotherapy
  • 49. 40-45 Gy, # 150-200 cGy 4-5 wks
  • 50. Local treatment options for RB • Laser photocoagulation • Cryotherapy • Thermotherapy • Plaque radiotherapy
  • 51. Laser photocoagulation • Laser‐ diode ,argon, or xenon arc photocoagulation • Used as a primary therapy or recurrent/new tumour • For tumor 4.5 mm or less in basal diameter and 2.5 mm or less in thickness with no vitreous seeds. • Tx is directed to delimit the tumor and coagulate all blood supply to the tumor • Two or three session at 1 month interval are usually adequate to control most tumor • It offers a 70% tumor control rate and a 30% recurrence rate • Not used with chemoreduction b/s it causes vascular coag. and minimizes chemotherapy delivery to tumours • Complication‐ transient serous RD, retinal vascular occlusion, retinal traction, retinal hole, preretinal fibrosis
  • 52. CRYOTHERAPY • Useful method for managing equatorial and peripheral small retinoblastoma(3.5 mm/2.0mm thickness without vitreous seeding) • May be used as a primary t/t or as a sec. t/t for recurrent tumor • Tumor destruction is usually achieved with one or two session of triple freeze thaw cryotherapy delivered at 1 month interval • 90% of tumor <3mm D are cured permanently with cryotherapy • Cryotherapy administered 24 hour before chemotherapy significantly increased the intravitreal penetration of carboplatin
  • 54. THERMOTHERAPY • Method of delivering heat to the eye using ultrasound, microwaves, or infrared radiation • Applies focused heat directly to tumour at sub photocoagulation levels (42‐60ºc) → apoptosis of tumor cells • Peripapillary tumour can be treated effectively with thermotherapy • Tumour < 3 mm in base & < 2mm in thickness can be treated with thermotherapy alone • Done by either a transpupillary route (T T T) through operating microscope or an indirect ophthalmoscope system or trans scleral route • 810 nm infrared laser – 1.6 ‐ 3.0 mm spot x 350‐1500 mw x 1‐5 minute
  • 55. ENUCLEATION • Indications – Advanced intraocular disease (stage v) – Active tumour in a blind eye, eg. long standing RD – Glaucoma from tumour invasion – Failure of other treatment – anterior chamber involvement or choroid, – optic nerve or orbital tumor extension – No expectation for useful vision eg. Vit hge
  • 56. PLAQUE RADIOTHERAPY • A method of brachytherapy in which a radioactive implant is placed on the sclera over the base of retinoblastoma to irradiate the tumor trans‐scleral • I125 is most commonly used • It requires an average of 2‐4 days of treatment to deliver the total dose of 4000 cGy to the apex of the tumor
  • 57. Management of Advanced Retinoblastoma • Following Categories are included under the definition of advanced retinoblastoma: • Group E tumors (International Classification Of Retinoblastoma) • Tumors with high risk histopathologic characteristics following enucleation. • Orbital Retinoblastoma. • Metastatic Retinoblastoma.