Kaposi's sarcoma classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2] Huda A. Karman, M.D.

Overview

Kaposi’s sarcoma may be classified according to the clinical setting of the disease into five subtypes include Classic Kaposi’s sarcoma, African cutaneous Kaposi’s sarcoma, African lymphadenopathic Kaposi’s sarcoma, Immunosuppression-associated Kaposi’s sarcoma, and AIDS-associated Kaposi’s sarcoma.

Classification

According to the clinical setting of the disease, clinical presentation, epidemiology, and prognosis Kaposi’s sarcoma may be classified into five different subtypes^[1]^[2]^[3]
African cutaneous Kaposi’s sarcoma and African lymphadenopathic Kaposi’s sarcoma could be classified under the term of endemic Kaposi’s sarcomas.

Types	Characteristics
Classic (Mediterranean) Kaposi’s sarcoma ^[4]^[5]^[6]^[7]	Vascular sarcoma Common in Mediterranean elderly men Indolent clinical behavior Pigmented macular–papular lesions on mucocutaneous surface Typically appears as dark blue or purple Lesions may present as solitary nodules or plaques and tumors to a size of several centimeters Usually, present in the skin of the lower extremities and progresses very slowly Mucous membranes of the mouth and GI tract and regional lymph nodes could be affected Factors that affect the selection of local therapy are: The extent and location of the lesions The rapidity of clinical change Local therapy includes: Surgery Intra-lesional injection of vinblastine, topical retinoid, radiotherapy, and cryotherapy. Radiotherapy: The treatment option for the multifocal but relatively localized KS.
Endemic African Kaposi’s sarcoma ^[8]	Common features
Usually seen in areas with high prevalence of HHV-8 such as sub-Saharan Africa, called, Kaposi’s sarcoma-associated herpesvirus (KSHV) Seropositivity in Sub-Saharan Africa is >50% If caused by an undiagnosed HIV infection, it can be treated by antiretroviral medications If caused by infection other than HIV, it can be treated by radiotherapy or chemotherapy The limitations of achieving an optimal and affordable therapeutic approach in sub-Saharan Africa is: Resource-limited settings and infrastructure issues such as lack of trained personnel to administer chemotherapy and lack of infusion centers, and the inability to monitor blood work, a high rate of co-morbid tuberculosis Oral agents such as etoposide may have the benefit of easing the administration
African cutaneous Kaposi’s sarcoma
Risk factors (other than HIV) Chronic malaria Chronic parasitic infections Malnutrition
African lymphadenopathic Kaposi’s sarcoma
A very aggressive form Affects very young children, < 10 years of age Not related to HIV Affects the skin and lymph nodes
Iatrogenic (transplant-related) Kaposi’s sarcoma (immunosuppression-associated Kaposi’s sarcoma)^[9]^[10]	The association of KS with immunosuppression is noted in solid organ transplant patients and other patients on chronic immunosuppressive agents Reduction or removal of immunosuppression may not always lead to a resolution of KS Systemic therapy with liposomal doxorubicin should be considered if immunosuppression removal didn’t work or was not possible
(Epidemic) AIDS-associated Kaposi’s sarcoma^[11]^[12]	The most common malignant neoplasm occurring in AIDS patients A highly varied neoplastic disease Characterized by multifocal mucocutaneous lesions, often with lymphatic and visceral involvement The first epidemic occurred in homosexual men in 1981 in NewYork and California. 95% of all AIDS_associated Kaposi’s sarcoma (AIDS-KS) are in homosexual men The remaining 5% occurs in other population who are at risk of AIDS Etiology hypotheses: Endothelial-tumor growth factors Oncogenic expression Genetic predisposition Environmental cofactors Direct virus-like agent unidentified yet All available treatments help control the lesions, none lengthens survival Treatment includes: Localized radiation therapy Cryotherapy Electrocauterization Surgical excision Systemic chemotherapeutic regimens Interferon

