Medical Care
The goal of therapy in patients with Kaposi sarcoma is to relieve ocular irritation, mass effect, and disfigurement. Therapy is not curative. If the patient has no ophthalmic complaints, the tumor is usually observed. For AIDS-related Kaposi sarcoma, consider immune reconstitution with triple antiviral medication. [20, 21]
Kaposi sarcoma tends to respond to chemotherapy. Ophthalmologists may want to delegate the use of chemotherapy to a chemotherapist or an oncologist familiar with the use of the agents. If the patient has systemic involvement that requires chemotherapy, the eye lesion often resolves or is reduced markedly after starting this therapy. However, recurrence commonly follows after discontinuation of chemotherapy. If local treatment is indicated, defer for at least 4 weeks after systemic chemotherapy to allow for regression of the lesions.
Conventional Adriamycin, bleomycin sulfate, and vinblastine sulfate (ABV) combination therapy
Doxorubicin hydrochloride (Adriamycin) is administered at 40 mg/m2 every 4 weeks or 20 mg/m2 every 2-3 weeks.
Bleomycin sulfate is administered at 10 U/m2 every 2 weeks.
Vinblastine sulfate 1.4 mg/m2 with 2 mg maximum dose every 2 weeks has been recommended with ABV combination therapy. See Medication.
Single-agent therapies
Liposomal daunorubicin (DaunoXome) is administered 40 mg/m2 intravenously (IV) every 2 weeks until complete response; the response is comparable to ABV but with less resultant neutropenia. [22]
Pegylated liposomal doxorubicin (Doxil) is administered at 20 mg/m2 every 3 weeks; the response is comparable to ABV or BV but with either lower or similar neutropenia risk.
Liposomal anthracyclines have a prolonged half-life when compared with their unencapsulated counterparts.
Alternative therapies
Paclitaxel (Taxol) is administered at 135 mg/m2 IV over 3 hours every 3 weeks or a total dose of 100 mg/m2 given IV over 3 hours every 2 weeks. All patients should be premedicated with the following 3 medications to prevent hypersensitivity reactions:
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Dexamethasone 10 mg orally (PO) 12 hours before treatment
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Diphenhydramine 50 mg IV 30 minutes prior to treatment
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Cimetidine or ranitidine IV 30 minutes before treatment
If the absolute neutrophil count is greater than 1,000 cells/mcL, then continue with treatments. Reduce the dose of subsequent treatments by 20% if the neutrophil count falls below 500 cells/mcL. See the Absolute Neutrophil Count calculator.
Other chemotherapeutic agents used in Kaposi sarcoma include etoposide (VePesid), dactinomycin (Cosmegen), cisplatin (Platinol), and interferon alfa-n3 (Alferon N).
Intralesional chemotherapy [23]
Interferon alfa-2a administered at 0.5 mL of 3 million IU in a subconjunctival injection adjacent to the tumor was reported to be efficacious in a 46-year-old man with AIDS- related conjunctival Kaposi sarcoma. [24]
A 31-year-old man on highly active antiretroviral therapy (HAART) and systemic doxorubicin who initially received topical 3 million IU/mL of interferon alpha-2b eyedrops with no improvement received 3 injections (3 million IU in 0.5mL, 1.5 million IU in 0.5mL, and 1.5 million IU in 0.5 mL) over a 4-week period, leading to a dramatic decrease in tumor mass. [25]
Investigational medications
Human chorionic gonadotropin–associated factors
Because males are at a higher risk for developing Kaposi sarcoma, some investigators believe that female hormones may play a protective role. Hermans et al experienced complete or partial regression of skin lesions in 38% of patients with AIDS-related Kaposi sarcoma after intralesional injections of human chorionic gonadotropin (hCG). [26]
Samaniego et al demonstrated that human chorionic gonadotropin–associated factors (HAF) can induce apoptosis of Kaposi sarcoma cells in vivo and in vitro. [27]
Iron chelators
Simonart et al is investigating the role of regulating iron concentration in tumor cells. [28] They demonstrated that the addition of iron salts strongly stimulates the growth of Kaposi sarcoma and that the use of iron chelators inhibits growth. Their findings are disclosing new therapeutics in tumor management with iron-chelating agents.
