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Malignant tumors of anterior segment of eye .pptx
- 1. M A LI 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 . S . O P H T H A L M O L O G Y
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- 3. WHO Classification ofEye Tumors • Tumors of Conjunctiva & Caruncle:- • epithelial tumors • conjunctival squamous cell carcinoma • conjunctival spindle cell carcinoma • adenosquamous carcinoma • keratoacanthoma • hereditary benign intraepithelial dyskeratosis of the conjunctiva • sebaceous carcinoma • oncocytoma • conjunctival squamous papilloma • tumor-like lesion • cysts • reactive epithelial hyperplasia • pseudoglandular and pseudoepitheliomatous hyperplasia
- 4. • melanocytic tumors •conjunctival melanoma • conjunctival melanocytic intraepithelial neoplasia • conjunctival junctional, compound, and subepithelial nevi • inflamed juvenile conjunctival nevus • blue nevus of the conjunctiva • Spitz nevus of the conjunctiva • benign epithelial melanosis of the conjunctiva • hematolymphoid tumors • lymphomas • papillary/follicular conjunctivitis • reactive lymphoid hyperplasia • soft tissue tumors • leiomyosarcoma • rhabdomyosarcoma • Kaposi sarcoma • angiosarcoma • fibrous histiocytoma • conjunctival stromal tumor • myxoma • hemangioma • lymphangioma • neurofibroma • schwannoma • juvenile xanthogranuloma
- 5. • hamartomas andchoristomas • epibulbar choristoma • epibulbar osseous choristoma • phakomatous choristoma • secondary tumors
- 6. Tumors of Iris& Ciliary Body • tumors of the iris • iris melanoma • melanocytic nevus • ocular melanocytosis • Lisch nodule • pigmented epithelial cyst • implantation cyst • ectopic lacrimal gland • histiocytic tumors • tumors of the ciliary body • uveal melanocytoma • adenocarcinoma • ciliary body adenoma • adenomatous hyperplasia • reactive epithelial hyperplasia • medulloepithelioma • leiomyoma • schwannoma • glioneuroma
- 7. Tumors of thelacrimal gland • epithelial tumors • adenoid cystic carcinoma • mucoepidermoid carcinoma • carcinosarcoma • carcinoma ex pleomorphic adenoma • pleomorphic ademona • myoepithelial tumors • oncocytic tumors • rare neoplasms • epithelial-myoepithelial carcinoma • low-grade intraductal carcinoma • secretory carcinoma • hybrid neoplasms • acinic cell carcinoma • Warthin tumor • hematolymphoid tumors • lymphomas • reactive lymphoid hyperplasia • secondary tumors
- 8. Tumors of thelacrimal drainage system • epithelial tumors • squamous cell carcinoma • non-keratinizing squamous cell carcinoma • adenocarcinoma • mucoepidermoid carcinoma • adenoid cystic carcinoma • lymphoepithelial carcinoma • exophytic papilloma • inverted papilloma • other tumors • melanoma • lymphomas • secondary tumors
- 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-coloredmass 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 theEye • 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 IN I C A L P I C T U R E & H I S T O L O G Y
- 15. Lacrimal Gland Tumors • Veryrare • 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 mostcommon 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 commonin 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.
- 20. Tumors of Eyelids •Benign Tumors - Epithelial tumors - Melanocytic tumors - Adnexal cystic lesions - Sweat gland origin - Hair follicle origin - Miscellaneous • Malignant Tumors - Basal cell carcinoma - Squamous cell carcinoma - Sebaceous carcinoma - Malignant melanoma
- 21. Signs of Malignancy • Painless,slow growing lesion • Ulceration, bleeding & crusting • Pigmentary changes • Destruction of normal eyelid margin • Central ulceration • Loss of vellus hair
- 22. Basal Cell Carcinoma • Malignantcutaneous tumor • Do not metastasize, extensive tissue invasion • Rodent ulcer • Risk factors – UV radiation, fair skin, unable to tan, exposure to arsenic. • Types : - Nodular - nodulo- ulcerative - sclerosing
- 23. 1. Nodular ornodulo- 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 orsclerosing 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 : - Smalllesion - 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 infair 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 (neuroendocrin e carcinomaof 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 • Mostlybenign, rarely malignant. • Conjunctival epithelial tumors are more common than conjunctival stromal tumors.
- 33. Classification of conjunctival tumors • Benign - Naevus -Papilloma - Epibulbar dermoid - Lipodermoid • Pre-malignant - Primary acquired melanosis - Intraepithelial neoplasia • Malignant - Melanoma - Squamous cell carcinoma - Kaposi sarcoma
- 34. Intraepithelia l neoplasia • Presentsin 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 melanoma 1. From PAMwith 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. Neoplasticcells 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 • Affectspatients 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
- 41. Uveal tract tumors •Iris - Neavi – benign, flat to slightly elevated lesions - Melanoma – 50- 60 yrs, elevated & pigmented lesions Treatment – local resection +/- radiotherapy Prognosis - good
- 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
- 46. S T AG I N G
- 47. TNM Staging • IrisMelanoma • 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.
- 48. Ciliary body &Choroid Melanoma
- 49. • T1: Thetumor 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: Thetumor 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: Theregional 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 metastasiscannot 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 • Ifthe 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, atumor 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 • CollaborativeOcular 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
- 59. T R EA T M E N T
- 60. Surgery Iridectomy: Removal ofpart 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.
- 61. Radiation Therapy • Brachytherapy(Plaque therapy) • External beam radiation therapy - Proton beam radiation therapy - Stereotactic radiosurgery
- 62. Laser Therapy • Transpupillarythermotherapy - 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 onlywhen 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)
- 64. Immunotherapy & targeteddrugs • Immunotherapy :- - Pembrolizumab - Ipilimumab - (uveal eye melanoma) • Targeted drugs :- - Only in BRAF mutation - BRAF inhibitors : - Vemurafenib, dabrafenib & encorafenib
- 65. Bibliography • Yanoff’s Ophthalmology5th edition • Kanski’s Clinical Ophthalmology 9th edition • Post graduate ophthalmology by Zia Chaudhary 2nd edition, volume 2 • Parson’s Diseases of the Eye 22nd edition
- 66. Thank you Take home message “Detect early,treat better.” “Save sight, save life”