Diagnosis and Management of Conjunctival Leiomyosarcoma

1. Diagnosis and Management of Conjunctival Leiomyosarcoma Matthew D. Council, George J. Harocopos, Andrew J. Huang A. Huang is a consultant speaker for Allergan, Inc. (Category B). The authors have no financial interest in the subject matter of this poster.

2. Patient History • 77 y/o white male with PMHx of CVA, hypertension, gout, arthritis, and psoriasis • Past ocular history • Primary open angle glaucoma s/p failed trabeculectomy OD – now controlled on topical therapy • s/p cataract extraction/lens implant OU • Presents with asymptomatic conjunctival lesion at the temporal limbus of the left eye

5. Patient History • Lesion did not stain with fluoroscein or Rose Bengal • Patient referred for impression cytology and treated with topical interferon alpha-2b 1 million units/cc QID • Impression cytology revealed clusters of dysplastic cells and vacuolation of cytoplasm in cells • Follow-up revealed no regression of lesion on topical interferon therapy for three months • Patient underwent local excision with adjunctive double freeze-thaw cryotherapy

7. Pathology • Keratinized epithelium with proliferation of spindle cells • Mild to moderate pleomorphism with mitotic figures • Trace amount of dystrophic calcification seen • Staining: • Smooth muscle actin (+) • Desmin (-) • Ki-67 relatively high proliferative index • Cytokeratin and CK7 highlighted epithelium only • CD68, Melan-A, CD34, Factor XIIIA, and S100 (-) in tumor cells

8. Patient History • Pathologic diagnosis = low grade leiomyosarcoma • Patient referred to oncologist for a metastatic work-up • Metastatic work-up (including CBC, CMP, CT of chest/abdomen, MRI of orbits/brain) was within normal limits without evidence of metastasis or distant primary • 12 month follow-up with ophthalmology and oncology showed the patient to be doing well without evidence of recurrent local or distant disease

9. Leiomyosarcoma Malignant neoplasm of smooth muscle Most commonly arises in stomach, small intestine, retroperitoneum, and uterus Can arise anywhere in the body from smooth muscle in blood vessels Represents 5-10% of soft tissue sarcomas Orbit is the most common ocular location but is rare

10. Conjunctival Leiomyosarcoma • 2 prior reports in the literature • Case Report #1 • Patient with xeroderma pigmentosum • Limbal lesion diagnosed as leiomyoma • Biopsy of recurrence showed leiomyosarcoma • Patient underwent exenteration and subsequently died from unrelated CNS melanoma metastasis • White VA, Damji KF, Richards JS, Rootman J. Leiomyosarcoma of the conjunctiva. Ophthalmology. 1991 Oct; 98(10): 1560-4.

11. Conjunctival Leiomyosarcoma • Case Report #2 • Patient with 26 year history of persistent eye irritation and redness • Biopsy of lesion revealed squamous carcinoma, treated with excision • Patient suffered from intermittent corneal ulceration treated as presumptive HSV keratitis • Patient underwent PKP and biopsy of “pseudopterygia” lesions • Lesions diagnosed as leiomyosarcoma • Patient underwent extensive excision of conjunctiva and biopsies including muscle • Multiple positive biopsies led to subtotal exenteration • De Wolff-Rouendaal D. Xeroderma pigmentosum with ophthalmological symptoms. Ophthalmologica. 1976; 173: 290-291.

12. Conjunctival Leiomyosarcoma • Conjunctival leiomyosarcoma is a rare ocular surface tumor. • This is the first reported case of primary conjunctival leiomyosarcoma successfully treated with local excision and cryotherapy. • This case highlights the importance of biopsying suspicious ocular surface lesions.