II. Epidemiology

  1. Highest frequency in HIV homosexual or bisexual men
  2. Middle aged to elderly white males
  3. Young Black patients in Africa
  4. Extremely uncommon in women and children
  5. Incidence
    1. Was more common (48%) early in the HIV epidemic
    2. Now only seen 18% of HIV cases in homosexual or bisexual men

III. Pathophysiology

  1. Endothelial tumor
  2. Usually multicentric
  3. Involves the skin and visceral organs
  4. Very rarely involves the brain
  5. May be caused by an enteric sexually transmitted agent
    1. Associated with Human Herpes Virus 8

IV. Risks

  1. More common in those with oral or anal sexual contacts

V. Symptoms

  1. Lesions typically non-pruritic
  2. Initial: Painless
  3. Later: Lesions can become large and painful

VI. Signs: Cutaneous lesions

  1. Pigmented (violaceous)
    1. Red-blue/purple or bluish-brown Plaques and Nodules
    2. Flush with skin or raised strawberry-like Plaques
    3. Lesions enlarge, coalesce and may bleed
  2. Can involve any area of the skin
    1. Especially common on the soles of the feet
    2. Rarely affects the palms of the hands
  3. Biopsy differentiates from
    1. Bacillary Angiomatosis
    2. Cutaneous cryptococcus

VII. Signs: Intraoral disease

  1. Intraoral disease present at time of diagnosis: 50%
  2. Higher risk of gastrointestinal involvement

VIII. Signs: Gastrointestinal involvement (Usually asymptomatic)

  1. Dysphagia
  2. Gastric outlet obstruction
  3. Gallbladder

IX. Signs: Pulmonary involvement

  1. Rapidly fatal if not treated
  2. Usually symptomatic
    1. Non-productive cough
    2. Bronchospasm
    3. Dyspnea
  3. Pleural Effusion in 20-50% cases at diagnosis
    1. Pleural Fluid negative for Kaposi's Sarcoma
    2. Transudative or exudative
  4. Chest XRay
    1. Reticulonodular Pulmonary Infiltrates
    2. Much coarser than with Pneumocystis carinii
  5. Normal gallium scan
  6. Bronchoscopy
    1. Characteristic endobronchial lesions
    2. Biopsy usually avoided secondary to bleeding

X. Management: General

  1. No treatment is curative!
  2. Slow the progression of systemic disease
  3. Manage local or regional problems

XI. Management: Medications

  1. Chemotherapy
    1. Cytotoxic drugs (Indicated for widespread disease)
      1. Doxorubicin
      2. Vinblastine
      3. Vincristine
      4. Bleomycin
    2. Interferon alpha
      1. Better with Less extensive cutaneous lesions
      2. Better if no edema or visceral disease
  2. Radiation Therapy
    1. Control Lymphedema
    2. Control painful or necrotic bulky tumors
  3. Surgical Resection
    1. Indicated for local complications
  4. Cryotherapy or Liquid Nitrogen
    1. Indicated to ablate single lesions

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Related Studies

Ontology: Kaposi Sarcoma (C0036220)

Definition (CHV) a cancer that causes patches of abnormal tissue to grow under the skin,
Definition (MEDLINEPLUS)

Kaposi's sarcoma is a cancer that causes patches of abnormal tissue to grow under the skin, in the lining of the mouth, nose, and throat or in other organs. The patches are usually red or purple and are made of cancer cells and blood cells. The red and purple patches often cause no symptoms, though they may be painful. If the cancer spreads to the digestive tract or lungs, bleeding can result. Lung tumors can make breathing hard.

Before the HIV/AIDS epidemic, KS usually developed slowly. In HIV/AIDS patients, though, the disease moves quickly. Treatment depends on where the lesions are and how bad they are. Treatment for HIV itself can shrink the lesions. However, treating KS does not improve survival from HIV/AIDS itself.

