Kaposi Sarcoma: History
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Contributor:
Alessandro Pileri
,
Bianca Maria Piraccini
,
Corrado Zengarini

Kaposi’s sarcoma is an angioproliferative neoplasm that typically occurs in the lower limbs and can enter into differential diagnosis with several other rarer skin diseases. The principal differential diagnosis concerns primary cutaneous lymphomas, of which mycosis fungoides represent the most frequent primary cutaneous T-cell lymphoma. Other rare forms include primary cutaneous B-cell lymphomas, which can be divided into indolent and aggressive forms, such as the primary cutaneous diffuse large B-cell lymphoma, leg type, and lymphomatoid papulomatosis (LyP). In the case of indolent lesions, skin-directed therapies, limited-field radiotherapy, and surgical approaches can be good options. At the same time, different management, with systemic chemotherapy and allogenic bone marrow transplant, is required with aggressive neoplasms, such as blastic plasmacytoid dendritic cell neoplasia or advanced mycosis fungoides. The dermatologist’s role can be crucial in recognizing such diseases and avoiding misdiagnosis, giving the pathologist the correct clinical information for an accurate diagnosis, and starting the suitable therapy.

  • Kaposi sarcoma
  • leg

Kaposi Sarcoma

Kaposi sarcoma (KS) is an angioproliferative neoplasm related to human herpes virus 8 (HHV8). Five clinical variants have been described, ranging from Classic KS (CKS), African endemic KS, immunosuppressed-related form, KS in HIV-negative men who have sex with men and AIDS-related KS [1][2][3][4][5]. The incidence of classic KS is higher in Mediterranean countries, and the overall Italian incidence rate has been estimated to be 1/100,000 in men and 0.4/100,000 in women. Iatrogenic KS is commonly detected in organ transplant recipients (OTR), with an increased risk estimated to be from 100 to 500 times higher than in the general population. Regarding the clinical aspects, skin manifestations can range from pink to violaceous papules, patches, and ulcerated nodules. CKS usually involves the legs, and lymphedema is the most common cutaneous complication. Oral, genital or conjunctival involvement can be detected at physical examination [6], and in advanced stages, lymph node and visceral (bladder, lung and kidney) involvement are possible. CKS has an indolent clinical behavior, while the remaining forms are more aggressive and easily involve lymph nodes or visceral organs [2]. Besides CKS, which usually affects the sixth decade, patients of any age can be affected, and pediatric cases have been recorded [7][8]. Histology is crucial for KS diagnosis. Skin lesions show similar features and patches with a perivascular infiltrate of scattered plasma cells and lymphocytes. A proliferation of oddly-shaped endothelial cell-lined vascular channels behind the existing blood vessels is a clue for KS diagnosis. Such channels become more prominent in patch lesions along with the presence of CD31+ and CD34+ spindle cells. In nodular lesions, a dermal mass of spindle cells is visible with light microscopy along with extravasated red blood cells.
In immunohistochemistry, staining against HHV-8 results in positive staining in spindle cells. Scheduled treatments depend on the clinical variant, KS extension and immune system status. Dermoscopy of KS can be helpful, and polychromatic color change (Rainbow pattern), collarette sign, white lines, white clouds, and serpentine vessels have been related to KS, especially in the case of nodular lesions [9]. The Italian recommendations [6] suggest treating with local therapy, (mainly intralesional vincristine or silver nitrate cauterization) as lesions can negatively affect patients’ quality of life in case of indolent KS lesions. Aggressive, diffuse and visceral KS should be managed with systemic treatments such as vinblastine alone, vinblastine associated with bleomycin, or paclitaxel [6] (Brambilla IJDV 2021). In immunosuppressed patients, medication changes usually lead to KS regression [2][10][11]. In HIV-related forms, pegylated liposome doxorubicin or paclitaxel are commonly used along with ART (Antiretroviral Therapy) [6][12][13].

