Kaposi's sarcoma

Last Updated: 2022-07-22

Author(s): Anzengruber F., Navarini A.

ICD11: 2B57.Z

Moritz Kaposi, 1872

Human herpes virus 8, HHV-8, sarcoma idiopathicum haemorrhagicum multiplex, Kaposi's disease, Kaposi's angiomatosis, Kaposi's idiopathic multiple pigment sarcoma, teleangiectatic pseudosarcoma, Kaposi's syndrome, angioreticulomatosis, Kaposi's sarcoma, Kaposi's sarcoma.

Multifocally localised angioproliferative neoplasia of different genesis occurring in all age groups with primary cutaneous occurrence and possible metastasis with a correspondingly poor prognosis.

  • Classical Kaposi's sarcoma: Occurring mainly in men from south-eastern Europe, Russia, Poland and the Mediterranean region (often patients of Turkish origin) after the age of 50. M:F = 10:1. incidence 1/10 million population/year. Ashkenazim Jews show a particularly high incidence.
  • Endemic Kaposi's sarcoma: African children and young men. V.a. in Equatorial Africa a frequent tumour.
  • HIV-associated Kaposi's sarcoma: Occurs mainly in homosexual men. With HIV infection, 5-7% of patients develop Kaposi's sarcoma (before cART it was 20%).
  • Iatrogenic Kaposi's sarcoma: Men are increasingly affected. In most cases, medication with corticosteroids, azathioprine, sirolimus or ciclospirine is established, especially with regard to organ transplanted patients. Kaposi's sarcomas occur more frequently with ciclosporin than with azathioprine. Furthermore, there was an increased incidence of underlying diseases in those affected, such as rheumatoid diseases, sarcoidosis or lupus erythemaodes.

Kaposi's sarcoma can be classified by clinic as follows:

  • Classical/sporadic Kaposi's sarcoma
  • African/endemic Kaposi's sarcoma
  • HIV-associated epidemic Kaposi's sarcoma
  • Kaposi's sarcoma in immunosuppressed patients/iatrogenic Kaposi's sarcoma

Further classification can be according to Mitsuyasu and Groopman, with staging from Sradium I to IV.

For HIV, ACTG staging should also be used.

Endemic Kaposi's sarcoma can in turn be divided into further subgroups depending on the clinic:

  • nodular
  • florid
  • infiltrative
  • lymphadenopathic (V.a. in adolescents)

  • The exact pathogenesis of the disease is not yet fully understood
  • Classical Kaposi's sarcoma: association with HLA DR5
  • HIV-associated Kaposi's sarcoma: HIV/AIDS disease associated with herpes type 8 infection as the cause. Molecular pathology shows that an inflammatory reaction with simultaneous suppression of p53 via LNA1 (latent nuclear antigen) is triggered by HHV8. In addition, the proliferation blockade of the RB protein (retinoblastoma protein) is lifted. Together with the Ras oncogene, this leads to a transformation of the endothelial cell. Furthermore, there is increased cytokine secretion, which promotes the formation of the angiogenic growth factor bFGF, whereby the endothelial cells undergo constant proliferation. However, the cells require not only a proliferation signal but also a spatial orientation signal, e.g. within the framework of a fibronectin, which is imitated by the tat protein expressed by HIV. The tat protein is able to bind to integrins of the edothelial cells. In addition to the described effect, the tat protein is also able to promote the production of collagenases. This enables the infiltrative behaviour of the sarcoma.
  • Iatrogenic Kaposi's sarcoma: Favouring factors are immunosuppression especially after organ transplantation or underlying diseases affecting the skin such as systemic lupus erythematosus or severe atopic eczema
  • Expression of growth factors play a role.

  • Initially, brown-red to purple macules present with a tendency to confluence and formation of marginal foci. Ulceration may occur, especially in mucosal lesions
  • Enorally, the palatum durum is particularly affected.
  • In tribal infestations, an exanthematous picture is seen along the skin cleavage lines
  • In cases of lymphatic drainage disorders, elephantiasis-like oedema formation may be observed
  • Spontaneous remissions occur side by side, as do different tumour stages
  • Staging according to Mitsuyasu and Groopman:
    • Stage IV B: Occurrence of fever and/or weight loss
    • Stage IV A: Absence of general symptoms
    • Stage IV: cutaneous and visceral involvement
    • Stage III: purely visceral involvement
    • Stage II: disseminated cutaneous involvement with > 10 foci or more than one anatomical region
    • Stage I: circumscribed cutaneous involvement with < 10 foci or only one anatomical region
  • In endemic Kaposi's sarcoma, four types are distinguished: nodular, florid, infiltrative and lymphadenopathic. The latter is the most common type, although in many cases only lymph node involvement can be seen. Bone metastases occur in the other types along with other metastasis.
  • Metastases: Preferably lymphogenic, but haematogenous spread to GI tract, heart, lung or liver also possible.
  • When the nails are affected, discolouration of the nail bed, sometimes with pressure pain,
  • occurs
  • In individual cases, there may be increased release of cytokines, which cause systemic changes such as leukaematoid reactions.
  • For a long time, there are no general symptoms.

