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Kaposi's sarcoma

Etiology: 1) may be a manifestation of Herpes virus type-8 2) Herpes virus type-8 found in lesions & peripheral blood mononuclear cells Epidemiology: 1) generally occurs in HIV-infected patients 2) occurs less frequently in HIV negative individuals 3) most common malignancy associated with HIV - most likely cause of violaceous plaques on skin of persons with HIV1 infection, even if well-controlled [9] 4) uncommon among women, men who acquired HIV through heterosexual contact or blood transfusions, & children Pathology: 1) proliferative tumor of vascular origin 2) spindle-shaped cells 3) proliferation of endothelial cells 4) inflammatory infiltrates 5) edema 6) multicentric tumor: involves skin, mucosal membranes, lymph nodes & visceral organs including the lungs & gastrointestinal tract Genetics: - at least 1 polymorphism in the IL-6 gene renders HIV-infected men susceptible to Kaposi sarcoma Clinical manifestations: 1) may appear as a vascular non-blanching papule a) pigmented lesions b) red-purple to dark brown-black c) 3-4 cm purple patches on skin of back (case presentation) 2) lesions may be few & small or large & disfiguring 3) lesions of the eyelids are associated with periorbital edema 4) leg edema may occur secondary to lymph node disease 5) oral lesions may occur [5] (image) * images [3,4,6] Laboratory: - biopsy with immunohistochemistry - CD34 & human herpesvirus 8 positive [5] (case report) - CD4 count low - see ARUP consult [2] Special laboratory: - bronchoscopy for pulmonary involvement - endobronchial violaceous macules or papules in the proximal airways - confluent hyperemic patches in the distal airways Differential diagnosis: - pneumocystis pneumonia (pulmonary) - endobronchial lesions are rare Management: 1) antiretroviral therapy 2) observation if asymptomatic 3) interventional therapy a) cryotherapy b) surgical excision c) intralesional chemotherapy d) intralesional interferon alpha-2A e) radiation therapy for moderate to severe disease f) systemic therapy for moderate to severe mucocutaneous or visceral disease - interferon alpha-2A - single or multiagent cytotoxic chemotherapy

Interactions

disease interactions

Related

human herpesvirus type 8; Kaposi's sarcoma-associated herpesvirus (HHV8, KSVK) interferon [IFN]-alfa 2a (Roferon-A)

General

soft tissue sarcoma (STS) vascular tissue neoplasm; angioma

Database Correlations

OMIM 148000

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
  2. ARUP Consult: Human Herpesvirus 8 - Kaposi Sarcoma The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/kaposi-sarcoma
  3. DermNet NZ. Kaposi sarcoma (images) http://www.dermnetnz.org/lesions/kaposi-sarcoma.html
  4. Schwartz RA, Elston EM (images) Medscape: Dermatologic Manifestations of Kaposi Sarcoma http://emedicine.medscape.com/article/1083998-overview
  5. Kennedy-LeJeune E, Cataldo VD (image) Kaposi's Sarcoma of the Oral Cavity. Engl J Med 2017; 376:1268. March 30, 2017 PMID: 28355516 http://www.nejm.org/doi/full/10.1056/NEJMicm1611553
  6. Brady MP (images) Cutaneous and Mucosal Manifestations of Viral Diseases. Medscape. March 2017 http://reference.medscape.com/features/slideshow/viral-skin
  7. De Paoli P, Carbone A. Kaposi's Sarcoma Herpesvirus: twenty years after its discovery. Eur Rev Med Pharmacol Sci. 2016 Apr;20(7):1288-94. Review. PMID: 27097948 Free Article
  8. Rothaus C A Man with Dyspnea, Odynophagia, and Abdominal Pain. NEJM Resident 360. Nov 20, 2019 https://resident360.nejm.org/clinical-pearls/a-man-with-dyspnea-odynophagia-and-abdominal-pain-2
  9. NEJM Knowledge+ Dermatology
  10. Kaposi's Sarcoma (PDQ): Treatment http://www.nci.nih.gov/cancertopics/pdq/treatment/kaposis/HealthProfessional