Infectious Diseases

Kaposi Sarcoma (KS)(Clinical Condition)

OVERVIEW: What every practitioner needs to know

Are you sure your patient has Kaposi sarcoma? What should you expect to find?

Patients with human immunodeficiency virus (HIV)-related Kaposi sarcoma (KS) can have a wide variety of clinical presentations, ranging from localized to diffuse cutaneous disease, mucocutaneous lesions, lymphadenopathy, and/or visceral involvement. The patient may have one or more of the following findings on physical examination:

  • Skin: localized or diffuse involvement with hyperpigmented to violaceous macules, papules (Figure 1, Figure 2), patches (Figure 3, Figure 4, Figure 5), and/or plaques. In advanced disease, the patients may also develop friable tumors on the skin (Figure 6, Figure 7).

  • Oral mucosa: violaceous patches, papules, or plaques on the palate, buccal mucosa, and/or gingiva; advanced lesions may be friable or ulcerated, and tumors can also develop on mucosal surfaces.

  • Other mucosal involvement: lesions can develop on any mucosal site, including the oral, ocular, nasal, and genital mucosa.

  • Lymph node involvement: patients may develop localized or diffuse lymphadenopathy, with the inguinal and cervical lymph nodes (Figure 8) being the most commonly involved. Associated lymphedema is often seen in patients with lymph node disease (Figure 9, Figure 10).

  • Internal involvement: in advanced disease, patients may develop pulmonary and/or gastrointestinal (GI) involvement, and associated symptoms may include nausea, vomiting, GI bleeding, perforation, ileus, cough, bronchospasm, and/or dyspnea.

Figure 1.

Involvement of the tip of the nose my Kaposi sarcoma.

Figure 2.

Diffuse violaceous papules or Kaposi sarcoma.

Figure 3.

Patch-stage Kaposi sarcoma, manifesting as hyperpigmented patches on the thigh.

Figure 4.

Violaceous and hyperpigmented patches of Kaposi sarcoma.

Figure 5.

Close up view of a violaceous papule of Kaposi sarcoma.

Figure 6.

Nodular cutaneous Kaposi sarcoma (KS) presenting on the thigh of a patient with underlying associated lymphedema and plaques of KS.

Figure 7.

Tumoral lesions of Kaposi sarcoma.

Figure 8.

Cervical lymph node involvement without skin disease in a child.

Figure 9.

Advanced Kaposi sarcoma with edema of the legs and genitals.

Figure 10.

Skin with diffuse involvement with violaceous papules and plaques, as well as lymphedema secondary to inguinal lymph node involvement.

How did the patient develop Kaposi sarcoma? What was the primary source from which the infection spread?

KS-associated herpesvirus, or human herpesvirus 8 (HHV-8) is the primary underlying factor, in addition to immunosuppression secondary to HIV infection, for the development of HIV-associated KS. The acquisition of HHV-8 varies from sexual transmission (predominantly in the developed world) to early childhood or mother-to-child transmission (more commonly in the developing world). The prevalence rate of HHV-8 varies throughout the world, with infection being very common in sub-Saharan Africa (prevalence rates >50%), to moderately common in Mediterranean countries (prevalence rates 20–30%), to uncommon in Europe, Asia, and the United States (prevalence rates <10%). In the United States, prevalence rates are highest among men who have sex with men (MSM).

In the United States and Europe, KS is most commonly seen in MSM. In this population, a low cluster of differentiation (CD)4 count has been linked to the developed of KS, and acquisition of the HHV-8 virus increases with the number of lifetime sexual partners. In Africa, because HHV-8 is acquired more commonly in early childhood, the development of HIV-related KS may occur in children and young adults as their CD4 count declines. Patients in Africa are also at risk for endemic KS, which is related to HHV-8 infection but is independent of HIV status.

Which individuals are of greater risk of developing Kaposi sarcoma?