  • Anamnesis (risk group, origin, underlying diseases)
  • Clinical
  • Dermatopathological confirmation
  • HIV serology
  • HHV8 detection (serological or from tissue sample)
  • Sonography

Generally: Enoral, legs, other localisations.

Classical Kaposi's sarcoma particularly affects the feet, lower and upper legs and shows a proximal spread.

In 5-20% there is visceral involvement.

HIV-associated Kaposi's sarcoma: tip of the nose, ear, glans penis and trunk area along the skin cleft lines. Extracutaneous manifestation is also frequently seen. 30% of patients have mucosal involvement.

In the course, metastases to the internal organs and lymph nodes may occur.

  • Spot stage: Bloodless clefts around vessels or adnexa. Characterised by siderophages and plasma cells presenting a pseudogranulomatous pattern. Orthokeratotic keratinisation, mostly lymphocytic and macrophagocytic infiltrate.
  • Plaque stage: Typical presence of spindle cell fascicles, blood-filled slits and dilated vessels. Orthokeratotic keratinisation, mostly lymphocytic and macrophagocytic infiltrate.
  • Tumour stage: One encounters an increased number of mitoses of the spindle cell nodules, without significant nuclear atypia. Intracytoplasmic hyaline globi are common. HHV-8 DNA sequences are detectable. Tumour cells are vimentin, often factor VIII positive and CD31 positive.

With regard to HIV-associated Kaposi's sarcoma, attention should be paid to protected sexual intercourse.

  • New data show no influence of CD4 cell count on prognosis in patients on HAART. Prognostically significant is the effectiveness of antiretroviral therapy as well as the stage of HIV disease.
  • Staging according to clinical type:
    • Kaposi's sarcoma in immunosuppression with a life expectancy of months to years.
    • HIV-associated Kaposi's sarcoma with a life expectancy of a few months to years. The prognosis is mainly determined by the stage of the HIV infection and the effectiveness of the antiretroviral therapy. In many cases, there is organ involvement with possible lymphatic, pulmonary or gastrointestinal involvement
    • Endemic Kaposi's sarcoma with a life expectancy of a few months to years
    • Classical Kaposi's sarcoma with a life expectancy of years to decades, although lymphoma is more common in these patients during the course. Spontaneous remission is possible.
    • Staging of HIV-associated epidemic Kaposi's sarcoma according to ACTG:
      • Early stage with good prognosis if all of the following are fulfilled: T0 stage with purely cutaneous or lymphogenic involvement, with mild oral involvement with non-elevated lesions of the hard palate also falling into this stage. Immune status I0 with CD4 cells > 200/µl. Symptoms S0 with absence of opportunistic infections, oral thrush or B-symptomatology.
      • Late stage with poor prognosis if any of the following: T1 stage with pulmonary or gastrointestinal involvement, extensive oral involvement and/or oedema and ulceration. Immune status I1 with CD4 cells < 200/µl. Symptoms S1 with anamnestically described opportunistic infections, oral thrush, malignant lymphoma, HIV-related neurological diseases or B symptoms.
    • Endemic Kaposi's sarcoma:
      • The nodular type has a good prognosis, with the infiltrative and florid types having a worse course due to their aggressiveness.
    • Iatrogenic Kaposi's sarcoma is significantly more aggressive than the classic type. A regression to complete disappearance of the lesions, on the other hand, can often be observed when immunosuppression is discontinued or reduced. If immunosuppression must be urgently maintained, off-label therapy with mycophenolate mofetil can be started, under which remission remains possible.

