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.

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

Patient History Lesion did not stain with fluoroscein or Rose Bengal 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 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 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 Follow-up revealed no regression of lesion on topical interferon therapy for three months Patient underwent local excision with adjunctive double freeze-thaw cryotherapy Patient underwent local excision with adjunctive double freeze-thaw cryotherapy

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

Patient History Pathologic diagnosis = low grade leiomyosarcoma Pathologic diagnosis = low grade leiomyosarcoma Patient referred to oncologist for a metastatic work-up 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 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 12 month follow-up with ophthalmology and oncology showed the patient to be doing well without evidence of recurrent local or distant disease

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

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

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

Conjunctival Leiomyosarcoma Conjunctival leiomyosarcoma is a rare ocular surface tumor. 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 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. This case highlights the importance of biopsying suspicious ocular surface lesions.