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Case 30a
by Professor Chua & Dr. Tan
Aik Kah
This 27 year-old woman with AIDS was referred by the
medical department because of a 6-month history of a slowly enlarging left
nasal limbal lesion (Figure 1 and 2). The lesion was non-tender and her
vision in both eyes were normal. Slit-lamp showed the lesion was confined to
the conjunctiva without deep tissue invasion. Fundoscopy showed no signs of
cytomegaloviral retinitis. She had no skin lesions.
The first attending ophthalmologist diagnosed Kaposi's
sarcoma and started the patient on topical mitomycin C.
Figure 1. Left pigmented nasal
limbal lesion.
Figure 2. CLose-up view of the
lesion.
a. Do you agree with the diagnosis?
Although Kaposi's sarcoma (KS) is a common lesion in
AIDS, this lesion is not typical of KS. Conjunctival KS is typically bright red
and may be mistaken for subconjunctival haemorrhage. In addition, KS usually
involves the lower fornix. About 25% of AIDS patients develop KS. Of these,
about 20% have ocular involvement. The most common locations being the eyelid
and the conjunctiva. Rarely orbital involvement has been reported.
Ocular KS may be the initial manifestation of AIDS-related Kaposi's
sarcoma. Ocular KS are usually slow growing and rarely invasive. Treatment is
usually for cosmetically disturbing lesions, discomfort or visual obstruction
from large lesions. Treatment options include excision for well-delineated
lesion, cryotherapy and irradiation.
b. How would you manage this patient?
Excisional biopsy is the treatment of choice for a definite tissue
diagnosis. The lesion was excised en bloc following the instillation of
topical and subconjunctival anaesthesia.
c. What is the most likely diagnosis?
Squamous cell carcinoma.
The diagnosis was confirmed from the biopsy report and the lesion
appeared to have been excised with tumour free margin and the base.
Squamous cell carcinoma is the most common malignant tumour of the
conjunctiva and typically affects the limbus. It typically occurs in people
over the age of 50. Because of its limbal location, it may sometimes be misdiagnosed
as pterygium or pingueculae. SCC is rare amongst healthy young people, its
occurrence in patients under the age of 50 should raise the possibilities of
AIDS1. In African countries where AIDS is endemic, SCC is now
recognized as an AIDS defining disease2. It is reported to be as
high as 7.4% in some centres treating AIDS patients3. The
treatment of choice for conjunctival SCC is excision with at least 2mm free
margin. The patient should be followed up at regular interval for recurrence.
Adjunctive treatment includes: i. the use of cryotherapy in a double
freeze-thaw manner, to the edges of the uninvolved conjunctiva and Tenon
capsule and ii. intraoperative application of 100% ethanol to exposed sclera
to devitalize any remaining tumor cells4.
In Sarawak, there are currently 650
registered cases of HIV and AIDS (80% are acquired through sexual
intercourse). To our knowledge, there are only three cases of conjunctival
squamous cell carcinoma (including this patient) amongst our HIV and AIDS
patients. Nonetheless, any expanding conjunctival lesions in an AIDS patient
should be excised for tissue diagnosis.
References:
1. Fogla R, Biswas J, Kumar
SK, Madhavan HN, Kumarasamy N,
Solomon S. Squamous cell carcinoma of the conjunctiva as initial presenting sign
in a patient with acquired immunodeficiency syndrome (AIDS) due to human
immunodeficiency virus type-2. Eye. 2000. Apr;14 ( Pt 2):246-7.
2. Orem J, Otieno MW, Remick SC. AIDS-associated cancer in
developing nations. Curr Opin Oncol. 2004 Sep;16(5):468-76. Review.
3. Chisi SK, Kollmann MK, Karimurio J.
Conjunctival squamous cell carcinoma in patients with human immunodeficiency
virus infection seen at two hospitals in Kenya. East
Afr Med J. 2006 May;83(5):267-70.
4. Fraunfelder FT, Wingfield D:
Management of intraepithelial conjunctival tumors and squamous cell
carcinomas. Am J Ophthalmol 1983 Mar; 95(3): 359-63.
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