Kaposi's Sarcoma

Tania J. Phillips, MD, FRCPC

Disclosures

Wounds. 2001;13(6) 

In This Article

Diagnosis and Discussion

Our patient has classic type Kaposi's sarcoma. The four types of Kaposi's sarcoma are classic, African-endemic, iatrogenic/immunosuppressive/drug associated, and AIDS-associated/epidemic and are compared in Table 1. The four types are likely different manifestations of the same pathological process. The etiology remains to be elucidated, but there is strong evidence that Kaposi's sarcoma is the result of infection with Kaposi's sarcoma-associated herpes virus (KSHV) or human herpes virus 8 since DNA fragments can be identified in tissue samples of greater than 95 percent of all variants of Kaposi's sarcoma lesions.[1,9] We will focus the discussion in this article to the classic type Kaposi's sarcoma.

Classic Kaposi's sarcoma primarily affects elderly men of Eastern European, Jewish, or Mediterranean descent. Most lesions of classic Kaposi's begin after the sixth decade.[5] Median age at diagnosis is 64 years (range is 26 years to 90 years old).[2] Men are affected up to 15 times more commonly than women.[2] Homosexual habits increase the risk for classic Kaposi's sarcoma, even if patients are not immunosuppressed.[2]

The typical histology of Kaposi's sarcoma include spindle-shaped cells with vascular channels lined by abnormal endothelial cells.[13] Extravasated erythrocytes, hemosiderin, and fibrosis can often be seen.[2]

Early lesions of classic Kaposi's sarcoma appear as firm, violaceous, bluish black, or reddish-brown plaques on the feet that may become nodular and coalesce. Macular lesions are often palpable and hard in consistency. They often begin as oval shaped and are arranged parallel to skin tension lines. Lesions may appear in sites of trauma, such as the acral regions.[13] The lesions often coalesce, and tumor formation, erosion, crusting, hyperkeratosis, and ulceration can occur. Lymphedema may occur secondary to deeper involvement of lymph nodes and lymphatics.[13] It may progress to the genitals and, in some cases, the face.[13] Areas of tense edema may have a peau d' orange appearance. Persistent lymphatic disease may result in fibrosis, contracture, and loss of use of a limb.[13] Periods of remission may occur, in which case the lesion involutes, leaving an atrophic hypopigmented scar. Patients who experience significant edema, ulcerated lesions on the lower extremities, or lesions that are nodular may complain of moderate to severe pain.

Lesions classically occur on the lower extremities or hands and slowly spread centripetally.[13] Rarely, the tip of the nose, ears, scalp, periorbital areas, and genital areas may become involved. The trunk is rarely affected.[13]

Visceral and/or mucosal involvement occurs in 10 percent of patients.[2] Mucosal lesions often occur on the hard palate and initially appear as a violaceous stain. Soft palate, pharynx, uvula, gingival, and tongue may also be involved.[13] The small intestine, liver, and spleen may become involved, but are usually asymptomatic, particularly in classic Kaposi's sarcoma.[13] Lungs, heart, liver, conjunctiva, adrenal glands, and lymph nodes of the abdomen may also be involved. Characteristic and diagnostic skeletal changes include cysts and cortical erosion.[12] Urethral or anal canal involvement may result in obstruction. If there is pulmonary involvement, patients may complain of bronchospasm, progressive coughing, or shortness of breath.[13]

The disease course tends to be chronic, and patients usually survive for 10 to 15 years after diagnosis. Over 35 percent of patients with the disease develop secondary malignancies, such as leukemia, myeloma, and lymphomas.[7,8,13]

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