  • General:
    • Whenever possible, the underlying disease should be treated.
    • For a few lesions, surgical excision may be appropriate. Cryotherapy is only suitable for superficial lesions.
    • For solitary lesions, radiotherapy (GD: 20-30Gy, ED:4-5Gy) has a 90% success rate.
    • Locally, retinoids such as Tretionin cream 0.005% can be used 1-2 times daily. However, there is an off-label use. For sensitive skin, the frequency of treatments can be increased to once daily or every 2 days. The duration of treatment is 6-14 weeks. Treatment should be avoided during pregnancy or breastfeeding and in cases of previously known rosacea.
  • According to tumour size:
    • Intraorally: intralesional vinblastine or 3% sodium tetradecyl sulphate
    • Large in area (> 4 cm in diameter; nodular, infiltrating, oral): irradiation using fast electrons or fractionated cobalt irradiation, additional compression measures can be implemented on the lower legs.
    • Medium-sized areas (1-4 cm in diameter; macular, nodular): intralesional vinca alkaloids or fractionated dermopan irradiation
    • Small area (≥ 1 cm² macular, nodular): cryosurgery or excision, intralesional vincristine, vinblastine or interferon, and alitretinoin gel 0.1%
    • If a specific effect is desired in the treatment, the following substances can be used:
      • Angiogenesis inhibitors: AGM 1470, TNP 470, glufanide disodium, IM 862, thalidomide, metalloprotease inhibitors
      • Keratocyte proliferation inhibitors: 9-cis-retinoic acid, liposomal tretinoin
      • Inhibition of angiogenesis, proliferation and matalloprotease activity: urinary HCG, or HCG fractions
  • HIV-associated Kaposi's sarcoma:
    • Stage T1, I0, S0-1 and > 200 CD4 lymphocytes/μl: interferon alfa-2a or interferon alfa-2b 3 times weekly at a dose of 6M IU s.c. with a remission rate of 40-50%. Possible side effects include fever, muscle pain, arthralgias and depressed mood, evidence level BIII
    • Stage T1, I1, S0-1: liposomal doxorubicin (pegylated) every 2 weeks at a dose of 20 mg/m2 KO i.v. with a remission rate of 60-80%. Possible side effects include neutropenia, anaemia, a sensation of heat, dyspnoea, back pain and palmoplantar erythrodysaesthesia, evidence level AI
    • Stage T1, I1, S0-1: Liposomal daunorubicin i.v. every 2 weeks at a dose of 40 mg/m2 KO with a remission rate of approximately 60%. Possible side effects include neutropenia, anaemia, a sensation of heat, dyspnoea , back pain and palmoplantar erythrodysaesthesia, evidence level AI
    • Stage T1, I1, S0-1: Paclitaxel (Taxol) every 2 weeks at a dose of 100 mg/m2 KO i.v. or every 3 weeks at a dose of 35 mg/m2 KO i.v. with a remission rate of 50-60%. Possible side effects include neutropenia, peripheral neuropathy, allergic skin reactions, hypotension, ECG changes and alopecia, evidence level BII
  • All therapies are applicable in combination with antiretroviral therapy.
  • Cidofovir can be used against HHV 8.
  • Therapy is administered in the palliative setting according to the treatment guidelines for angiosarcomas. The following options exist here:
    • Paclitaxel at a dose of 80mg/m2 on days 1, 8 and 15 for a total duration of 4 weeks. According to the ANGIOTAX study by Penel et al, progression-free survival can be seen in 74% and 45% of patients after 2 and 4 weeks, respectively. A retrospective analysis by Schlemmer et al. (EORTC study), in which a wide variety of therapy regimens were investigated, was able to demonstrate a response rate of 62%.
    • Gemcitabine at a dose of 1000mg/m2 initially every week for a period of 1-3 weeks followed by a 4-week interval. Stacchiotti et al. studied 25 patients with a clinical response and response rate of 68%
    • Paclitaxel in combination with peg.lip. Doxorubicin can be given at a dose of 140mg/m2 for 6 days every 4 weeks and 50mg/m2 every 4 weeks. Skubitz et al, Cancer, 2005.
    • Also possible is a combination treatment with trofosfamide (3x50mg daily), rofecoxib (25mg daily) as well as pioglitazone (45mg daily) within the framework of a "second line" therapy after the possible previous therapies mentioned according to Vogt et al., Cancer 2003. This is a long-term therapy.
  • Follow-up care:
    • In HIV-associated and endemic Kaposi's sarcoma, a check of the skin, mucous membranes and lymph nodes should be performed every 3 months, and a conventional radiological check of the lungs and a sonographic or endoscopic check of the GI tract is indicated every 6-12 months. This is due to the usually short duration of remission.
    • Classical Kaposi's sarcoma is inferior to other diseases in terms of mortality. A dermatological examination is indicated every 6 months. In case of recurrence of skin changes, a shorter interval should be chosen.

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