Hermans et al speculate that the lower risk for developing Kaposi sarcoma in females may be because women have lower iron stores. [29] The analysis concludes that patients with renal transplants are more susceptible to Kaposi sarcoma because they tend to have higher iron loads from repeated blood transfusions.
Other experimental medications include fumagillin analogues (AGM 1470), bacterial cell-wall peptidoglycan (SP-PG derivatives), interleukin 4, and L-glutamine-tryptophan.
Intralesional cidofovir (an antiherpetic medication) injections have demonstrated poor results. Bailey et al have reported that all-trans -retinoic acid has not demonstrated optimistic results. [30]
Irradiation
Most patients respond immediately to radiation therapy, but recurrence is common. Consequently, irradiation is primarily used as a palliative measure. A typical treatment includes 2,000-3,000 cGy with the total dose given in fractions of 200-300 cGy per session over 3 weeks.
Ocular complications of radiation include lid atrophy, skin necrosis, ectropion, telangiectasia, tearing, eyelash loss, keratitis, cataract formation, conjunctival keratinization, skin erythema, keratoconjunctivitis sicca, alopecia, optic neuropathy, and lid notching.
Surgical Care
Cryotherapy protocol
Subcutaneous injection of 2% lidocaine with epinephrine applied directly under the lesion is administered.
Cryoprobe with circulating liquid nitrogen is set at -30°C. Cryoprobe is applied to the tumor and extended out to 1-2 mm of clear margin until the lesion becomes bright white.
Antibiotic ointment is applied to the treated site.
Follow-up care is arranged for the following day.
Ocular complications of cryotherapy include depigmentation and erythema, lid notching, and epilation.
Surgical excision
Indications for local excision include cosmetically disturbing lesions, discomfort, and obstruction of vision from tumor bulk. Consider treating a lesion to prevent entropion formation with trichiasis and exposure keratopathy and corneal ulcer.
Protocol for local excision is as follows:
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A cotton swab is soaked in 0.5% tetracaine hydrochloride, then applied directly over the tumor
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Lidocaine 2% with epinephrine is injected subconjunctivally with a 25-gauge needle
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Westcott scissors are used for blunt and sharp dissection to free the tumor. Clear margins of 1-2 mm are excised
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The specimen is marked for orientation
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Antibiotic ointment is applied, and the eye is patched
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Patient should receive follow-up care the next day.
Protocol for fluorescein angiography demarcation of conjunctival lesions is as follows:
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A 5 mL bolus of fluorescein dye is injected intravenously and photographed using a Zeiss SE-40 blue exciter filter.
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The fluorescein angiography is used to mark tumor vessels with heat cautery.
Recurrence usually occurs within 4 weeks after surgical excision.
Conjunctival tumors
Dugel et al recommended an effective treatment regimen based on the clinical and histopathologic stage of the tumor. [31]
If the lesion is stage I or II and confined to the bulbar conjunctiva, excisional biopsy with 1-2 mm of clear margins is suggested.
For stage III tumors involving the bulbar conjunctiva, surgical excision guided by fluorescein angiography delineation of tumor margins resulted in no lesion recurrence during 4-8 months of follow-up care.
Alternative treatment can include intralesional mitomycin [32] or intralesional injections of interferon alfa-2b [25]
Recurrence usually occurs within 4 weeks after surgical excision.
Eyelid tumors [33]
Stage I and II lesions may be treated with cryotherapy. Stage II lesions may require radiation therapy.
Consultations
If an eye lesion is identified, a thorough workup is necessary to rule out systemic involvement and HIV/AIDS infection.
After a thorough ophthalmologic examination, refer to an internist or oncologist/chemotherapist.
Disseminated Kaposi sarcoma may necessitate bronchoscopy, sigmoidoscopy, or lymph node biopsy for a definitive diagnosis and systemic chemotherapy. [34]
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Section of eyelid with Kaposi sarcoma lesion under high magnification. This tissue section demonstrates increased angiogenesis and spindle-shaped cells. Courtesy of Ben Glasgow, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
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Section of eyelid with Kaposi sarcoma lesion under high magnification. This tissue section demonstrates endothelium-lined vascular channels and proliferation of spindle-shaped cells. Increased angiogenesis with erythrocyte extravasation is observed. Courtesy of Ben Glasgow, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
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The inferior conjunctiva is involved more commonly than the superior conjunctiva in Kaposi sarcoma. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
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Kaposi sarcoma involvement of the eyelid. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
