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Melanom conjunctival Dr. Hapca Madalina.pptx
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- 2. Anamnesis • M, 36years, urban location • Chief Complaints: • Brown conjunctival lesion in the right eye, with significant growth over the past two months. • Past medical history: • Unremarkable • Socio-economic/Family history: • Normal • Personal history: • Non smoker, non alcoholic
- 3. History of Present Illness According tothe patient, the current condition began about two months ago, marked by the significant growth of a conjunctival lesion in the right eye. The patient presents to the Ophthalmology Department of Cluj County Emergency Clinical Hospital for diagnosis and treatment.
- 4. Ocular Examination Right eyeLeft eye BCVA 0.6 1 Pupillary light reflex Present Present Ocular Motility Normal Normal RAPD Absent Absent Slit lamp Round, prominent, vascularized, brown conjunctival lesion, 5mm diameter, located nasaly, perilesional conjunctival congestion and dilated, tortuous vessels converging towards the lesion (feeder vessles). the lesion lifts the upper eyelid off the ocular surface. Normal Fundus Normal Normal
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- 6. Provisional Diagnosis • OD: Suspicionof conjunctival malignant melanoma
- 7. Characteristics Conjunctival NevusPrimary Aquired melanocitosis (PAM) Pigmented intraocular tumor with extension to the subconjunctival region Age Any age, more often children Adults Any age Location Limbus, bulbar conjunctiva Any area of the conjunctiva Subconjunctival Pigmentation Uniform or absent Uniform, diffuse Intense, due to uveal melanin Growth Very slow (or static) Diffuse, slowly progressive Increases with the growth of the intraocular tumor Vascularization Absent Absent It may involve adjacent scleral or episcleral vessels Associated signs Mobile, intralesional cysts Flat pigmentation without cysts Associated with intraocular tumor Risk of malignancy Benign If there is severe atypia Malign (due to the origin of the primary tumor)
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- 9. Histo- pathological examination • Nodular lesionwith a diameter of 0.5 cm • Microscopy: Tumor proliferation consisting of epithelioid melanocytes with marked cytological atypia, arranged in nests and trabeculae. The tumor invades the corium to a maximum depth of 2.6 mm and also exhibits an intraepithelial component. • Mitotic activity: 2 mitoses/mm². • No evidence of angiolymphatic or perineural invasion. • A non-brisk lymphocytic inflammatory infiltrate is present within the tumor. • Resection margins: Clear. • The closest lateral resection margin is 1 mm from the junctional component. • The deep resection margin is 0.3 mm from the tumor. • At the periphery of the lesion, melanocytes without atypia are present within the stroma, most likely from a preexisting nevus. • Immunohistochemical profile: • Tumor cells are diffusely HMB45-positive. • PRAME is non-contributory. • D2-40 immunostaining does not reveal lymphatic tumor emboli. • Conclusion: The described morphological findings correspond to a conjunctival melanoma associated with a conjunctival nevus. pT1b (>1 but ≤2 quadrants) L0V0PNI0 R0
- 10. Final Diagnostic • OD: Conjunctivalmalignant melanoma
- 11. Postoperative period • BCVAAO: 1 sc • OD: Conjunctival hyperemia in the nasal sector, conjunctival sutures in place. • Discharge Recommendations: - Oncological evaluation for systemic screening and long-term follow-up. - Ophthalmologic evaluation (every 4-6 months in the first 4-5 years, then annually for recurrence detection). 1st day postop 1 month postop
- 12. Oncological evaluation pT1b (L0V0Pn0)N0 M0, R0 - STD IA CT CAP (26.10.2023): Right renal microlithiasis. No detectable secondary tumors on CT exam. Clinical status (7.11.2023): Performance index-1, no signs of local recurrence. Cerebral MRI (19.11.2023): Chronic pansinusitis. Normal brain MRI appearance. No detectable tumor masses in the orbits on cerebral MRI exam
- 13. Prognosis • Short term– good • Long term – poor; needs frequent follow-up. - at risk for metastasis Increased tumor thickness in conjunctival melanoma is associated with an increased risk of regional nodal and distant metastasis. In conjunctival melanomas with more than 2 mm thickness, regional nodal metastases are more frequent!
- 14. Conjunctival melanoma • Conjunctivalmelanoma represents 12% of conjunctival tumors and 2% of ocular malignancies. • 75% originate from an area of PAM (primary acquired melanosis) with atypia, 25% from a pre-existing junctional or compound nevus, and rarely de novo. • The 5-year mortality rate is 19%, and the 10-year mortality rate is 30%. • Metastasis occurs in 20-30% of cases, particularly in regional lymph nodes, lungs, brain, and liver. • Factors associated with a poor prognosis include: Caruncular location, at the fornix, or at the eyelid margin • Tumor thickness 2 mm. ≥
- 15. Conjunctival melanoma • A blackor gray vascularized nodule, which may be fixed to the episclera. • Amelanotic tumors can pose diagnostic challenges. • The limbus is a common site, but it can occur anywhere on the conjunctiva. • Conjunctival melanoma can extend into the eyelid. • Histology shows melanoma-like cellular atypia and invasion into the subepithelial stroma.
