Iatrogenic Kaposi`s sarcoma C46.-

Last updated on: 22.08.2021

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Definition
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Iatrogenic Kaposi's sarcoma (KS), also called drug-induced KS or immunosuppression-associated Kaposi's sarcoma, is a vascular neoplasia caused by human herpesvirus 8 (HHV-8) that manifests on the skin and mucous membranes of individuals with artificially impaired immune defenses.

Classification
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Kaposi's sarcoma is classified as follows:

  • Classic indolent Kaposi's sarcoma: This occurs in males in the 6th to 7th decade of life; often the initial lesion is a blue-red nodule of the skin associated with edema of the affected limb; subsequently there is a slow increase in the size as well as the number of lesions, ulceration may also occur. In a random Turkish collective of KS patients (n=137), the classic variant was the most common (64%).
  • Endemic Kaposi's sarcoma: affects HIV-negative adults and children in Africa.
  • AIDS-associated Kaposi 's sarcoma: most aggressive form of Kaposi's sarcoma in patients with AIDS; frequent skin lesions of the face, genitalia, and lower extremity; also frequent involvement of the oral mucosa, gastrointestinal tract, lymph nodes, or lungs.
  • Iatrogenic Kaposi's sarcoma: affects patients after organ transplantation and on immunosuppressive therapy; very rare; develops after a few months to years after transplantation or immunosuppressive therapy. In a random Turkish collective of KS patients (n=137), the iatrogenic variant was the second most common variant at 27%. Approximately 30% of these patients were transplant recipients. 16 iatrogenic KS patients were systemically treated with a corticosteroid. 4 patients suffered from myasthenia gravis, 3 from rheumatoid arthritis (Baykal C et al. 2019).

Basically, from a clinical point of view, iatrogenic Kaposi's sarcoma can be distinguished between 2 collectives:

  • Iatrogenic KS, after organ transplantation (0.5-5.3% of organ transplanted patients).
  • Iatrogenic KS in various underlying diseases in which a long-term or permanent immunosuppressive therapy was necessary.

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Occurrence/Epidemiology
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The iatrogenic variant is mainly associated with the use of immunosuppressive therapy.

Associated KS has been reported in the following underlying diseases:

The following (immunosuppressive) drugs have been associated with Kaposi's sarcoma:

Etiopathogenesis
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While many affected patients are organ transplant patients, it must be considered that other forms of immunosuppression may also lead to the development of Kaposi's sarcoma. In transplant patients, iatrogenic KS can be observed due to the necessary application of systemic immunosuppressants.

Basically, HHV-8 is necessary for the development of Kaposi's sarcoma. Co-infection with human cytomegalovirus (HCMV) leads to pathogenicity enhancement of HHV-8 (Wells R et al. 2009). Furthermore, the type and duration of immunosuppressive therapy, age, and hormonal, geographic, ethnic, genetic, and environmental factors play a role in iatrogenic KS.

Histology
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However, the histological picture of the four subtypes does not differ. While the early lesions of the skin are uncharacteristic, in the so-called patch stage an increased number of vascular clefts can be recognized. These are lined by flat to oval endothelial cells and show little or no atypia. Concomitantly, a lymphoplasmacytic cellular infiltrate may be present. Extravascular erythrocytes and hemosiderin are detectable.

In the plaque stage, all signs of the patch stage are somewhat more pronounced. In addition, hyaline globules may be found.

The nodular stage follows, in which the lesions are very sharply demarcated. They consist of spindle cells with only minor cytologic atypia and numerous slit-like cavities filled with erythrocytes. Again, hyaline deposits may be apparent both inside and outside the spindle cells.

Immunohistochemically, the cells of KS are positive for the endothelial markers CD31, CD34 and ERG as well as for the lymphatic marker D2-40. Likewise, they show nuclear expression of HHV8.

Therapy
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The first option is to discontinue or reduce the dose of the responsible immunosuppressive drugs. It is hoped that this will lead to healing or extensive regression of the lesions.

In addition,:

  • radiotherapy for oral and cutaneous lesions
  • Chemotherapy (local or systemic)
  • Surgery (skin and intestine)
  • Immunotherapy (local or systemic with interferon)
  • Retinoic acid
  • Imiquimod and
  • cryosurgery as therapeutic options.

Clinical staging is essential for treatment. Localized disease can be treated with different local therapies, although there are no randomized trials comparing these different modalities. Aggressive, disseminated KS and cases with visceral involvement usually require systemic chemotherapy, usually with vinblastine, bleomycin , or paclitaxel.

KS after transplantation should be switched to m-TOR inhibitors if possible.

Ulcerative colitis: In cases of colonic involvement, proctocolectomy is the treatment of choice, as this therapeutic intervention can lead to cure of KS and ulcerative colitis (Li J et al. 2020; Svrcek M et al. 2009).

Progression/forecast
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Iatrogenic KS, which develops in chronic rheumatic diseases due to prolonged use (consciously or unconsciously) of steroids, usually has a favorable course and often regresses with discontinuation and reduction of the dose of the drug. In patients receiving immunosuppressive treatment for nontransplant reasons, KS develops 2 to 4 times less frequently and much more slowly (Baykal C et al. 2019).