Patients with clinical acquired immune deficiency syndrome (AIDS) are at greater risk of developing HIV-related KS. Once patients develop disease, HIV-related KS can have a variable course, but patients may worsen with a decline of the CD4 count. In addition, patients may develop a flare of KS due to immune reconstitution syndrome following initiation of highly active antiretroviral therapy (HAART).

In a study by Shiels and colleagues, the authors report that between 1980 and 2007 in the United States, an estimated 81.6% (95% confidence interval [CI], 81.2–81.9%) of the 83,252 KS cases occurring among persons with HIV. The proportion of KS in persons with AIDS peaked in the early 1990s (1990–1995: KS, 90.5% [95% CI, 90.2–90.8%]).

Beware: there are other diseases that can mimic Kaposi sarcoma:

There are several entities that can mimic cutaneous KS clinically and may require a biopsy for differentiation. These include:

  • Lichenoid tissue reactions: lichen planus and lichenoid-type drug eruptions can present with hyperpigmented to violaceous patches, papules, or plaques on the skin. Lichenoid drug eruptions can occur as a result of HAART or antibiotics, which are commonly taken by patients at risk for KS.

  • Acroangiodermatitis: this presents as violaceous patches and plaques, typically on the lower extremities of patients with venous hypertension.

  • Angiosarcoma: this presents most commonly on the head and neck region of elderly persons as an erythematous or blue-black bruise-like patch or plaque, which may ulcerate or bleed. Patients with chronic lymphedema or a history of radiation therapy are also at risk.

  • Other entities in differential diagnosis include: pyogenic granuloma, bacillary angiomatosis, hemangiomas, ecchymosis, or pseudolymphoma/lymphoma.

What laboratory studies should you order and what should you expect to find?

Results consistent with the diagnosis

Although clinical examination alone by an experienced clinician may be sufficient for diagnosis, there are numerous entities that can mimic KS in the skin and a biopsy is needed for confirmation. Given the implications of the diagnosis, biopsy is recommended.

Histologic analysis of clinical lesions (i.e., skin, lymph nodes, or visceral organs) can allow for confirmation of the diagnosis. Histology shows a proliferation of slit-like vascular spaces lined by atypical endothelial cells and surrounded by a spindle cell proliferation. The slit-like vascular spaces may dissect among collagen bundles, and a plasmacytic infiltrate may be present. Immunohistochemical staining with antibodies against a latent nuclear antigen (LNA-1) can highlight the presence of HHV-8 within lesional nuclei.

Results that confirm the diagnosis

Histologic analysis of clinical lesions (i.e., skin, lymph nodes, or visceral organs) can allow for confirmation of the diagnosis. Histology shows a proliferation of slit-like vascular spaces lined by atypical endothelial cells and surrounded by a spindle cell proliferation. The slit-like vascular spaces may dissect among collagen bundles, and a plasmacytic infiltrate may be present. Immunohistochemical staining with antibodies against a latent nuclear antigen (LNA-1) can highlight the presence of HHV-8 within lesional nuclei.

What imaging studies will be helpful in making or excluding the diagnosis of Kaposi sarcoma?

Imaging studies are not particularly useful in making or confirming the diagnosis of KS; however, imaging, such as a chest X-ray or computed tomography (CT) scan, may be helpful in staging and estimating the extent of disease once the diagnosis is made on histology.

What consult service or services would be helpful for making the diagnosis and assisting with treatment?

If you decide the patient has Kaposi sarcoma, what therapies should you initiate immediately?

Dermatology may be the most useful service for diagnosing cutaneous KS, since they can assist in clinico-pathologic correlation of skin disease using physical examination and biopsies. If the patient only has oral manifestations, oral medicine may be helpful in performing a confirmatory biopsy. For potential lymph node or visceral disease, the general surgery or gastrointestinal service may assist in making the diagnosis by performing biopsies on the involved tissue.

  • Curative therapy is not available, and the goal of treatment is to control symptoms and limit/slow progression.

