Kaposi's sarcoma

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Classification according to ICD-10
C46 Kaposi's sarcoma [Sarcoma idiopathicum multiplex haemorrhagicum]
ICD-O M9140 / 3
ICD-10 online (WHO version 2019)

The Kaposi sarcoma ([ ˈkɒpoʃi ]) is a rare one, especially in people with a weakened immune system ( immunosuppression ), u. a. in AIDS occurring cancer caused by the human herpes virus type 8 (HHV-8) is triggered.

The disease manifests itself through the appearance of brownish-bluish tumor nodules, especially in the skin, mucous membranes and intestines. In the form associated with AIDS, numerous brown-bluish spots usually appear on the skin of the legs and arms.

history

Kaposi's sarcoma is named after the Austrian dermatologist of Hungarian origin Moriz Kaposi (1837–1902), who first described it in Vienna in 1872 in five men of Mediterranean origin as an idiopathic multiple pigment sarcoma of the skin .

When AIDS was first described in California homosexual men in 1981, Kaposi's sarcoma was one of the AIDS-defining conditions.

distribution

The human herpes virus 8 is sexually transmitted, but presumably also vertically from mother to child and horizontally via saliva between siblings.

Kaposi's sarcoma usually only develops when the immune system is weak, as in AIDS or immunosuppressive therapy, but also in old age and with other serious underlying diseases. Men are affected more frequently than women. In the case of severe immunosuppression, there is often a disseminated, multifocal occurrence; otherwise, individual manifestations are more likely to be observed.

After transplants there is also an increased risk of disease due to the weakened immune system due to the immunosuppressive drugs . The disease often attacks the internal organs directly and does not show any symptoms of the skin.

Symptoms

Kaposi's sarcoma in an AIDS patient
Multiple Kaposi's sarcomas of the gums in an AIDS patient
Cutaneous Kaposi's sarcoma in an AIDS patient

Single or multiple livid spots ( papules ) and lumps ( nodules ) on the skin of the feet, and especially on the soles of the feet, as well as on the legs, trunk and face, are typical . The skin lesions often have an irregular and fuzzy border and are painless. There is no fever - in contrast to bacillary angiomatosis , which is triggered by Bartoneles and also causes livid, irregularly defined patches of skin, but is associated with fever.

Kaposi's sarcoma can spread to the regional lymph nodes , which can sometimes lead to pronounced lymphedema of the dependent (distal) limb sections. If it spreads disseminated throughout the body, the mouth , gastrointestinal tract , urogenital tract and internal organs can also be involved with the risk of gastrointestinal bleeding and vomiting of blood ( hematemesis ). If the lungs are involved, coughing up blood ( hemoptysis ) can occur. In the mouth there are often very small and inconspicuous livid spots, especially on the hard and soft palate , on the gums and on the mucous membrane of the cheek.

To confirm the diagnosis one is biopsy with historical pathological workup necessary.

therapy

In the treatment of Kaposi's sarcoma, it is primarily important to maintain the function of the immune system, which is why combination antiviral therapy (ART) is of great importance in HIV and AIDS patients . The same goes for transplant recipients. A change in immunosuppressive therapy can lead to long-term control and even partial regression of Kaposi's sarcoma.

There are also other therapeutic approaches aimed directly at the tumor:

In HIV-infected people, chemotherapy shows a fairly high response rate, but there is no clinical evidence that chemotherapy offers an additional survival benefit beyond the effects of antiretroviral therapy (ART) alone. It is therefore only recommended if Kaposi's sarcoma is highly disseminated, has made massive progress, has already led to organ insufficiency, or is progressing despite antiretroviral therapy. Most HIV patients, however, are adequately treated with ART and without chemotherapy.

literature

  • SR Mallery, P. Pei, DJ Landwehr, CM Clark, JE Bradburn, GM Ness, FM Robertson: Implications for oxidative and nitrative stress in the pathogenesis of AIDS-related Kaposi's sarcoma . In: Carcinogenesis , 25, 2004, pp. 597-603, PMID 14656937
  • SR Mallery, RT Bailer, CM Hohl, CL Ng-Bautista, GM Ness, BE Livingston, BL Hout, RE Stephens, GP Brierley: Cultured AIDS-related Kaposi's sarcoma (AIDS-KS) cells demonstrate impaired bioenergetic adaptation to oxidant challenge: implication for oxidant stress in AIDS-KS pathogenesis . In: J Cell Biochem. , 59, 1995, pp. 317-328, PMID 8567750
  • L. Feller, NH Wood, J. Lemmer: HIV-associated Kaposi sarcoma: pathogenic mechanisms . In: Oral Surg Oral Med Oral Pathol Oral Radiol Endod. , December 1, 2006, PMID 17142074
  • A. Crispo, M. Tamburini, MR de Marco, P. Ascierto, P. Silvestro, D. Ronga, V. Tridente, S. Desicato, S. Carbone, G. Fabbrocini, D. Spiteri, M. Montella: HHV- 8 prevalence, immunosuppression and Kaposi's sarcoma in South Italy . In: Int J Mol Med. May 7, 2001, pp. 535-538, PMID 11295117
  • DV Ablashi, LG Chatlynne, JE Whitman Jr., E. Cesarman: Spectrum of Kaposi's sarcoma-associated herpesvirus, or human herpesvirus 8, diseases . In: Clin Microbiol Rev. , 15, 2002, pp. 439-464, PMID 12097251
  • E. Guttman-Yassky, J. Dubnov, Z. Kra-Oz, R. Friedman-Birnbaum, M. Silbermann, M. Barchana, R. Bergman, R. Sarid: Classic Kaposi sarcoma. Which KSHV-seropositive individuals are at risk? In: Cancer , 106, 2006, pp. 413-419, PMID 16353205
  • EE Brown, D. Whitby, F. Vitale, V. Marshall, G. Mbisa, C. Gamache, C. Lauria, AJ Alberg, D. Serraino, P. Cordiali-Fei, A. Messina, JJ Goedert: Virologic, hematologic , and immunologic risk factors for classic Kaposi sarcoma . In: Cancer , 107, 2006, pp. 2282-2290, PMID 16998933
  • Karl Holubar : Kaposi, Moriz and Kaposi sarcoma. In: Werner E. Gerabek , Bernhard D. Haage, Gundolf Keil , Wolfgang Wegner (eds.): Enzyklopädie Medizingeschichte. De Gruyter, Berlin / New York 2005, ISBN 3-11-015714-4 , p. 717.

Web links

Commons : Kaposi's Sarcoma  - Collection of Images, Videos, and Audio Files

Individual evidence

  1. ↑ David T. Durack: Opportunistic Infections and Kaposi's Sarcoma in Homosexual Men New England Journal of Medicine 1981, Volume 305, Issue 24 of December 10, 1981, pages 1465-1467, DOI: 10.1056 / NEJM198112103052408
  2. ↑ M. Sand, D. Sand, C. Thrandorf, V. Paech, P. Altmeyer, FG Bechara: Cutaneous lesions of the nose. In: Head & face medicine Volume 6, 2010, p. 7, ISSN  1746-160X . doi: 10.1186 / 1746-160X-6-7 . PMID 20525327 . (Review article in Open Access ).
  3. ↑ a b Michael Saag, Dexter P. Mendoza, Marc S. Sherman, MD, Gregory M. Cote, MD, Angela R. Shih: Case 36-2019: A 34-Year-Old Man with Dyspnea, Odynophagia, and Abdominal Pain , New England Journal of Medicine 2019, Volume 381, Issue 21, Nov. 21, 2019, pages 2052-2061, DOI: 10.1056 / NEJMcpc190962599