Literature
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  1. Akao S et al (2020) Iatrogenic penile Kaposi sarcoma. Intern Med 59:2445.
  2. Avalos-Peralta P et al (2006) Localized Kaposi's sarcoma in a patient with pemphigus vulgaris. J Eur Acad Dermatol Venereol 20: 79-83.

  3. Anton E (2008) Kaposi's sarcoma in an immunocompetent patient following corticosteroid therapy. Eur J Intern Med 19: 226.
  4. Ascoli V et al. (2009) Cause-specific mortality in classic Kaposi's sarcoma: A population-based study in Italy (1995-2002) Br J Cancer 101:1085-1090.
  5. Baykal C et al (2019) The Spectrum of Underlying Causes of Iatrogenic Kaposi's Sarcoma in a Large Series: A Retrospective Study. Indian J Dermatol 64:392-399.
  6. Berti A et al (2015) Kaposi's Sarcoma in a Patient with Eosinophilic Granulomatosis with Polyangiitis While Taking Mycophenolate Mofetil. J Allergy Clin Immunol Pract 3:431-432.

  7. Billon E et al (2018) Reversible rituximab-induced rectal Kaposi's sarcoma misdiagnosed as ulcerative colitis in a patient with HIV-negative follicular lymphoma. Clin Sarcoma Res 8:11.
  8. Buonaguro FM et al (2003) Kaposi's sarcoma: aetiopathogenesis, histology and clinical features. J Eur Acad Dermatol Venereol17:138-154.
  9. Camcioglu Y et al (2004) HHV-8-associated Kaposi's sarcoma in a child with IFNgammaR1 deficiency. J Pediatr 144:519-523.
  10. Chen SA et al (2021) Extensive cutaneous iatrogenic Kaposi's sarcoma after bullous pemphigoid treatment with oral methylprednisolone: a rare Chinese case report. J Int Med Res 49:300060520982829.
  11. de Fijter JW (2017) Cancer and mTOR inhibitors in transplant recipients. Transplantation 101:45-55.
  12. Frangiamore R etal. (2021) Iatrogenic Kaposi's sarcoma in myasthenia gravis: learnings from two case reports. Neurol Sci 42:2081-2083.
  13. Innes AJ et al (2017) Immunosuppression-associated Kaposi's sarcoma following stem cell transplantation. Br J Haematol 178:9.
  14. Lee SYet al (2012) Disseminated cutaneous and visceral Kaposi sarcoma in a woman with rheumatoid arthritis receiving leflunomide. Rheumatol Int 32:1065-1068.
  15. Li J et al. (2020) Kaposi sarcoma combined with severe ulcerative colitis: A case report and literature review. Beijing Da Xue Xue Bao Yi Xue Ban 52:373-377.
  16. Mantero V et al (2013) Myasthenia gravis developing in an HIV-negative patient with Kaposi's sarcoma. Neurol Sci 34:1249-50.
  17. Omine T et al (2020) Iatrogenic Kaposi's sarcoma in a myelofibrosis patient treated with ruxolitinib. J Dermatol 47:e131-e132.
  18. Papa V et al (2020) Human herpesvirus 8-associated colonic Kaposi's sarcoma during vedolizumab treatment in ulcerative colitis: a case report and review of the literature. BMC Gastroenterol 20:76.
  19. Senturk N et al (2001) Human herpesvirus 8 (HHV-8) in non-HIV associated forms of Kaposi's sarcoma from Turkey. Turk J Med Sci 31:503-508.
  20. Svrcek M et al. (2009) KSHV/HHV8-associated intestinal Kaposi's sarcoma in patient with ulcerative colitis receiving immunosuppressive drugs: report of a case. Dis Colon Rectum 52:154-158.
  21. Taniguchi T et al. (2011) Disseminated cutaneous and visceral Kaposi's sarcoma in a patient with rheumatoid arthritis receiving corticosteroids and tacrolimus. Mod Rheumatol 21: 309-312.
  22. Tourlaki A et al. (2018) Autoimmune bullous diseases in non-HIV Kaposi's sarcoma: a retrospective study in a large cohort of patients. J Eur Acad Dermatol Venereol 32:1777-1783.
  23. Tremblay C et al (2017) Kaposi's Sarcoma Associated With Iatrogenic Immunosuppression: A Rare Complication of Bullous Pemphigoid Treatment. J Cutan Med Surg 21:449-451.
  24. Ulbright TM et al (1981) Kaposi's sarcoma: relationship with hematologic, lymphoid, and thymic neoplasia. Cancer47:963-973.
  25. Windon AL et al. (2018) Iatrogenic Kaposi's sarcoma in an HIV-Negative Young Male With Crohn's Disease and IgA Nephropathy: A Case Report and Brief Review of the Literature. Int J Surg Pathol 26:276-282.
  26. Weissmann A et al. (2000) Epidemiological study of classic Kaposi's sarcoma: A retrospective review of 125 cases from Northern Israel. J Eur Acad Dermatol Venereol14:91-95.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 22.08.2021