References

↑ Uldrick TS, Whitby D (June 2011). “Update on KSHV epidemiology, Kaposi Sarcoma pathogenesis, and treatment of Kaposi Sarcoma”. Cancer Lett. 305 (2): 150–62. doi:10.1016/j.canlet.2011.02.006. PMID 21377267.
↑ Krigel RL, Laubenstein LJ, Muggia FM (June 1983). “Kaposi’s sarcoma: a new staging classification”. Cancer Treat Rep. 67 (6): 531–4. PMID 6861160.
↑ Grayson W, Pantanowitz L (July 2008). “Histological variants of cutaneous Kaposi sarcoma”. Diagn Pathol. 3: 31. doi:10.1186/1746-1596-3-31. PMC 2526984. PMID 18655700.
↑ Vincenzi B, D’Onofrio L, Frezza AM, Grasso RF, Fausti V, Santini D; et al. (2015). “Classic Kaposi Sarcoma: to treat or not to treat?”. BMC Res Notes. 8: 138. doi:10.1186/s13104-015-1076-1. PMC 4395989. PMID 25889316.
↑ Stratigos, John; Potouridou, Irene; Katoulis, Alexander; Hatziolou, Eftichia; Christofidou, Eleftheria; Stratigos, Alexander; Hatzakis, Angelos; Stavrianeas, Nicholas (1997). “Classic Kaposi’s sarcoma in Greece: a clinico-epidemiological profile”. International Journal of Dermatology. 36 (10): 735–740. doi:10.1046/j.1365-4362.1997.00284.x. ISSN 0011-9059.
↑ Landau HJ, Poiesz BJ, Dube S, Bogart JA, Weiner LB, Souid AK (2001). “Classic Kaposi’s sarcoma associated with human herpesvirus 8 infection in a 13-year-old male: a case report”. Clin Cancer Res. 7 (8): 2263–8. PMID 11489800.
↑ Cetin, Bulent; Aktas, Bilge; Bal, Oznur; Algin, Efnan; Akman, Tulay; Koral, Lokman; Kaplan, Mehmet Ali; Demirci, Umut; Uncu, Dogan; Ozet, Ahmet (2018). “Classic Kaposi’s sarcoma: A review of 156 cases”. Dermatologica Sinica. 36 (4): 185–189. doi:10.1016/j.dsi.2018.06.005. ISSN 1027-8117.
↑ Uldrick TS, Whitby D (2011). “Update on KSHV epidemiology, Kaposi Sarcoma pathogenesis, and treatment of Kaposi Sarcoma”. Cancer Lett. 305 (2): 150–62. doi:10.1016/j.canlet.2011.02.006. PMC 3085592. PMID 21377267.
↑ Siegel JH, Janis R, Alper JC, Schutte H, Robbins L, Blaufox MD (1969). “Disseminated visceral Kaposi’s sarcoma. Appearance after human renal homograft operation”. JAMA. 207 (8): 1493–6. PMID 4884743.
↑ Klepp O, Dahl O, Stenwig JT (1978). “Association of Kaposi’s sarcoma and prior immunosuppressive therapy: a 5-year material of Kaposi’s sarcoma in Norway”. Cancer. 42 (6): 2626–30. doi:10.1002/1097-0142(197812)42:6<2626::aid-cncr2820420618>3.0.co;2-7. PMID 728865.
↑ Thomas S, Sindhu CB, Sreekumar S, Sasidharan PK (2011). “AIDS associated Kaposi’s sarcoma”. J Assoc Physicians India. 59: 387–9. PMID 21751599.
↑ Friedman-Kien, Alvin E.; Saltzman, Brian R. (1990). “Clinical manifestations of classical, endemic African, and epidemic AIDS-associated Kaposi’s sarcoma”. Journal of the American Academy of Dermatology. 22 (6): 1237–1250. doi:10.1016/0190-9622(90)70169-I. ISSN 0190-9622.

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[pmid21377267-1] Uldrick TS, Whitby D (June 2011). “Update on KSHV epidemiology, Kaposi Sarcoma pathogenesis, and treatment of Kaposi Sarcoma”. Cancer Lett. 305 (2): 150–62. doi:10.1016/j.canlet.2011.02.006. PMID 21377267.

