Kaposi Sarcoma Clinical Presentation

Updated: Apr 11, 2019
  • Author: Jessica Katz, MD, PhD, FACP; Chief Editor: Edwin Choy, MD, PhD  more...
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Presentation

History

AIDS-related Kaposi sarcoma (KS) has a variable clinical course ranging from minimal mucocutaneous disease to widespread organ involvement. The lesions may involve the skin, oral mucosa, lymph nodes, and visceral organs. Most patients present with cutaneous disease. Visceral disease may occasionally precede cutaneous manifestations. Lesions involving virtually every organ have been reported in autopsy series. The brain is spared, however.

Cutaneous lesions occur in virtually all patients. Note the following:

  • Multiple skin lesions - See Physical for description.

  • Usually multicentric in a continuum of development

  • Tumor-associated lymphedema - Typically manifested by lower extremity or facial involvement, thought to occur secondary to obstruction of lymphatic channels

  • Pain associated with ambulation - Due to lesions involving the soles of the feet

Gastrointestinal lesions can occur anywhere within the gastrointestinal tract. Lesions are often asymptomatic and clinically indolent. Gastrointestinal disease is usually an indicator of more advanced HIV infection. Symptoms include the following:

  • Odynophagia, dysphagia
  • Nausea, vomiting, abdominal pain
  • Hematemesis, hematochezia, melena
  • Bowel obstruction

Pulmonary involvement may be difficult to distinguish from opportunistic infections. Symptoms include the following:

  • Cough
  • Dyspnea
  • Hemoptysis
  • Chest pain
  • Pulmonary lesions may be an asymptomatic radiographic finding.
  • Pleural effusions are often exudative and bloody.

Lymphadenopathy may be the only site of disease requiring a lymph node biopsy. It may lead to significant lymphedema.

Classic Kaposi sarcoma has a more indolent course of 10-15 years or more with very gradual enlargement of cutaneous lesions and development of new ones. These lesions result in venous stasis and lymphedema of the lower extremities. Visceral lesions occur in the GI tract, lymph nodes, and other organs but are usually incidental findings at autopsy. As many as one third of patients develop a second neoplasm, most often a non-Hodgkin lymphoma.

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Physical

See the list below:

  • Cutaneous lesions may occur at any location but typically are concentrated on the lower extremities and the head and neck region.

  • Lesions may have macular, papular, nodular, or plaquelike appearances.

  • Nearly all lesions are palpable and nonpruritic.

  • Lesions may range in size from several millimeters to several centimeters in diameter.

  • Lesions may assume a brown, pink, red, or violaceous color and may be difficult to distinguish in dark-skinned individuals.

  • Lesions may be discrete or confluent and typically appear in a linear, symmetric distribution, following Langer lines.

  • Mucous membrane involvement is common (palate, gingiva, conjunctiva). Ulcerated or bulky tumor involvement may interfere with speech or mastication.

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Causes

Etiologic factors include the following:

  • Coinfection with HIV and HHV-8 (Kaposi sarcoma-associated herpesvirus [KSHV])

  • Iatrogenic immunosuppression (including corticosteroids)

  • Elevated degree of expression of numerous cytokines and angiogenic growth factors, including tumor necrosis factor alpha, interleukin-6, basic fibroblast growth factor, HIV-tat protein, and oncostatin M

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