  • Limited, small, or slow growing lesions may only be clinically monitored, unless they are in a cosmetically sensitive area, painful, bleeding, or otherwise symptomatic.

  • If patients develop HIV-associated KS and they are not on HAART, HAART should be initiated.

  • Limited disease can be treated with conservative therapy, such as cryotherapy, radiation therapy, topical immunotherapy with imiquimod, topical retinoids, or intralesional chemotherapy.

  • Systemic chemotherapy:

    • Diffuse or disseminated disease is best treated with systemic chemotherapy.

    • Side effects for pegylated liposomal doxorubicin most commonly include hematologic toxicity, including leukopenia, lymphopenia, and neutropenia. Significant neutropenia, alopecia, and sensory neuropathy are more commonly seen with paclitaxel than pegylated liposomal doxorubicin.

    • Pegylated liposomal doxorubicin and paclitaxel are both active agents for patients with advanced, systemic KS in the HAART era, with response rates ranging from 50 to 60%.

Anti-infective agents (Table I)

Table I.

Treatment Options for Kaposi Sarcoma
Type of therapy Medication Instructions for use Evidence level
Topical Cryotherapy Treatment is given on average every 3 weeks to allow for healing. Treatment of each lesion consists of two freeze thaw cycles. B
Topical Alitretinoin gel Apply topically to lesions twice daily. A
Topical Imiquimod Apply to lesions under occlusion twice daily for 24 weeks. C
Localized Radiotherapy Dose and duration are to be determined by radiation oncologist. B
Localized Intralesional vinblastine Single intralesional injection C
Localized Intralesional interferon 1 million units intralesionally three times weekly for 6 weeks C
Systemic Pegylated liposomal doxorubicin Administered 20mg/m2 intravenously every 2–3 weeks B
Systemic Paclitaxel Administered 100mg/m2 intravenously every 2–3 weeks B

What complications could arise as a consequence of Kaposi sarcoma?

What should you tell the family about the patient's prognosis?

Complications include:

  • ulceration of skin lesions

  • bleeding of the skin, mucous membranes, GI tract

  • hemoptysis

  • anemia

  • lymphedema

  • shortness of breath

  • respiratory failure

Risk factors for poor outcome include:

  • visceral involvement

  • diffuse mucocutaneous disease

  • progressive disease (especially progression despite therapy)

  • development of complications (such as internal bleeding or respiratory failure)

  • poor immune status

Prognosis depends on extent of disease, development of complications, and response to treatment.

A significant decrease in the incidence of KS, particularly visceral disease, has been observed in the era of HAART.

Recent studies show a trend toward better clinical outcome in treatment-naive KS patients who are treated with HAART plus chemotherapy compared with HAART alone.

There is no significant evidence that HHV-8 antibody titers in HIV-related KS patients predicts prognosis.

How do you contract Kaposi sarcoma and how frequent is this disease?

KS-associated herpesvirus, or HHV-8, is the primary underlying factor, in addition to immunosuppression secondary to HIV infection, for the development of HIV-associated KS. The acquisition of HHV-8 varies from sexual transmission (predominantly in the developed world) to early childhood or mother-to-child transmission (more commonly in the developing world).

What pathogens are responsible for this disease?

KS-associated herpesvirus, or HHV-8, is the primary pathogen responsible for disease.

How do these pathogens cause Kaposi sarcoma?

Patients infected with HHV-8 and immunosuppressed from HIV are at risk for disease. KS is a vascular endothelial malignancy caused by HHV-8, but there is no agreement on the exact mechanism by which HHV-8 leads to the development of KS. Immunosuppression by the HIV virus, in particular, seems to allow for replication and progression of the HHV-8 virus.

How can Kaposi sarcoma be prevented?

HIV-related KS can be best prevented by not acquiring HIV infection or early initiation of HAART in patients with HIV infection with tight viremic control and maintenance of CD4 count. Because of the wide variety of potential methods of transmission of HHV-8 infection, acquisition is difficult to control.

