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Orbital tumors
1. M A L I G N A N T
T U M O R S O F
A N T E R I O R
S E G M E N T
O F E Y E
P R E S E N T E R : D R . A A K A N K S H A B E L E
M O D E R A T O R : D R . K H E R M A ’ A M
C O N D U C T O R : D R . S A P A N S I R
J A W A H A R L A L N E H R U M E D I C A L
C O L L E G E
9. Ocular
examination
• THE MAGIC “SEVEN P”
• 3 P from history :
- Pain – infection, inflammation, hemorrhage, bone & neural invasion
- Progression – Hours : hemorrhage; Days : infection; Week : inflammation;
Months : Malignant; Year : benign
- Past medical history – Thyroid, trauma, cancer
• 4 P from examination :
- Proptosis
- Palpation – Retrobulbar resistant mass
- Pulsation – AV fistula
- Periorbital change
10. Periorbital Changes
Sign Etiology
Salmon-colored mass in the cul-de-sac Lymphoma
Eyelid retraction, vascular congestion over rectus
muscle
Thyroid Eye Disease
Corkscrew conjunctival vessels AVF
S-shaped eyelid Plexiform neurofibroma or lacrimal gland mass
Ecchymosis of eyelid skin Metastatic neuroblastoma, leukemia, amyloidosis
Frozen globe Metastases or zygomycosis
Edematous swelling of lower lid Meningioma, inflammatory tumor, metastases
Facial asymmetry Fibrous dysplasia
11. Tumors of
Anterior
Segment of
the Eye
• Tumors of the Orbit
• Tumors of the Eyelid
• Tumors of the Conjunctiva
• Tumors of the Uveal Tract
12. Rhabdomyosarcoma
• Myogenic tumor
• Most common primary malignant tumor of orbit in children.
• Highly malignant.
• Age of presentation – 7 yrs.
• Origin – extra-ocular muscles, nasopharynx or paranasal sinus.
• Usually present in – superomedial orbit
• May produce bone destruction.
• Presentation – rapidly progressive exophthalmos.
• CT- bulky aggressive looking mass, slightly hyperdense, shows uniform enhancement.
13. • Treatment :
- Rhabdomyosarcoma can grow rapidly and if the tumor grows into the brain or spreads to
the lung, survival is poor.
- Prompt biopsy of a rhabdomyosarcoma followed by a combination of surgery,
chemotherapy and irradiation offers the best chance of survival.
- Orbital exenteration reserved for very severe cases.
14. C L I N I C A L
P I C T U R E &
H I S T O L O G Y
15. Lacrimal Gland
Tumors• Very rare
• Histopathologically 2 types – Epithelial & Non Epithelial
• Most common benign– Mixed benign tumor
• Most common malignant – adenoid cystic
adenocarcinoma.
• Clinical features :
- Firm mass near medial canthus
- Must be differentiated clinically from dacrocystitis
• Management :
- Dacryocystectomy
- Irradiation or chemotherapy
- May later require reconstruction of drainage system
16. Mucoepidermoid
carcinoma
• Rare
• Age – 12- 81yrs
• Clinical features : painless, slow
growing mass in lacrimal fossa
• Management : Perform
exenteration, radiation & resection
of involved orbital bone for patient
with high-grade tumors
• Extirpation with or without adjuvant
radiation for patient with low grade
tumor
17. Adenoid cystic
carcinoma
• 2nd most common epithelial tumor of
lacrimal gland
• Age of presentation – avg 40 yrs.
• Clinical features : Pain, globe
displacement, mass, lacrimation, ptosis
• CT – globular or round, irregular & serrated
borders
• MRI – assessing the invasion of the tumor
into cavernous sinus, brain & bone marrow.
• Pathology : Cribriform (swiss cheese),
solid, sclerosing, comedocarcinomatous &
tubular.
18. • Management :
- Surgical removal of tumor & postoperative radiotherapy
- Radiation therapy – 50-60 Gy after local resection of adenoid
cystic carcinoma delays the onset of recurrence
- Intra-arterial chemotherapy.
19. Adenocarcinoma
• More common in males
• Age – 18- 80 yrs
• 7% of epithelial neoplasm of lacrimal gland
• Rapidly growing mass, exceeding the limits of
adequate surgical excision at the time of
presentation
• Clinical feature : Proptosis, pain, globe
displacement, ptosis, diplopia
• Management :
- Exenteration followed by radiation therapy
- Monobloc craniofacial orbitectomy combined
with regional LN dissection.