- 17. Efficacy of primarytherapies for conjunctival malignant melanoma
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- 19. Take home messages •Conjunctival melanoma is an uncommon but increasingly important and potentially fatal tumor of the eye. • Prognosis is poor, with recurrence and metastasis commonly occurring despite excision with tumor free margins. • Current management is wide local excision with cryotherapy to the margins. Adjuvant therapies can include topical chemotherapy, cryotherapy, and radiotherapy. • Follow-up should include referral to an oncologist.
- 20. References • Shields CL.Conjunctival melanoma: rare but deadly. American Academy of Ophthalmology; 2008 May 8. Available from: https:// www.aao.org/education/current-insight/conjunctival-melanoma-rare-deadly • Yanoff M, Duker JS. Atlas of Ophthalmology. 5th ed. Philadelphia: Elsevier; 2020 • American Academy of Ophthalmology. (2023). Basic and clinical science course: Section 04: Ophthalmic pathology and intraocular tumors (2023–2024 ed.). San Francisco, CA: American Academy of Ophthalmology. • Salmon, J. F. (2024). Kanski's clinical ophthalmology: A systematic approach (10th ed.). Elsevier. • Wong JR, Nanji AA, Galor A, Karp CL. Management of conjunctival malignant melanoma: a review and update. Expert Rev Ophthalmol. 2014 Jun;9(3):185-204. doi: 10.1586/17469899.2014.921119. PMID: 25580155; PMCID: PMC4285629.
Editor's Notes
- #7 Differential diagnosis includes the progenitors of CMM: PAM and conjunctival nevi. Like CMM, PAM is typically unilateral but presents as a flat, asymmetric, noncystic, pigmented patch on the conjunctiva or cornea. The lesion may be multifocal [23,34].
- #8 After clinical diagnosis is made based on history and slit lamp examination, the current standard of care is surgical excision. the mainstay of treatment of conjunctival melanoma in the majority of centers is wide local excision and biopsy with “no-touch” technique and cryotherapy to the margins. Local Anesthesia: parabulbar lidocaine 4%, 6 ml Surgical Procedure: Excision with 3 mm safety margins using the "no-touch" technique. Double freeze-thaw cryotherapy applied to the resection margins. Conjunctival closure with Vicryl 7.0 absorbable sutures. Postoperative Care: Betabioptal eye drops, 1 drop 3 times/day, and Betabioptal ointment, applied twice daily for 7 days.
- #15 It presents as a
- #16 Here is an an algorithm for approaching the management of CMM. After clinical diagnosis is made based on history, slit lamp findings, and imaging, the current standard of care is surgical excision. A trial of mitomycin C (MMC) can be considered prior to excision if there is significant primary acquired melanosis (PAM) for chemoreduction. Surgical excision includes wide margins (~4mm), cryotherapy to the margins, and closure or placement of amniotic membrane transplant (AMT). Sentinel lymph node biopsy (SLNB), if done, is usually done at time of excision but may be done afterwards. Adjuvant therapies include topical MMC or interferon alpha-2b (IFN-α2b), internal radiotherapy (brachytherapy) or external radiotherapy (teletherapy). All cases should be referred to an oncologist for detection of metastasis.
- #17 A total of 4 case series were identified in the literature that evaluated the outcomes of the surgical technique of wide excision with cryotherapy, involving 457 patients and 5 series involving 10 patients who underwent treatment with topical MMC as primary therapy. As shown in this table, primary surgical excision exhibited a more favorable outcome for CMM, with less exenteration and recurrence rates when compared with MMC alone. The poor outcomes of MMC when used as primary therapy for conjunctival melanoma (CMM) strongly emphasize the need to avoid its use as a primary treatment for CMM. MMC does not penetrate the basement membrane, restricting its effectiveness to surface lesions.
- #18 Metastasis and recurrence are unfortunately common for patients with CMM. In this section we will consider the main prognostic outcomes of local recurrence, regional lymph node metastasis, systemic metastasis, and melanoma-related mortality In this table is shown the main prognostic outcomes of local recurrence, regional lymph node metastasis, systemic metastasis, and melanoma-related mortality. A total of 770 cases revealed an overall average local recurrence of 40 % over a mean interval of 2.4 years. At 3.5 years after diagnosis, approximately 19% of cases exhibited lymph node metastasis. 5 large studies including 734 patients revealed an average frequency of systemic metastasis of 19% over a mean interval of 3.4 years. five large studies covering 734 cases revealed an average frequency of melanoma-related death following surgical resection with tumor-free margins of 18% (114/649) over a mean interval of 4.9 years