Spotlight on ocular Kaposi’s sarcoma: an update on the presentation, diagnosis, and management options

ABSTRACT

Introduction

Kaposi’s sarcoma (KS) is a multifocal low-grade vascular neoplasm that can affect the skin, mucus membranes, visceral organs, and lymph nodes. KS can also affect the ocular surface and adnexa and can masquerade as other entities, delaying prompt diagnosis.

Areas covered

In this review, the manifestations of ocular KS are discussed along with theories for pathogenesis, common risk factors, and management options.

Expert opinion

KS is caused by the oncogenic human herpesvirus 8 (HHV8). Immunosuppression in patients with HIV and AIDS contributes to the development of KS but conjunctival and ocular adnexal KS lesions are now uncommon in the era of anti-retroviral therapy. A high index of suspicion is required to diagnose ocular KS as these lesions can be mistaken for alternative entities. Prompt diagnosis can reduce significant morbidity and mortality by prompting a multidisciplinary systemic evaluation, particularly in immunosuppressed individuals. While surgical excision, cryotherapy, intralesional or systemic chemotherapy, and radiation are all viable treatment options, ongoing research to identify novel therapies and molecular treatment targets will help expand the armamentarium of therapeutics available for this disease.

KEYWORDS:

• Kaposi’s sarcoma
• conjunctival Kaposi’s sarcoma
• ocular adnexal Kaposi’s sarcoma
• opportunistic diseases
• human herpesvirus 8
• human immunodeficiency virus
• acquired immunodeficiency syndrome

Article highlights

• Kaposi’s sarcoma (KS) is a multifocal low-grade vascular neoplasm that can be found on the skin, mucocutaneous areas, as well as lymphatic and visceral organs.

• When affecting the eye, KS lesions can manifest on the eyelids, conjunctiva, and uncommonly extend onto the cornea and into the orbit.

• KS is caused by the oncogenic human herpesvirus 8 (HHV8). Immunosuppression in patients with HIV and AIDS can contribute to the development of KS but conjunctival and ocular adnexal KS lesions are now uncommon in the era of anti-retroviral therapy.

• KS can be found in both immunocompetent and immunosuppressed individuals with varying manifestations.

• Histopathology coupled with immunohistochemistry can confirm the diagnosis of KS after incisional or excisional biopsies.

• Treatment modalities for ocular KS depend on the location of the lesions, systemic involvement, and co-morbid infections. Common therapeutic options include immune reconstitution with anti-retroviral therapy in the setting of HIV, surgical excision, cryotherapy, intralesional injections of chemotherapeutic agents, radiation, or systemic chemotherapy.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

The authors have received funding from the following sources for this paper: NIH Center Core Grant P30EY014801, RPB Unrestricted Award and Career Development Awards, Dr. Ronald and Alicia Lepke Grant, The Lee and Claire Hager Grant, The Robert Farr Family Grant, The Grant and Diana Stanton-Thornbrough, The Robert Baer Family Grant, The Roberto and Antonia Menendez Grant, The Emilyn Page and Mark Feldberg Grant, The Calvin and Flavia Oak Support Fund, The Robert Farr Family Grant, The Jose Ferreira de Melo Grant, The Richard and Kathy Lesser Grant, The Michele and Ted Kaplan Grant and the Richard Azar Family Grant (institutional grants). Department of Veterans Affairs, Veterans Administration, Office of Research and Development, Clinical Sciences R&D (CSRD) I01 CX002015 (Dr. Galor) and Biomedical Laboratory R&D (BLRD) Service I01 BX004893 (Dr. Galor), Department of Defense Gulf War Illness Research Program (GWIRP) W81XWH-20-1-0579 (Dr. Galor) and Vision Research Program (VRP) W81XWH-20-1-0820 (Dr. Galor), National Eye Institute R01EY026174 (Dr. Galor) and R61EY032468 (Dr. Galor).