View source for Kaposi's sarcoma

==Background==
{{Skin anatomy background images}}
*Different classifications of Kaposi’s sarcoma based on patient population
*Associated with [[HHV-8]]
*Most common cancer in [[HIV]]/[[AIDS]] patients
*Kaposi’s Sarcoma can be unmasked or induced by initiation of steroids or HAART
*Cutaneous lower extremity lesions most common presentation
*Kaposi’s sarcoma is an AIDS-defining illness
*Treat with HAART and/or chemo

===History===
*First described by Hungarian Dr. Moritz Kaposi in 1872; rare until 1980s with emergence of HIV/AIDS (1981 cluster of Kaposi’s sarcoma in young men in NYC and California; 1982 listed as AIDS-defining illness by CDC)
*Human Herpesvirus 8 (HHV8)
**Large, double-stranded DNA virus
**Necessary but NOT sufficient to cause disease
**Also associated with body-cavity based lymphomas, multicentric Castleman’s disease (angiofollicular lymph node hyperplasia)

===Classifications===
*Classic Kaposi’s (CK)
**Indolent, chronic form, rarely disseminated
**Skin most commonly effected, lower extremities
**~3:1 Male:Female, Age > 60 years old with Mediterranean, Eastern European or Middle Eastern origin

*Endemic Kaposi’s sarcoma (Africa)
**Male adults or children of both sexes 
**Equatorial/Sub-Saharan Africa
**Aggressive form with lymph node involvement (lymphedema)
**27%, 35%, and 24% of total cancer burden in Uganda, Zimbabwe and Mozambique, respectively

*AIDS-KS
**Developed countries: Men who have sex with men
**Developing countries: Heterosexual men and women (Africa)
**Aggressive disease – most commonly in lungs, gastrointestinal tract, mouth, and genitalia 
**Most common cancer in HIV/AIDS patients

*Iatrogenic due to immunosuppression – Most commonly organ transplant-associated
**Increased risk after solid organ transplantation
**Transplant itself may transmit HHV-8 infection
**Male > Female (3.3:1), average age 43 yo
**Cutaneous lesion presentation most common
**More common in liver than kidney transplant patients

*Immune Reconstitution Inflammatory Syndrome (IRIS) Phenomenon 
**Initiation of retroviral therapy can cause the development of KS
**True unmasked KS-IRIS begins within 6 months of initiating therapy, and the rates remain unclear

==Clinical Features==
[[File:Kaposis sarcoma 01.jpg|thumb|Kaposi sarcoma. Characteristic purple lesions on the nose in an HIV-positive female.]]
[[File:Kaposis Sarcoma Lesions.jpg|thumb|Kaposi's sarcoma skin lesion]]
[[File:Patch stage Kaposi's sarcoma.jpg|thumb|Patch stage Kaposi's sarcoma. Red to brownish irregularly shaped macules and plaques.]]
[[File:Kaposi’s sarcoma intraoral AIDS 072 lores.jpg|thumb|An HIV-positive person presenting with a Kaposi's sarcoma lesion with an overlying candidiasis infection in their mouth.]]
*[[rash|Cutaneous]]
*Heterogeneous appearance 
**Color – dark blue, reddish, purple, violaceous, dark brown/black 
**Location – extremities, most commonly the feet 
**Lesions: macules, plaques, nodules
**Associated [[lymphedema]]
*Pulmonary
**Gradual Onset (2-4 weeks)
**[[Cough]]
**[[Dyspnea]]
**[[Fever]]
**[[Hemoptysis]]
**Asymptomatic
*Gastrointestinal
**[[GI bleed|Bleeding]] 
**[[SBO|Obstruction]]

==Differential Diagnosis==
*Cutaneous Lesions:<ref>Cesarman E, et al. Kaposi sarcoma. Nat Rev Dis Primers. 2019;5(1):9. PMID 30705286</ref>
**Bacillary angiomatosis
**[[Purpura]]
**Hematomas
**Angiomas
**Dermatofibromas
**Nevi
*Pulmonary Lesions in HIV/AIDs:
**Bacterial [[pneumonia]]
**[[PCP pneumonia|PCP]]
**[[TB]]
**[[Fungal infections]]
*Anorectal Lesions in HIV/AIDs:
**Common: [[Anal fissure]], [[perianal abscess|abscess]] and/or fistula, [[hemorrhoid]], [[pilonidal cyst|[pilonidal disease]]
**STDs: [[Gonorrhea]], [[chlamydia]], [[HSV|herpes]], [[chancroid]], [[syphilis]], [[condyloma acuminata]]
**Infectious: [[TB]], [[CMV]] infection, [[actinomyces|actinomycosis]], [[cryptococcosis]]
**Neoplastic: [[Lymphoma]], [[Kaposi’s sarcoma]], [[squamous cell carcinoma]]

