Case Reports

Isolated bulbar conjunctival Kaposi’s sarcoma Neil Murray, FRACO, FRACS* Peter McCluskey, FRACO, FRACS* Denis Wakefield, FRACP, FRCPA* .Paul Beaumont, FRACSf

Abstract We present a case of an isolated bulbar conjunc- tival Kaposi’s sarcoma in a man with HIV infection. This is an unusual site for the initial presentation of Kaposi’s sarcoma and in this site can be confused with a caroticocavernous fistula, cavernous haeman- gioma or chronic subconjunctival haemorrhage. Biopsy is required to confirm the diagnosis in isolated disease. Local radiotherapy is the preferred treatment for isolated Kaposi’s sarcoma.

Key words: Conjunctiva, Kaposi’s sarcoma, management.

Case report A 27-year-old white man presented in February 1991 with a one-year history of a gradually en- larging right conjunctival vascular lesion. He had been assaulted seventeen months previously, re- ceiving blows to the right side of his face. A presumptive diagnosis of traumatic right carotico- cavernous fistula was made, but orbital and cerebral CT scans were normal. The lesion continued to enlarge and its appearance began to worry the patient. Several ophthalmologists were consulted over the next few weeks and further imaging of the orbit was performed. A digital subtraction carotid angiogram was normal.

In February 1991, the patient was reviewed at our clinic. HIV. serology had been performed in March

1990 and was positive, but the patient did not reveal this fact to his physicians until he was specifically questioned at our clinic. Clinical examination revealed a vascular area with dilated tortuous vessels, over the nasal bulbar conjunctiva (Figure 1). There was no proptosis, ophthalmoplegia or bruit, and systemic examination was otherwise normal. A provi- sional diagnosis of Kaposi’s sarcoma was made and an incisional biopsy was performed.

Histopathological examination of the conjunctival biopsy showed a cellular tumour with angiomatoid spaces (Figure 2). There were small, thin-walled vascular channels and more solid areas of spindle cells. Factor VIII antigen staining was strongly positive for normal capillary endothelium, but weakly positive over only a few tumour cells. These features are consistent with Kaposi’s sarcoma.

Figure 1 Vascular lesion of right bulbar conjunctiva. This is a superficial spreading Kaposi’s sarcoma.

*From the Laboratory of Ocular Immunology, School of Pathology, University of PO Box 1, Kensington, NSW2033, and Ophthalmology Department, St Vincent’s Hospital, Sydney, N S W 2000. 7187 Macquarie Street, Sydney, N S W 2000. Reprints: Dr PJ McCluskey.

Isolated bulbar conjunctival Kaposi’s sarcoma 81

Figure 2 Histopathology of the conjunctival biopsy showing spindle-shaped cells and angioid spaces.

In the absence of multifocal disease the patient was treated with fractionated superficial radio- therapy. The tumour regressed completely within six weeks and has not recurred after 18 months of follow-up (Figure 3).

Discussion Kaposi's sarcoma occurs in up to 25% of homo- sexual patients with advanced HIV infection and is an AIDS-defining illness. In patients with AIDS, isolated conjunctival lesions are uncommon, with an incidence of 4%.' It typically involves the palpebral conjunctiva and eyelids, most commonly in the lower fornix. Isolated bulbar conjunctival Kaposi's sarcoma is rare. Clinically, such unusual Kaposi's sarcomas may mimic caroticocavernous fistula, cavernous haemangioma or subconjunctival haemorrhage. Biopsy may be necessary to confirm the diagnosis.

This case illustrates the usefulness of incisional biopsy in the diagnosis of an unusual conjunctival

Figure 3 Regression of the conjunctival lesion after superficial radiotherapy.

lesion. This is particularly so in HIV-infected patients, who may have atypical presentations.

Treatment options for Kaposi's sarcoma include: chemotherapy, radiotherapy and immun~therapy. '~~,~ Conjunctival Kaposi's sarcoma tends to be slow growing and is rarely invasive. Treatment should reflect the clinical situation. Multifocal disease requires systemic therapy and ocular lesions should be carefully observed for regression. Radiotherapy or simple excision are options for local disease, although recurrences can occur after excision.'

References 1. Shuler JD, Holland GN, Miles SA, Miller BJ, Grossman I.

Kaposi's sarcoma of the conjunctiva and eyelids associated with the acquired immunodeficiency syndrome. Arch Ophthalmol 1989;107:858-62.

2. Brunt AM, Goodman AG, Phillips RH, Youle MS, Gazzard BG. Short communication on the safety of intravenous chemotherapy and zidovudine when treating epidemic Kaposi's sarcoma. AIDS 1989;3:457-60.

3. Cooper JS, Fried PR. Treatment of aggressive epidemic Kaposi's sarcoma of the conjunctiva by radiotherapy. Arch Ophthalmol 1988;106:20-1.

02 Australian and New Zealand Journal of Ophthalmology 1994; 22(1)