Kaposi Sarcoma Treatment (PDQ®)–Health Professional Version

General Information About Kaposi Sarcoma

Epidemiology

Kaposi sarcoma (KS) was first described in 1872 by the Hungarian dermatologist, Moritz Kaposi. From that time until the current HIV disease epidemic identified with AIDS, KS remained a rare tumor. While most of the cases seen in Europe and North America have occurred in elderly men of Italian or Eastern European Jewish ancestry, the neoplasm may also occur in patients receiving immunosuppressive therapy.[1] The disseminated, fulminant form of KS associated with HIV disease is referred to as epidemic KS to distinguish it from the classic and transplant-related varieties of the neoplasm.[2]

Histopathology

Although the histopathology of the different types of the Kaposi tumor is essentially identical in all of these groups, the clinical manifestations and course of the disease differ dramatically.[2] Human herpes virus type 8 , also known as Kaposi sarcoma herpes virus, was identified in KS tissue biopsies from virtually all patients with classic, transplant-related, and AIDS-associated KS but was absent from noninvolved tissue.[2]

Classic Kaposi Sarcoma

Considered a rare disease, classic KS occurs more often in males, with a ratio of approximately 10 to 15 males to 1 female. In North Americans and Europeans, the usual age at onset is between 50 and 70 years. Classic KS tumors usually present with one or more asymptomatic red, purple, or brown patches, plaques, or nodular skin lesions. The disease is often limited to single or multiple lesions usually localized to one or both lower extremities, especially involving the ankles and soles.

Classic KS most commonly runs a relatively benign, indolent course for 10 to 15 years or more, with slow enlargement of the original tumors and the gradual development of additional lesions. Venous stasis and lymphedema of the involved lower extremity are frequent complications. In long-standing cases, systemic lesions can develop along the gastrointestinal tract, in lymph nodes, and in other organs. The visceral lesions are generally asymptomatic and are most often discovered only at autopsy, though clinically, gastrointestinal bleeding can occur. As many as 33% of the patients with classic KS develop a second primary malignancy, which is most often non-Hodgkin lymphoma.[3]

Epidemic Kaposi Sarcoma (HIV-Associated Kaposi Sarcoma)

The introduction of highly active antiretroviral therapy (HAART) has delayed or prevented the emergence of drug-resistant HIV strains, profoundly decreased viral load, led to increased survival, and lessened the risk of opportunistic infections.[4] The use of HAART has been associated with a sustained and substantial decline in KS incidence in multiple large cohorts.[5-7] KS can still appear during HAART with complete suppression of HIV; most cases in the United States occur in patients with high CD4 counts and ongoing HAART.[8]

The lesions that develop may involve the skin; oral mucosa; lymph nodes; and visceral organs, such as the gastrointestinal tract, lung, liver, and spleen. Most patients with HIV disease who present with the mucocutaneous lesions of KS feel healthy and are usually free of systemic symptoms, as compared with HIV patients who first develop an opportunistic infection. The sites of disease at presentation of epidemic KS are much more varied than the sites seen in other types of this neoplasm. While most patients present with skin disease, KS involvement of lymph nodes or the gastrointestinal tract may occasionally precede the appearance of the cutaneous lesions. The disease often progresses in an orderly fashion from a few localized or widespread mucocutaneous lesions to more numerous lesions and generalized skin disease with lymph node, gastrointestinal tract, and other organ involvement. Pleuropulmonary KS is an ominous sign usually occurring late in the course of the disease, especially in patients whose death is directly attributed to KS.

References
  1. Ruocco E, Ruocco V, Tornesello ML, et al.: Kaposi’s sarcoma: etiology and pathogenesis, inducing factors, causal associations, and treatments: facts and controversies. Clin Dermatol 31 (4): 413-422, 2013 Jul-Aug. [PUBMED Abstract]
  2. Uldrick TS, Whitby D: Update on KSHV epidemiology, Kaposi Sarcoma pathogenesis, and treatment of Kaposi Sarcoma. Cancer Lett 305 (2): 150-62, 2011. [PUBMED Abstract]
  3. Safai B, Good RA: Kaposi’s sarcoma: a review and recent developments. Clin Bull 10 (2): 62-9, 1980. [PUBMED Abstract]
  4. Lodi S, Guiguet M, Costagliola D, et al.: Kaposi sarcoma incidence and survival among HIV-infected homosexual men after HIV seroconversion. J Natl Cancer Inst 102 (11): 784-92, 2010. [PUBMED Abstract]
  5. Portsmouth S, Stebbing J, Gill J, et al.: A comparison of regimens based on non-nucleoside reverse transcriptase inhibitors or protease inhibitors in preventing Kaposi’s sarcoma. AIDS 17 (11): F17-22, 2003. [PUBMED Abstract]
  6. Carrieri MP, Pradier C, Piselli P, et al.: Reduced incidence of Kaposi’s sarcoma and of systemic non-hodgkin’s lymphoma in HIV-infected individuals treated with highly active antiretroviral therapy. Int J Cancer 103 (1): 142-4, 2003. [PUBMED Abstract]
  7. Grabar S, Abraham B, Mahamat A, et al.: Differential impact of combination antiretroviral therapy in preventing Kaposi’s sarcoma with and without visceral involvement. J Clin Oncol 24 (21): 3408-14, 2006. [PUBMED Abstract]
  8. Yanik EL, Achenbach CJ, Gopal S, et al.: Changes in Clinical Context for Kaposi’s Sarcoma and Non-Hodgkin Lymphoma Among People With HIV Infection in the United States. J Clin Oncol 34 (27): 3276-83, 2016. [PUBMED Abstract]