Ophthalmologic Manifestations of Kaposi Sarcoma 

  • Author: Jacqueline Freudenthal, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Feb 22, 2010
 

Background

In 1872, Kaposi was the first to report an idiopathic, multiple-pigmented sarcoma of the skin that predominantly affected elderly men of Mediterranean or Ashkenazi ancestry. This classic type has an indolent course and commonly presents on the lower extremities.

Since the original description, 3 additional forms have been described. Endemic Kaposi sarcoma is prevalent in central Africa, primarily affecting young men with aggressive skin and visceral lesions. Transplant-related Kaposi sarcoma has been reported in patients with renal transplants on immunosuppressive therapy. Epidemic Kaposi sarcoma, better known as AIDS-related Kaposi sarcoma, commonly affects homosexual males with AIDS. Prior to the AIDS epidemic of 1981, fewer than 25 reported cases of ophthalmic Kaposi sarcoma existed in the literature.

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Pathophysiology

Whether the 4 variants of Kaposi sarcoma represent the same disease process or different processes that manifest the same end-stage lesion is unclear.

Although the etiology has not been elucidated, the human herpesvirus 8 (HHV-8) or Kaposi sarcoma-associated herpesvirus (KSHV) has been implicated. Chang et al identified this virus in more than 90% of patients with AIDS-related Kaposi sarcoma.[1]

Kaposi sarcoma is most likely caused by multiple factors, including deregulated expression of oncogenes and oncosuppressor genes by KSHV/HHV-8 combined with decreased immune surveillance and the release of cytokines (interleukin [IL]-6) and growth factors by HIV acting on infected cells. IL-6 induces signal transducers and activators of transcription 3 (STAT3) gene activation, thus driving oncogene expression. Although the exact mechanisms by which KSHV/HHV-8 mediates oncogenesis have not been fully elucidated, numerous KSHV/HHV-8 viral oncogenes that may contribute to neoplasia have been described.[2]

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Epidemiology

Frequency

United States

Prior to 1981, less than 25 reported cases of ophthalmic Kaposi sarcoma existed in the literature. Currently, the overwhelming majority of ophthalmic Kaposi sarcoma is AIDS related. In 1986, the Centers for Disease Control and Prevention (CDC) reported that Kaposi sarcoma occurs in approximately 24% of patients with AIDS and in 35% of all homosexual men with AIDS.[3] The CDC reported that during the period from 1992-1997, Kaposi sarcoma occurred in 23.8% of males with AIDS and in 27.4% of all homosexual men with AIDS.[4] During the same time period, only 2.3% of women with AIDS developed Kaposi sarcoma.

Ophthalmic involvement occurs in 20-24% of patients with AIDS-related Kaposi sarcoma. Ophthalmic presentation was the initial manifestation of AIDS-related Kaposi sarcoma in 4-12% of patients. Eye lesions are neither an early nor a late manifestation of Kaposi sarcoma. A review of the literature reports that most eye lesions involve the conjunctivae or eyelids; 10-75% of patients have conjunctival lesions, and 25-80% of patients have eyelid lesions.

In recent years, the incidence of Kaposi sarcoma among HIV patients has significantly declined, from 60.6% in 1992 to 19.7% in 1997. The exact cause for this decline is unclear, but the introduction of protease inhibitors, combination HIV therapy, and safer sexual practices may have played significant roles.

Mortality/Morbidity

Tumors have been linked to significant mortality and morbidity. Kaposi sarcoma can disseminate to visceral organs (ie, lungs, liver, adrenal glands, kidneys, bone marrow, GI tract). Bowel obstruction, lower extremity edema, shortness of breath, hemorrhage, and pain have been reported. Visceral and lung involvement usually portends a poor prognosis.

Generally, ophthalmic Kaposi sarcoma is indolent. Ocular tumor growth can result in severe damage to the ocular adnexa, the ocular surface, and even the orbit. One case report has also described a widely disseminated Kaposi sarcoma tumor that included the choroid in both eyes.[5]

Involvement of the eyelids can cause significant disfigurement and lid dysfunction. Trichiasis can develop from mechanical ectropion or entropion. Lagophthalmos and trichiasis can result in profound irritation and dryness, infections, and corneal scarring. Large lid tumors can induce irregular corneal astigmatism. Conjunctival involvement may result in recurrent subconjunctival hemorrhages. Ultimately, vision could be lost from lid dysfunction, corneal surface changes, or visual obstruction.

Race

Classic Kaposi sarcoma usually involves elderly men with Mediterranean or Ashkenazi ancestry. Endemic Kaposi sarcoma usually involves young black males from central Africa. Transplant-related or AIDS-related Kaposi sarcoma has no racial predilection.

Sex

Classic Kaposi sarcoma usually involves elderly men. Endemic Kaposi sarcoma usually involves young males. AIDS-related Kaposi sarcoma predominantly involves homosexual males.

Age

Classic Kaposi sarcoma affects the older population. Endemic Kaposi sarcoma affects children with a male-to-female ratio of 1:1; after puberty, males are predominantly affected. AIDS-related Kaposi sarcoma usually affects males aged 20-49 years.

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Contributor Information and Disclosures
Author

Jacqueline Freudenthal, MD  Co-Investigator, Ophthalmic Consultants Centre, Toronto

Jacqueline Freudenthal, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, and Canadian Ophthalmological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kevin Ryan Yuhan, MD  Attending Physician, Cornea, Cataract, Refractive and External Diseases, Southern California Permanente Medical Group

Kevin Ryan Yuhan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Timothy T You, MD  Consulting Surgeon in Ophthalmology, Private Practice

Timothy T You, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Anastasios J Kanellopoulos, MD  Assistant Program Director, Clinical Associate Professor, Department of Ophthalmology, Manhattan Eye, Ear, and Throat Hospital, New York University

Anastasios J Kanellopoulos, MD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, Eye Bank Association of America, and International Society of Refractive Surgery

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Institute

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

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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.
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.
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.
Kaposi sarcoma involvement of the eyelid. Courtesy of Gary N Holland, MD, University of California, Los Angeles, Department of Ophthalmology, Jules Stein Eye Institute.
 
 
 
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