This entry is adapted from the peer-reviewed paper 10.3390/dermato3010005

References

  1. Marcoval, J.; Bonfill-Ortí, M.; Martínez-Molina, L.; Valentí-Medina, F.; Penín, R.M.; Servitje, O. Evolution of Kaposi Sarcoma in the Past 30 Years in a Tertiary Hospital of the European Mediterranean Basin. Clin. Exp. Dermatol. 2019, 44, 32–39.
  2. Etemad, S.A.; Dewan, A.K. Kaposi Sarcoma Updates. Dermatol. Clin. 2019, 37, 505–517.
  3. Lodi, S.; Guiguet, M.; Costagliola, D.; Fisher, M.; de Luca, A.; Porter, K.; the CASCADE Collaboration. Kaposi Sarcoma Incidence and Survival Among HIV-Infected Homosexual Men After HIV Seroconversion. JNCI J. Natl. Cancer Inst. 2010, 102, 784–792.
  4. Cahoon, E.K.; Linet, M.S.; Clarke, C.A.; Pawlish, K.S.; Engels, E.A.; Pfeiffer, R.M. Risk of Kaposi Sarcoma after Solid Organ Transplantation in the United States: Kaposi Sarcoma in Transplant Recipients. Int. J. Cancer 2018, 143, 2741–2748.
  5. Parkin, D.M.; Sitas, F.; Chirenje, M.; Stein, L.; Abratt, R.; Wabinga, H. Part I: Cancer in Indigenous Africans—Burden, Distribution, and Trends. Lancet Oncol. 2008, 9, 683–692.
  6. Brambilla, L.; Genovese, G.; Berti, E.; Peris, K.; Rongioletti, F.; Micali, G.; Ayala, F.; Della Bella, S.; Mancuso, R.; Calzavara Pinton, P.; et al. Diagnosis and Treatment of Classic and Iatrogenic Kaposi’s Sarcoma: Italian Recommendations. Ital J Dermatol Venerol 2021, 156, 356–365.
  7. Silva, L.; Azurara, L.; Monteiro, A.F.; Miroux-Catarino, A.; Amaro, C.; Viana, I. Pediatric Kaposi’s Sarcoma Associated with Immune Reconstitution Inflammatory Syndrome. Pediatr. Dermatol. 2020, 37, 239–240.
  8. El-Mallawany, N.K.; McAtee, C.L.; Campbell, L.R.; Kazembe, P.N. Pediatric Kaposi Sarcoma in Context of the HIV Epidemic in Sub-Saharan Africa: Current Perspectives. PHMT 2018, 9, 35–46.
  9. Micali, G.; Nasca, M.R.; De Pasquale, R.; Innocenzi, D. Primary Classic Kaposi’s Sarcoma of the Penis: Report of a Case and Review. J. Eur. Acad. Dermatol. Venereol. 2003, 17, 320–323.
  10. Requena, C.; Alsina, M.; Morgado-Carrasco, D.; Cruz, J.; Sanmartín, O.; Serra-Guillén, C.; Llombart, B. Sarcoma de Kaposi y angiosarcoma cutáneo: Directrices para el diagnóstico y tratamiento. Actas Dermo-Sifiliográficas 2018, 109, 878–887.
  11. Naimo, E.; Zischke, J.; Schulz, T.F. Recent Advances in Developing Treatments of Kaposi’s Sarcoma Herpesvirus-Related Diseases. Viruses 2021, 13, 1797.
  12. Yarchoan, R.; Uldrick, T.S. HIV-Associated Cancers and Related Diseases. N. Engl. J. Med. 2018, 378, 1029–1041.
  13. Poizot-Martin, I.; Lions, C.; Cheret, A.; Rey, D.; Duvivier, C.; Jacomet, C.; Allavena, C.; Huleux, T.; Bani-Sadr, F.; Obry-Roguet, V.; et al. Kaposi Sarcoma in People Living with HIV: Incidence and Associated Factors in a French Cohort between 2010 and 2015. AIDS 2020, 34, 569–577.
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