[pmid6861160-2] Krigel RL, Laubenstein LJ, Muggia FM (June 1983). “Kaposi’s sarcoma: a new staging classification”. Cancer Treat Rep. 67 (6): 531–4. PMID 6861160.

[pmid18655700-3] Grayson W, Pantanowitz L (July 2008). “Histological variants of cutaneous Kaposi sarcoma”. Diagn Pathol. 3: 31. doi:10.1186/1746-1596-3-31. PMC 2526984. PMID 18655700.

[pmid25889316-4] Vincenzi B, D’Onofrio L, Frezza AM, Grasso RF, Fausti V, Santini D; et al. (2015). “Classic Kaposi Sarcoma: to treat or not to treat?”. BMC Res Notes. 8: 138. doi:10.1186/s13104-015-1076-1. PMC 4395989. PMID 25889316.

[StratigosPotouridou1997-5] Stratigos, John; Potouridou, Irene; Katoulis, Alexander; Hatziolou, Eftichia; Christofidou, Eleftheria; Stratigos, Alexander; Hatzakis, Angelos; Stavrianeas, Nicholas (1997). “Classic Kaposi’s sarcoma in Greece: a clinico-epidemiological profile”. International Journal of Dermatology. 36 (10): 735–740. doi:10.1046/j.1365-4362.1997.00284.x. ISSN 0011-9059.

[pmid11489800-6] Landau HJ, Poiesz BJ, Dube S, Bogart JA, Weiner LB, Souid AK (2001). “Classic Kaposi’s sarcoma associated with human herpesvirus 8 infection in a 13-year-old male: a case report”. Clin Cancer Res. 7 (8): 2263–8. PMID 11489800.

[CetinAktas2018-7] Cetin, Bulent; Aktas, Bilge; Bal, Oznur; Algin, Efnan; Akman, Tulay; Koral, Lokman; Kaplan, Mehmet Ali; Demirci, Umut; Uncu, Dogan; Ozet, Ahmet (2018). “Classic Kaposi’s sarcoma: A review of 156 cases”. Dermatologica Sinica. 36 (4): 185–189. doi:10.1016/j.dsi.2018.06.005. ISSN 1027-8117.

[pmid213772672-8] Uldrick TS, Whitby D (2011). “Update on KSHV epidemiology, Kaposi Sarcoma pathogenesis, and treatment of Kaposi Sarcoma”. Cancer Lett. 305 (2): 150–62. doi:10.1016/j.canlet.2011.02.006. PMC 3085592. PMID 21377267.

[pmid4884743-9] Siegel JH, Janis R, Alper JC, Schutte H, Robbins L, Blaufox MD (1969). “Disseminated visceral Kaposi’s sarcoma. Appearance after human renal homograft operation”. JAMA. 207 (8): 1493–6. PMID 4884743.

[pmid728865-10] Klepp O, Dahl O, Stenwig JT (1978). “Association of Kaposi’s sarcoma and prior immunosuppressive therapy: a 5-year material of Kaposi’s sarcoma in Norway”. Cancer. 42 (6): 2626–30. doi:10.1002/1097-0142(197812)42:6<2626::aid-cncr2820420618>3.0.co;2-7. PMID 728865.

[pmid21751599-11] Thomas S, Sindhu CB, Sreekumar S, Sasidharan PK (2011). “AIDS associated Kaposi’s sarcoma”. J Assoc Physicians India. 59: 387–9. PMID 21751599.

[Friedman-KienSaltzman1990-12] Friedman-Kien, Alvin E.; Saltzman, Brian R. (1990). “Clinical manifestations of classical, endemic African, and epidemic AIDS-associated Kaposi’s sarcoma”. Journal of the American Academy of Dermatology. 22 (6): 1237–1250. doi:10.1016/0190-9622(90)70169-I. ISSN 0190-9622.

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