{{HIV associated conditions}}
{{DDX dark raised lesions}}

==Evaluation==
*Clinical impression
*Biopsy is gold standard for definitive diagnosis
*WHO Clinical Staging of HIV/AIDS 
**Stage 4: Presumptive Diagnosis of HIV/AIDS can be made from clinical signs of Kaposi’s
*GI manifestations
**Unlikely visible on XR; can be seen on flex sig or colonoscopy
*Pulmonary Manifestations
**Heterogeneous on [[CXR]] - nodular, interstitial and/or alveolar infiltrates, pleural effusion, hilar and/or mediastinal adenopathy, or an isolated nodule

===Staging===
*Classic Kaposi's Sarcoma
**No staging system. It is excluded in TNM Staging of soft tissue sarcomas by American Joint Committee on Cancer (AJCC)

*AIDS-KS Staging
{| {{table}}
| align="center" style="background:#f0f0f0;"|''' '''
| align="center" style="background:#f0f0f0;"|'''Good Risk (all of the following)'''
| align="center" style="background:#f0f0f0;"|'''Poor Risk (any of the following)'''
|-
| Tumor, T||T0: Skin and/or lymph nodes and/or minimal oral disease||TI: Tumor-associated edema or ulceration, extensive oral KS, GI KS, KS in other non-nodal viscera
|-
| Immune System, I||CD4 > 200||CD4 < 200
|-
| Systemic Illness, S||S0: No history of OI or thrust, No "B" Symptoms (fever, night sweats, weight loss, diarrhea > 2 weeks) Karnofsky performance > 70||SI: History of OI and/or thrush, "B" symptoms, Karnofsky performance <70, Other HIV-related illness
|-
|}

==Management==
*Highly Active Anti-retroviral therapy (HAART):
**Can achieve remission in 60-90% remission in stage T0 disease
**Can combine with chemotherapy
*Chemotherapy:
**First-line: Pegylated liposomal doxorubicin (PLD) 20mg/m2 every three weeks (unless cardiac contraindication)
**Second-line: Vinblastine, Bleomycin, Paclitaxel, Etoposide, Gemcitabine
*Cutaneous Specific Treatment:
**Alitretinoin (9-cis-retinoic acid) topical gel 0.1% FDA-approved for cutaneous AIDS-associated KS 
**Intralesion Injection: Vinblastine or Bleomycin or INF-alpha
**Vinblastine 0.2 – 0.3mg/mL solution with a volume of 1 mL per 0.5 cm2 of lesion
**IFNa - FDA approved for AIDS-associated KS
*Radiation Therapy
**30 Gy in 15 daily 2 Gy fractions 
*Other Drug Trials:
**Bevacizumab - an anti-VEGF-A 
**Imatinib - monoclonal antibody tyrosine kinase receptor
*[[Steroids]]
**Associated with generation and exacerbation of KS
**Important implications because of the high use of steroids in this patient population (HIV/AIDS, transplant)
**Once steroids are removed, KS can regress

==See Also==
*[[HIV - AIDS (Main)]]

==External Links==
*http://www.ncbi.nlm.nih.gov/pubmed/25256409
*http://www.ncbi.nlm.nih.gov/pubmed/20413015

==References==
*Farge, D. "Kaposi's Sarcoma in Organ Transplant Recipients. The Collaborative Transplantation Research Group of Ile De France." The European Journal of Medicine 2.6 (1993): 339-43. 
*Fenig, Eyal, Baruch Brenner, Erica Rakowsky, Moshe Lapidoth, Alan Katz, and Aaron Sulkes. "Classic Kaposi Sarcoma." American Journal of Clinical Oncology 21.5 (1998): 498-500. 
*Krown, SE, C. Metroka, and JC Wemz. "Kaposi's Sarcoma in the Acquired Immune Deficiency Syndrome: A Proposal for Uniform Evaluation, Response and Staging Criteria." Journal of Clinical Oncology 7.9 (1989): 1201-207. 
*Krown, SE, MA Testa, and J. Huang. "AIDS-related Kaposi's Sarcoma: Prospective Validation of the AIDS Clinical Trials Group Staging Classification. AIDS Clinical Trials Group Oncology Committee." Journal of Clinical Oncology 15.9 (1997): 3085-092. 
*Moosa, M.r. "Kaposi's Sarcoma in Kidney Transplant Recipients: A 23-year Experience." QJM 98.3 (2005): 205-14. 
*Osawa, R., N. Kato, T. Yanagi, and N. Yamane. "Clearance of Recurrent, Classical Kaposi’s Sarcoma Using Multiple Paclitaxel Treatments." Acta Derm Venereol Acta Dermato-Venereologica 87.5 (2007): 435-36. Web.
*Robey, Rebecca C., and Mark Bower. "Facing up to the Ongoing Challenge of Kaposiʼs Sarcoma." Current Opinion in Infectious Diseases 28.31 (2015).
*Rosen, Peter, and John A. Marx. Rosen's Emergency Medicine: Concepts and Clinical Practice. 8th ed. Philadelphia: Elsevier Saunders, 2014. 
*Safai, B., and R. A. Good. "Kaposi's Sarcoma: A Review and Recent Developments." CA: A Cancer Journal for Clinicians 31.1 (1981): 2-12.

[[Category:Dermatology]]
[[Category:ID]]