Ophthalmic Manifestations of Kaposi’s Sarcoma

What Is Kaposi’s Sarcoma?

Kaposi's sarcoma (KS) is a type of cancer originating in the cells that line blood vessels or lymphatic vessels. Usually manifesting as lesions on the skin or mucous membranes, it is characterized by aberrant blood vessel growth and tumor formation. These lesions can range from pink to dark purple and may appear singly or in clusters.

Kaposi's sarcoma is caused by infection with human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). Although sexual contact is the most typical way for this virus to spread, organ transplants and blood transfusions can also spread it, as well as from mother to child during childbirth. There are several forms of Kaposi's sarcoma:

• Classic Kaposi's Sarcoma: This primarily affects older adults of Mediterranean or Eastern European descent.

• Endemic (African) Kaposi's Sarcoma: This occurs predominantly in sub-Saharan Africa and affects children and young adults, often in association with HIV/AIDS.

• Epidemic (AIDS-related) Kaposi's Sarcoma: This is the most common form today, particularly in people with weakened immune systems due to HIV infection or immunosuppressive medications.

• Iatrogenic (Transplant-Related) Kaposi's Sarcoma: Those who have had organ transplants and are on immunosuppressive medications to prevent organ rejection have this type of disease.

What Are the Ophthalmic Manifestations of Kaposi’s Sarcoma?

Ophthalmic manifestations of Kaposi's sarcoma (KS) can occur when the disease affects the eyes or surrounding tissues. While less common than skin lesions, ocular involvement can lead to significant complications and requires careful monitoring and management. One of the most common ocular manifestations of KS is the development of lesions on the eyelids or conjunctiva. These lesions may appear as raised nodules or masses and can cause irritation, redness, and swelling. Sometimes, they may interfere with vision if they obstruct the line of sight.

Kaposi's sarcoma lesions can also affect the orbit, the bony socket that houses the eye. Tumors in the orbit can cause proptosis or bulging of the eye, pain, double vision, and limitation of eye movements. If treatment for orbital tumor compression of the optic nerve is not received, the condition may result in blindness or visual impairment.

In addition to the direct involvement of the eye and surrounding structures, KS can manifest as secondary immunosuppression complications. For example, individuals with AIDS-related KS are at increased risk of opportunistic infections such as cytomegalovirus (CMV) retinitis, which can cause inflammation and damage to the retina, leading to vision loss if not promptly treated.

How Can the Ophthalmic Manifestations of Kaposi’s Sarcoma be Diagnosed?

Clinical examination, ocular signs of Kaposi's sarcoma (KS), and other methods, such as imaging studies and possibly tissue biopsy, can be used to diagnose the condition.

1. Clinical Examination: An ophthalmologist will conduct a thorough examination of the eyes, eyelids, conjunctiva, and orbit. This may involve visual acuity testing, slit-lamp biomicroscopy, and dilated fundus examination to identify characteristic lesions such as nodules or masses.

2. Imaging Studies: Computed tomography (CT) or magnetic resonance imaging (MRI) may be used to assess the extent of orbital involvement and evaluate the relationship of KS lesions to adjacent structures. Imaging helps determine tumors' size, location, and characteristics within the orbit and any associated complications.

3. Tissue Biopsy: A tissue biopsy may be performed in cases where diagnosis remains uncertain or malignancy is suspected. Biopsy samples obtained from suspicious lesions are examined histologically to confirm the presence of KS cells, characterized by spindle-shaped cells and vascular proliferation.

Healthcare providers can accurately identify ocular involvement in KS through these diagnostic approaches and tailor treatment plans to preserve vision and manage complications effectively.

How Can Ophthalmic Manifestations of Kaposi’s Sarcoma be Treated?

Treating ophthalmic manifestations of Kaposi's sarcoma (KS) involves a multidisciplinary approach to control tumor growth, preserve vision, and manage associated complications. Many factors influence the treatment decision, including the degree of ocular involvement, the patient's general health, and the particulars of the KS lesions.

• Surgery: Surgical excision may be considered for localized KS lesions affecting the eyelids, conjunctiva, or orbit. This approach removes visible tumors and alleviates symptoms such as pain, irritation, or visual obstruction. In cases of orbital involvement, surgical debulking may be performed to reduce tumor burden and relieve pressure on surrounding structures.

• Radiation Therapy: External beam radiation therapy (EBRT) or brachytherapy may be used to target KS lesions that are not amenable to surgical resection or for palliative purposes in cases of advanced disease. Radiation therapy can help control tumor growth, alleviate symptoms, and improve local tumor control.

• Chemotherapy: Systemic chemotherapy may be indicated for patients with extensive or rapidly progressing ocular KS, particularly in cases of HIV/AIDS-related disease. Chemotherapeutic agents such as liposomal Doxorubicin, Paclitaxel, or Vincristine may be used as monotherapy or combination regimens to induce tumor regression and improve outcomes.

• Immunotherapy: Immunomodulatory agents such as interferon-alpha may be considered adjunctive therapy for KS, particularly in cases where antiretroviral therapy is ineffective or contraindicated. Interferon-alpha has been shown to have antiviral and antiangiogenic effects, which may help control KS progression.

• Antiretroviral Therapy (ART): In cases of HIV/AIDS-related KS, optimizing antiretroviral therapy is essential to suppress HIV replication, restore immune function, and prevent disease progression. ART improves overall immune status and has a direct antitumor effect by reducing viral load and inhibiting Kaposi's sarcoma-associated herpesvirus (KSHV) replication.

• Supportive Care: Measures such as lubricating eye drops, topical steroids, or antibiotics may be prescribed to manage ocular symptoms such as dryness, inflammation, or infection associated with KS lesions.

Overall, treating ophthalmic manifestations of Kaposi's sarcoma requires a tailored approach that addresses the specific needs and challenges posed by ocular involvement while considering the patient's overall health status and treatment goals. Close collaboration between ophthalmologists, oncologists, infectious disease specialists, and other healthcare providers is essential to optimize outcomes and ensure comprehensive care for individuals with ocular KS.

Conclusion

Patients with Kaposi sarcoma should have routine ocular examinations, the frequency of which varies according to the severity of eye involvement. Most eye lesions may be observed. Severe eye pain, deformity, and malfunction suggest local treatment. A complete surgical excision, combined with the intraoperative use of MMC and grafts to restore the scleral, conjunctival, and corneal surfaces, could avoid KS recurrence.