23. 1. Nodular or nodulo- ulcerative :-
• Clinical features :
- Elevated mass
- Thickened, fairly well-defined erythematous margins
- Central ulcer
- Loss of cilia
• Histopathology :
- well-defined lesion
- Central ulcer
- Lobules of closely-packed nuclei
- Connective tissue septa
24. 2. Morpheaform or sclerosing type :
• Clinical features :
- Poorly defined border
- Lacks ulceration
- Loss of cilia
• Histopathology :
- Poorly defined margins and cords of tumor
cells
- Deeper invasion into dermis
25. Treatment :
- Small lesion - primary excision; Larger lesion: biopsy
prior to definitive surgery
- Final surgery depends on extent of lesion
- Frozen sections of chemosurgery usually advisable
- Closure: primary closure, skin flap or graft
- Cryotherapy: recurrent lesions, usually in medial
canthus
- Orbital exenteration for deep invasive lesions
- Irradiation for recurrent cases
26. Squamous cell carcinoma
• Arises from actinic keratosis
• 2nd most common eyelid tumor
• Risk factors : UV rays or Exposure to sunlight, Immunosuppression, Albinism, Chronic
skin lesion
• Clinical features :
- Nodular or plaque like lesion
- Ulceration
- Rolled out edges
- Greyish white keratinization
- Can metastasize to regional lymph node
• Histopathology :
- Proliferative invasive squamous cells
- Dyskeratosis & mitotic activity
• Treatment : Similar to BCC; may require orbital exenteration
27. Sebaceous
carcinoma
• About 5 % of all malignant eyelid tumors; can metastasize
to regional lymph nodes and distant organs
• Origins: Meibomian glands, Zeis glands, or caruncle
• May be multicentric in origin
• Clinical features :
- More common in upper eyelid
- Usually presents as a solitary yellow nodule, resembling a
chalazion
- Loss of cilia
- Unlike basal cell carcinoma, it does not ulcerate early
- Diffuse form resembles unilateral blepharoconjunctivitis.
- Diffuse form is a result of pagetoid growth pattern
- Can involve both eyelids and conjunctiva
- Zeis gland tumor--yellowish nodule near eyelid margin
28. • Pathology :
- Lobules or sheets of malignant tumor cells
- More anaplastic than basal cell carcinoma
- Contain lipid that can be seen with lipid stains
• Spread – intraepithelial / “pategoid” spread
- Lymphatic & hematogenous spread.
• Management :
- Same as BCC
- Wide local excision & close follow up
29. • Muir-Torre Syndrome :
- Hereditary: usually autosomal dominant
- Sebaceous gland tumors (hyperplasia,
adenoma or carcinoma)
- Keratoacanthomas
- Internal malignancy (colon cancer and
others)
30. Malignant
melanoma
• Common in fair skinned
• Rare in eyelid
• C/f : pigmented eyelid mass, ulcerates
& bleeds
• May be nodular
• Spread : regional lymph nodes &
systemically
• Histology : atypical melanocytes within
dermis
• Management : wide surgical excision &
systemic follow up
31. Merkel cell tumor
(neuroendocrine
carcinoma of
skin)
• Arises from merkel cells
• Clinical features :
- Older patients
- Reddish blue sausage shaped lesion
- Usually in upper eyelid
- Metastasis & death in 25% cases
• Pathology :
- Nodules of large basophilic cells
- Resembles sebaceous gland carcinoma
• Management :
- Excision & eyelid reconstruction
- Similar to BCC
32. Tumors of
Conjunctiva
• Mostly benign, rarely
malignant.
• Conjunctival epithelial tumors
are more common than
conjunctival stromal tumors.
34. Intraepithelial
neoplasia
• Presents in late adulthood
• Juxtalimbal fleshy avascular
mass
• May become vascular &
extend on cornea
• Rarely transform to malignant
35. Primary acquired
melanosis (PAM)
• Presents in late adulthood
• Unilateral
• Irregular areas of flat brown
pigmentation
• Involve any part of conjunctiva
• Pathology :
- Without atypia – benign
- With atypia – pre-malignant
- Abnormal melanocytes in basal layer
of epithelium
36. Conjunctival
melanoma1. From PAM with atypia – most common, sudden
appearance of nodules.