WHAT'S THE EVIDENCE for specific management and treatment recommendations?

Bihl, F, Mosam, A, Henry, LN. "Kaposi's sarcoma-associated herpesvirus-specific immune reconstitution and antiviral effect of combined HAART/chemotherapy in HIV clade C-infected individuals with Kaposi's sarcoma". AIDS. vol. 21. 2007. pp. 1245-52.

(This study shows a trend toward better clinical outcome of treatment-naive KS patients treated with HAART plus chemotherapy treatment compared with HAART alone.)

Cianfrocca, M, Lee, S, Von Roenn, J. "Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy". Cancer. vol. 116. 2010. pp. 3969-77.

(Treatment with either paclitaxel or pegylated liposomal doxorubicin appears to produce significant improvements in pain and swelling in patients with advanced, symptomatic, HIV-associated KS treated in the HAART era. Side effects for pegylated liposomal doxorubicin most commonly include hematologic toxicity, including leukopenia, lymphopenia, and neutropenia. Significant neutropenia, alopecia, and sensory neuropathy are more commonly seen with paclitaxel than pegylated liposomal doxorubicin. Pegylated liposomal doxorubicin and paclitaxel are both active agents for patients with advanced, systemic KS in the HAART era, with response rates ranging from 50 to 60%.)

Cobb, CW, Manders, SM., Lebwohl, MG, Heymann, WR, Berth-Jones, J, Coulson, I. "Kaposi sarcoma". Treatment of skin disease: comprehensive therapeutic strategies. Saunders-Elsevier. 2010. pp. 340-42.

(This book evaluates the evidence for treatments for many different diseases of the skin. It grades them as A: double-blind studies, B: clinical trial with greater than or equal 20 subjects, C: clinical trial with less than 20 subjects, D: case series of more than four subjects, and E: anecdotal case reports.)

Kovarik, C., Williams, H, Bigby, M, Diepgen, T, Herxheimer, A, Naldi, L, Rzany, B. "Kaposi's sarcoma". Evidence-based dermatology. BMJ Books. 2010. pp. 316-26.

(This chapter reviews the clinical trial evidence for the treatment of Kaposi sarcoma.)

Madkan, V, Sra, K, Brantley, J, Carrasco, D, Mendoza, N, Tyring, S., Bolognia, JL, Jorizzo, JL, Rapini, RP. "Human herpesviruses". Dermatology. Mosby-Elsevier. 2008. pp. 1199-217.

(This is an outstanding review of Kaposi sarcoma, including its relationship to patients immunocompromised by HIV infection.)

Silverberg, MJ, Lau, B, Achenbach, CJ, Jing, Y. "Cumulative Incidence of Cancer Among Persons With HIV in North America: A Cohort Study". Ann Intern Med.. vol. 163. 2015 Oct 6. pp. 507-18.

(The most comprehensive study on this controversial subject so far. Cumulative incidences of Kaposi's sarcoma by age 75 years for persons with and without HIV, respectively, 4.4% and 0.01%.)

Djawe, K, Buchacz, K, Hsu, L, Chen, MJ. "Mortality Risk After AIDS-Defining Opportunistic Illness Among HIV-Infected Persons--San Francisco, 1981-2012". J Infect Dis.. vol. 212. 2015 Nov 1. pp. 1366-75.

(5-year survival rates, stratified according to diagnosis and year, demonstrating the spectacular effectiveness of HAART.)

DRG CODES and expected length of stay

ICD-9 codes:

(176) Kaposi sarcoma

  • (176.0) Kaposi sarcoma skin

  • (176.1) Kaposi sarcoma soft tissue

  • (176.2) Kaposi sarcoma palate

  • (176.3) Kaposi sarcoma gastrointestinal sites

  • (176.4) Kaposi sarcoma

  • (176.5) Kaposi sarcoma lymph nodes

  • (176.8) Kaposi sarcoma other specified sites

  • (176.9) Kaposi sarcoma unspecified site

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