NIH: National Cancer Institute

Definition (NCI) A malignant neoplasm characterized by a vascular proliferation which usually contains blunt endothelial cells. Erythrocyte extravasation and hemosiderin deposition are frequently present. The most frequent site of involvement is the skin; however it may also occur internally. It generally develops in people with compromised immune systems including those with acquired immune deficiency syndrome (AIDS).
Definition (NCI_NCI-GLOSS) A type of cancer characterized by the abnormal growth of blood vessels that develop into skin lesions or occur internally.
Definition (MSH) A multicentric, malignant neoplastic vascular proliferation characterized by the development of bluish-red cutaneous nodules, usually on the lower extremities, most often on the toes or feet, and slowly increasing in size and number and spreading to more proximal areas. The tumors have endothelium-lined channels and vascular spaces admixed with variably sized aggregates of spindle-shaped cells, and often remain confined to the skin and subcutaneous tissue, but widespread visceral involvement may occur. Kaposi's sarcoma occurs spontaneously in Jewish and Italian males in Europe and the United States. An aggressive variant in young children is endemic in some areas of Africa. A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients. (From Dorland, 27th ed & Holland et al., Cancer Medicine, 3d ed, pp2105-7) HHV-8 is the suspected cause.
Definition (CSP) multicentric, malignant neoplastic vascular proliferation characterized by the development of bluish-red cutaneous nodules, usually on the lower extremities, most often on the toes or feet, and slowly increasing in size and number and spreading to more proximal areas; occurs spontaneously in Jewish and Italian males; aggressive variant in young children is endemic in some areas of Africa; a third form occurs in about 0.04% of kidney transplant patients; there is also a high incidence in AIDS patients; human herpesvirus 8 is the suspected cause.
Concepts Neoplastic Process (T191)
MSH D012514
ICD9 176.9, 176
ICD10 C46 , M9140/3, C46.9
SnomedCT 49937004, 109385007, 154604006, 190112000, 188483007
English Kaposi Sarcoma, Kaposi's Sarcoma, Sarcoma, Kaposi, Sarcoma, Kaposi's, Kaposis Sarcoma, Kaposi's sarcoma, unspecified, Kaposi's sarcoma,unspecifd, [X]Kaposi's sarcoma, unspecified, [X]Kaposi's sarcoma,unspecifd, [M]Kaposi's sarcoma, Kaposi's sarcoma, morphology, Kaposi's sarcoma (diagnosis), malignant neoplasm sarcoma Kaposi's, Multiple idiopathic hemorrhagic sarcoma, Kaposi's sarcoma NOS, Kaposi sarcoma, morphology, Kaposi sarcoma, Sarcoma, Kaposi [Disease/Finding], kaposi sarcoma, Sarcoma;Kaposis, Kaposi's sarcoma of unspecified site, Multiple Idiopathic Pigmented Hemangiosarcoma, Kaposi's sarcomas, Kaposi's sarcoma, unspecified site, Multiple idiopathic haemorrhagic sarcoma, [X]Kaposi's sarcoma, unspecified (disorder), Multiple hemorrhagic sarcoma, Kaposi's sarcoma, KS - Kaposi's sarcoma, Multiple haemorrhagic sarcoma, Kaposi's sarcoma (clinical), Kaposi's sarcoma (disorder), Kaposi's sarcoma, morphology (morphologic abnormality), sarcoma, multiple hemorrhagic, multiple hemorrhagic sarcoma, sarcoma, Kaposi's, Skin cancer, Kaposi's sarcoma, HHV8, Kaposi; sarcoma, unspecified site, Kaposi; sarcoma, sarcoma; Kaposi, unspecified site, sarcoma; Kaposi, Kaposi's sarcoma (morphologic abnormality), KS, Multiple Hemorrhagic Sarcoma, HHV 8, Kaposis sarcoma