2. From naevus – very rare, sudden increase in size
or pigmentation.
3. Primary – Solitary nodule, mostly juxtalimbal.
Treatment :
- Localized – excision, adjunctive cryotherapy
- Diffuse – excision of nodules, adjunctive cryotherapy
or mitomycin C
- Orbital recurrence – excision & radiotherapy or
exenteration
37. Conjunctival squamous
cell carcinoma
• Clinical features :
- Fleshy, placoid limbal mass
- Usually in interpalpebral fissure
- Occasionally in fornices
- May show leukoplakia (hyperkeratosis)
- May have a papillomatous configuration
- Can invade the orbit and globe
- Distant metastasis very rare
38. • Pathology
1. Neoplastic cells arising in epithelium
2. Conjunctival intraepithelial neoplasia (CIN)
- Mild : ("Dysplasia")
Partial thickness replacement of epithelium by neoplastic cells
- Severe : ("Carcinoma in situ")
Full thickness replacement of epithelium by neoplastic cells
3. Invasive squamous cell carcinoma
a. Breaches the basement membrane
b. Invades the conjunctival stroma
c. Can invade the globe and orbit
d. Mucoepidermoid and spindle cell variants more invasive
39. • Treatment – surgical
- Varies with the clinical findings
- Good preoperative clinical evaluation
- Local retrobulbar anaesthesia
- Superficial alcohol partial epitheliectomy
- Excision by partial lamellar sclerokeratoconjunctivectomy
- "no touch" approach
- Double freeze-thaw cryotherapy to conjunctival margins
- Closure of conjunctiva with absorbable sutures
- Supplemental treatment
1. Topical chemotherapy - mitomycin C or 5-fluorouracil (5FU)
2. Irradiation (plaque or external beam)
40. Kaposi sarcoma
• Affects patients with AIDS
• Vascular, slow growing tumor of low malignancy
• One or more red lesions; resembles
conjunctival hemorrhage
• Pathology :
- Malignant proliferation of vascular tissue
- Typical slit-like vascular channels
• Management :
- Excision
- Chemotherapy
- Very sensitive to radiotherapy
42. Iris
• Malignant melanoma :
1. Circumscribed type :
• Clinical features :
- Nodular mass arising from iris stroma
- Secondary ectropion, angle involvement, cataract
- Spontaneous hyphema can occur
- Growth is the most reliable sign of malignancy
• Pathology : Usually low-grade spindle B cells, Occasional
epithelioid cells
• Management :
- Varies with clinical circumstances
- Usually removal by sector iridectomy
- Iridocyclectomy if trabecular meshwork involved
- Plaque radiotherapy or enucleation if not resectable
43. 2. Diffuse type :
• Clinical features :
- Acquired hyperchromic heterochromia
- Ipsilateral secondary glaucoma
- Gonioscopy shows angle involvement
• Pathology :
- Often spindle cells
- Loosely cohesive epithelioid cells are frequent
• Management :
- varies with clinical circumstances
- Often require enucleation
- Fine need aspiration prior to enucleation
- Plaque radiotherapy in selected cases
• Prognosis :
- Metastatic patterns similar to posterior uveal melanoma
- Metastasis most often involves liver
- Mortality rates vary from 5 to 14%
44. Ciliary body melanoma
• 10% of uveal melanoma
• Only visualized when pupil is widely dilated
• Presentation : depends on size & location.
- Lens – subluxation or localized lens opacities
- Sentinal vessels
- Erosion into anterior chamber
- Posterior extension – retinal detachment
• Treatment : enucleation, local resection, radiotherapy
• Prognosis - poor
45. Tests for Eye
Cancer
• Vision
• Direct & indirect ophthalmoscopy
• Slit lamp bio microscopy
• Gonioscopy
• Routine blood investigations
• Imaging :
- Ultrasound
- Ultrasound bio microscopy
- Optical coherence tomography
- Fluorescein angiography
- Chest x ray
- Computed tomography
- Magnetic resonance imaging
- Biopsy – FNAC, Incisional or excisional biopsy
- Fine needle biopsy, Liquid biopsy
47. TNM Staging
• Iris Melanoma
• T1: The tumor is limited to the iris.
• T1a: The tumor is in one quadrant (one-fourth) or less of the iris.
