eMedicine Specialties > Ophthalmology > Dermatologic Disorders

Kaposi Sarcoma

Author: Jacqueline Freudenthal, MD, Co-Investigator, Ophthalmic Consultants Centre, Toronto
Coauthor(s): Kevin Ryan Yuhan, MD, Attending Physician, Cornea, Cataract, Refractive and External Diseases, Southern California Permanente Medical Group; Timothy T You, MD, Consulting Surgeon in Ophthalmology, Private Practice
Contributor Information and Disclosures

Updated: Feb 22, 2010

Introduction

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.

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

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.

Clinical

History

• Identify risk factors for Kaposi sarcoma. The clinician should ask about the following:
  • Demographics
  • Immune status
  • Previous skin lesions
  • Previous treatment for Kaposi sarcoma
  • History of opportunistic infections
  • Current medication use
• Symptoms of Kaposi sarcoma include the following:
  • Pain
  • Photophobia
  • Recurrent red or bloody eyes
  • Irritation and foreign body sensation
  • Epiphora
  • Dry eyes
  • Mucopurulent discharge
  • Heavy or swollen eyelids
  • Cosmetic disfigurement of the eyelids
  • Eyelashes rubbing against the eyes
  • Inability to close the eyes
  • Visual obstruction
  • Blurred vision

Physical

• Full ocular examination should include the following:
  • Inspect and evert the eyelids and lashes.
  • Perform slit lamp biomicroscopy.
  • Examine palpebral and bulbar conjunctivae and fornices in detail.
  • Palpate the lacrimal gland, and examine for masses.
  • Examine both eyes for proptosis in the rare likelihood of orbital involvement. Fortunately, intraocular Kaposi sarcoma has never been reported.
• The lesions are purplish red to bright red and highly vascular with surrounding telangiectatic vessels. They may be macular, plaquelike, or nodular.
• Dugel et al described 3 clinical stages that may help direct therapy.
• Stage I and II tumors are patchy and flat. These lesions have a thickness of less than 3 mm in vertical height and are younger than 4 months.
• Stage III tumors are nodular and elevated with a vertical height of greater than 3 mm. They tend to be older than 4 months.
• Ophthalmic Kaposi sarcoma lesions are found on the eyelids, conjunctiva, caruncle, and lacrimal sac. They rarely are found inside the orbit. An example of eyelid involvement is shown in the example below.
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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.

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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.

• Of ophthalmic Kaposi sarcoma cases, 6-16% are eyelid lesions, and the superior and inferior eyelids tend to be involved equally.
• Of ophthalmic Kaposi sarcoma cases, 7-18% are conjunctival lesions. Many conjunctival lesions tend to involve the inferior conjunctiva (as is shown in the image below) and fornix.
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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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  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.

• Lesions tend to be indolent, but, as the tumor grows, it can alter ocular adnexal structures and the ocular surface. The mass effect of the tumor on the eyelids can cause mechanical ectropion or entropion with trichiasis and lagophthalmos and irregular astigmatism.
  • Ectropion or entropion can result in poor lid apposition, trichiasis, and lagophthalmos. Consequently, the patient may experience epiphora, poor tear clearance and drainage, recurrent corneal abrasions, pain and discomfort, foreign body sensation, dry eyes, and photophobia.
  • Long-standing trichiasis and exposure can result in corneal infection, scarring, and opacification.
• Rarely, tumor bulk may block the visual axis by ptosis or direct obstruction. Tumor bulk may even prevent the complete closure of the eyelid.
• Conjunctival involvement may present with subconjunctival hemorrhage, injection, and chemosis.

Causes

• Human herpesvirus-8 (HHV-8) DNA or Kaposi sarcoma–associated herpesvirus (KSHV) has been implicated with patients who are HIV-negative and HIV-positive.²
• Homosexual males with HIV are at an increased risk. This risk is markedly increased with the number of partners.
• Patients who have had organ transplants and use immunosuppressive agents and steroids are at an increased risk.
• Elderly males of Mediterranean or Ashkenazi ancestry are at an increased risk.

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