Italian Sarcomi di Kaposi, Sarcoma di Kaposi non specificato, Sarcoma emorragico multiplo, Sarcoma emorragico idiopatico multiplo, Sarcoma di Kaposi NAS, Sarcoma emorragico idiopatico, Sarcoma di Kaposi
Dutch multipel idiopathisch hemorragisch sarcoom, multipel hemorragisch sarcoom, Kaposi-sarcoom, niet-gespecificeerd, multipel haemorrhagisch sarcoom, Kaposi-sarcoom NAO, Kaposi; sarcoom, niet gespecificeerde lokalisatie, Kaposi; sarcoom, sarcoom; Kaposi, niet gespecificeerde lokalisatie, sarcoom; Kaposi, Kaposi-sarcoom, niet gespecificeerd, Kaposi-sarcomata, Kaposi-sarcoom, Sarcoom, Kaposi-
French Sarcome de Kaposi SAI, Sarcomatose multiple hémorragique, Sarcome hémorragique idiopathique multiple, Sarcome de Kaposi non précisé, Sarcomes de Kaposi, Sarcome de Kaposi, Sarcomatose multiple hémorragique de Kaposi, Sarcomatose pigmentaire idiopathique, Maladie de Kaposi
German mehrfaches haemorrhagisches Sarkom, mehrfaches idiopathisches haemorrhagisches Sarkom, Kaposi Sarkom, unspezifisch, Kaposi Sarkom NNB, Kaposi-Sarkom [Sarcoma idiopathicum multiplex haemorrhagicum], Kaposi-Sarkom, nicht naeher bezeichnet, Kaposi Sarkome, Kaposi-Sarkom, Sarkom, Kaposi-
Portuguese Sarcoma hemorrágico múltiplo, Sarcoma de Kaposi NE, Sarcoma hemorrágico idiopático múltiplo, Sarcomas de Kaposi, Sarcoma de Kaposi
Spanish Sarcoma de Kaposi NEOM, Sarcoma hemorrágico idiopático múltiple, Sarcoma hemorrágico múltiple, Sarcoma de Kaposi, no especificado, sarcoma de Kaposi, morfología (anomalía morfológica), sarcoma de Kaposi (anomalía morfológica), sarcoma de Kaposi, morfología, [X]sarcoma de Kaposi, no especificado (trastorno), [X]sarcoma de Kaposi, no especificado, (M)sarcoma de Kaposi, morfología de sarcoma de Kaposi, sarcoma de Kaposi (clínico), sarcoma de Kaposi (trastorno), sarcoma de Kaposi, sarcoma hemorrágico múltiple, Sarcomas de Kaposi, Sarcoma de Kaposi
Japanese 多発性特発性出血性肉腫, カポジ肉腫、詳細不明, カポジ肉腫NOS, カポジニクシュショウサイフメイ, タハツセイトクハツセイシュッケツセイニクシュ, カポジニクシュ, カポジニクシュNOS, タハツセイシュッケツセイニクシュ, Kaposi肉腫, カポジー肉腫, カポジ肉腫, 多発出血性肉腫, 多発性出血性肉腫, 特発性多発出血性肉腫, 特発性多発性出血性肉腫, 肉腫-Kaposi, 肉腫-カポジ
Swedish Sarkom, Kaposis
Czech sarkom Kaposiho, Kaposiho sarkomy, Kaposiho sarkom, Blíže neurčený Kaposiho sarkom, Mnohočetný idiopatický hemoragický sarkom, Kaposiho sarkom NOS, Mnohočetný hemoragický sarkom
Finnish Kaposin sarkooma
Korean 상세불명의 카포시 육종, 카포시 육종
Polish Mięsak krwotoczny mnogi Kaposiego, Mięsak Kaposiego, Mięsakowatość mnoga krwotoczna
Hungarian Kaposi-sarcoma k.m.n., Többszörös haemorrhagiás sarcoma, Többszörös ismeretlen eredetű haemorrhagiás sarcoma, Többszörös vérzéses sarcoma, Kaposi-sarcomák, Többszörös idiopathiás haemorrhagiás sarcoma, Kaposi-sarcoma, nem meghatározott, Kaposi-sarcoma
Norwegian Kaposis sarkom, Multippelt idiopatisk pigmentert hemangiosarkom