• T1b: The tumor is in more than one quadrant of the iris.
• T1c: The tumor is only in the iris, but there is melanomalytic glaucoma. This means that a buildup of certain cells in the
eye blocks the flow of fluid in the eye, causing pressure.
• T2: The tumor has joined or grown into the ciliary body and/or choroid.
• T2a: The tumor has joined or grown into the ciliary body and/or choroid with melanomalytic glaucoma.
• T3: The tumor has joined or grown into the ciliary body and/or choroid and extends to the sclera (outer wall of the
eyeball).
• T3a: The tumor has joined or grown into the ciliary body and/or choroid and extends to the sclera in association with
melanomalytic glaucoma.
• T4: The tumor has spread to the outside of the eyeball, the optic nerve, or to the eye socket. This is called extraocular
extension.
• T4a: The tumor has spread less than 5 millimeters (mm) outside of the eye.
• T4b: The tumor has spread more than 5 mm outside of the eye.
49. • T1: The tumor is size category 1.
• T1a: The tumor is size category 1 and does not involve the ciliary body or other parts of the eye.
• T1b: The tumor is a category 1 and involves the ciliary body.
• T1c: The tumor is size category 1 that does not involve the ciliary body. But, there is a very small area (5 mm
or less in diameter) of visible spread beyond the eyeball. This is called extraocular spread.
• T1d: The tumor is a size category 1 that involves the ciliary body with extraocular spread less than 5 mm.
• T2: The tumor is size category 2.
• T2a: The tumor is size category 2 and does not involve the ciliary body or other parts of the eye.
• T2b: The tumor is size category 2 and involves the ciliary body.
• T2c: The tumor is size category 2 that does not involve the ciliary body. But, there is a very small area (5 mm
or less in diameter) of visible spread beyond the eyeball.
• T2d: The tumor is size category 2 that involves the ciliary body with extraocular spread less than 5 mm.
50. • T3: The tumor is size category 3.
• T3a: The tumor is size category 3 and does not involve the ciliary body or other parts of the eye.
• T3b: The tumor is size category 3 and involves the ciliary body.
• T3c: The tumor is size category 3 that does not involve the ciliary body. But, there is a very small area
(5 mm or less in diameter) of visible spread beyond the eyeball.
• T3d: The tumor is size category 3 that involves the ciliary body with extraocular spread less than 5
mm.
• T4: The tumor is size category 4.
• T4a: The tumor is size category 4 and does not involve the ciliary body or other parts of the eye.
• T4b: The tumor is size category 4 and involves the ciliary body.
• T4c: The tumor is size category 4 that does not involve the ciliary body. But, there is a very small area
(5 mm or less in diameter) of visible spread beyond the eyeball.
• T4d: The tumor is size category 4 that involves the ciliary body with extraocular spread less than 5
mm.
• T4e: The tumor is any size category with extraocular spread of more than 5 mm in diameter.
51. Node (N)
NX: The regional lymph
nodes cannot be evaluated.
N0 (N plus zero): There is
no regional lymph node
metastasis.
N1: There is regional lymph
node metastasis.
52. Metastasis
(M)
MX: Distant metastasis cannot be evaluated.
M0 (M plus zero): There is no distant metastasis.
M1: There is metastasis to other parts of the body.
M1a: There is metastasis to other parts of the body and
the largest metastasis is 3 centimeters (cm) or less in
diameter.
M1b: There is metastasis to other parts of the body and
the largest metastasis is between 3.1 cm and 8 cm in
diameter.
M1c: There is metastasis to other parts of the body and
the largest metastasis is larger than 8 cm in diameter.
53. Grade &
Histopathology
• If the cancer looks similar to healthy tissue and contains
different cell groupings, it is called differentiated or a low-
grade tumor.
• If the cancerous tissue looks very different from healthy
tissue, it is called poorly differentiated or a high-grade
tumor.
• After a biopsy or when the tumor is surgically removed,
doctors may look at the types of cells that are in the
tumor; this is called histopathology. Three types of
histopathology patterns may be present in the tumor:
- Spindle cell melanoma (the cells are longer and tapered
at the ends)
- Epithelioid melanoma (the cells are oval-shaped)
- Mixed cell melanoma (both spindle and epithelioid)
54. • Generally, a tumor made up of spindle cells has a better
prognosis than a tumor made up of epithelioid cells.
• The tumor is given a grade (G) to describe the
composition of its cells.
• A lower grade generally indicates a better prognosis
than a higher grade.
• GX: Grade cannot be evaluated
• G1: A spindle cell melanoma
• G2: A mixed cell melanoma
• G3: An epithelioid melanoma
55. Combining
T, N, M & G
staging
• Stage I: The tumor is size category 1 and does not involve
the ciliary body or other parts of the eye, nor has it spread to
the regional lymph nodes or to other areas of the body (T1a,
N0, M0).
• Stage IIA: The tumor is either a size category 1 that may or
may not involve the ciliary body, with or without extraocular
extension, or it is a size category 2 that does not involve the
ciliary body. There is no spread to the regional lymph nodes
or to other areas of the body (T1b, T1c, T1d, or T2a; N0, M0).
• Stage IIB: The tumor is either a size category 2 that involves
the ciliary body but has not spread beyond the eyeball, or it is
a size category 3 that has not spread to the ciliary body or
eyeball. It has not spread to the regional lymph nodes or to
other areas of the body (T2b or T3a; N0, M0).
56. • Stage IIIA: Stage IIIA describes any one of these conditions:
• A tumor of size category 2 with extraocular spread to a diameter of 5 mm or less, with or without
ciliary body involvement that has not spread to the lymph nodes or to other parts of the body (T2c
or T2d, N0, M0)
• A tumor of size category 3 that may or may not involve the ciliary body, with or without extraocular
spread to a diameter of 5 mm or less, but hasn’t spread to the lymph nodes or to other parts of the
body (T3b or T3c, N0, M0)
• A tumor of size category 4 that does not involve the ciliary body and has not spread to the lymph
nodes or to other parts of the body (T4a, N0. M0)
• Stage IIIB: Stage IIIB describes any one of these conditions:
• The tumor is a size category 3 with ciliary body involvement and extraocular spread that has not
spread to the lymph nodes or to other parts of the body (T3d, N0, M0).
• The tumor is a size category 4 with or without ciliary body involvement that may or may have
spread outside the eyeball. It has not spread to the regional lymph nodes or to other areas of the
body (T4b or T4c, N0, M0).
57. • Stage IIIC: The tumor is a size
category 4 that involves the ciliary
body and has spread outside the
eyeball. However, it has not spread to
the regional lymph nodes or to other
areas of the body (T4d or T4e; N0,
M0).
• Stage IV: This stage describes a
tumor of any size that has spread to
the lymph nodes and/or to other parts
of the body outside of the eye (any T,
N1, M0; or, any T, any N, M1).
58. COMS Tumor
classification
• Collaborative Ocular Melanoma
Study:-
• Small: 1 mm to 3 mm in height and 5
mm to 16 mm in diameter
• Medium: 3.1 mm to 8 mm in height
and not more than 16 mm in diameter
• Large: More than 8 mm in height and
more than 16 mm in diameter
60. Surgery
Iridectomy: Removal of part of the iris .
Iridotrabeculectomy: Removal of part of the iris, plus a
small piece of the outer part of the eyeball.
Iridocyclectomy: Removal of a portion of the iris and the
ciliary body.
Transscleral resection: Surgically removing just a
melanoma of the ciliary body or choroid.
Enucleation: Removal of the entire eyeball.
Orbital exenteration: Removal of the eyeball and some
surrounding structures such as parts of the eyelid and
muscles, nerves, and other tissues inside the eye socket.
62. Laser Therapy
• Transpupillary thermotherapy
- Most common type of laser treatment for eye melanoma
- Uses infra red light to heat & kill tumor
- Side effects – bleeding, retinal detachment, blockage of vessels in eye, high risk of recurrence.
- Used as adjuvant treatment after brachytherapy
• Laser photocoagulation
- Uses highly focused, high energy light beams to burn tissue
- Rarely used now due to high risk of recurrence
• Main concern with laser therapy is damage to parts of eye that could result in loss of vision.
63. Chemotherapy
• Used only when cancer has become widespread.
• Possible side effects :
• - Hair loss
• Mouth sores
• Loss of appetite
• Nausea and vomiting
• Diarrhea or constipation
• Increased chance of infections (from having too few white blood cells)
• Easy bruising or bleeding (from having too few blood platelets)
• Fatigue (from having too few red blood cells)