Kaposi Sarcoma
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Introduction[edit | edit source]
Kaposi sarcoma (KS) is a soft tissue tumor that affects immunocompromised people including organ transplant recipients and people with acquired immunodeficiency syndrome (AIDs).[1][2]
Moritz Kaposi, an Austro-Hungarian dermatologist, first published "Idiopathisches multiples Pigmentsarkom der Haut" in 1872. He reported several cases of a multifocal pigmented sarcoma of the skin in elderly European men, all of whom died within a span of two years.[2][3]
Mechanism of Injury / Pathological Process[edit | edit source]
Human herpesvirus/Kaposi sarcoma herpesvirus (HHV-8) was discovered as a causative agent of Kaposi sarcoma as the AIDS epidemic progressed in the 1980s.[1] HHV-8 interferes with many normal cell functions and requires cofactors like cytokines or specific proteins to result in the development of Kaposi sarcoma.[4]
Clinical Presentation[edit | edit source]
There are four forms of KS:[1]
- Classic KS- presents in individuals without HIV infection in older men, typically occurring in elderly men of Mediterranean and Eastern European descent on the lower extremities.
- Endemic KS is found in sub-Saharan Africa and has generalized lymph node involvement in children.
- HIV-related KS - commonly occurring with patients not taking highly active antiretroviral therapy (HAART). Manifestations include diffuse involvement of the skin and internal organs
- Iatrogenic KS - seen in patients treated with immunosuppressive therapy, especially organ transplant recipients. This subtype presents with diffuse involvement of the skin and internal organs.
Diagnostic Procedures[edit | edit source]
Histological Diagnosis[edit | edit source]
Biopsy is required for a definite diagnosis of KS with identical histology across its different epidemiologic forms. The identification and localisation of HHV8 within KS lesional cells using a monoclonal antibody against HHV-8 latent nuclear antigent (LANA) is the most diagnostically helpful immunostaining technique available to differentiate KS from its simulators since it is specific of KS [5]
HHV-8 Diagnostic Tools[edit | edit source]
No other specific HHV-8 tool is routinely used aside from immunohistochemistry for LANA. These diagnostic tools are conducted on an individual basis:
- Serology
- PCR
Outcome Measures[edit | edit source]
add links to outcome measures here (see Outcome Measures Database)
Management / Interventions[edit | edit source]
Local therapies[edit | edit source]
According to European consensus-based interdisciplinary guidelines on the diagnosis and treatment of KS (2019), localized and symptomatic lesions can be treated using local approaches. Currently, no randomized clinical trials are comparing the different local treatment modalities. There are few controlled studies in this area but it is not possible to compare studies, because of the lack of standardized classification systems for disease activity and clinical outcomes.[5]
Local therapies include: [5]
- Radiotherapy - One of the most efficient treatments for all forms of localized KS. Overall response rates range from 47% to 99%[5]
- Surgical excision- Surgical excision is grieved with a high recurrence rate. It should not be used in extensive lesions but can be applied on a few well-defined limited and superficial lesions; however, repeated surgical excisions can cause severe functional impairment
- Cryosurgery and Laser - CO2-Laser and superficial cryotherapy can be temporarily efficient in superficial lesions with 80 to 90% overall response rate. The patient should be informed of the risk of sequelae hypopigmentation
- Local or intralesional chemical or immune modifying agents
Systemic Treatments[edit | edit source]
The KS type, the extent of the disease, the disease course and symptoms will determine the course of treatment. Systemic treatment is mainly for disease control and symptom relief rather than a cure.[5]
The recommended first-line agents in terms of systemic treatment are:[5]
- Pegylated liposomal doxorubicin
- Paclitaxel
Differential Diagnosis
[edit | edit source]
add text here relating to the differential diagnosis of this condition
Resources
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add appropriate resources here
References[edit | edit source]
- ↑ 1.0 1.1 1.2 Bishop BN, Lynch DT. Kaposi sarcoma. InStatPearls [Internet] 2022 Jun 11. StatPearls Publishing.
- ↑ 2.0 2.1 Cesarman E, Damania B, Krown SE, Martin J, Bower M, Whitby D. Kaposi sarcoma. Nature reviews Disease primers. 2019 Jan 31;5(1):9.
- ↑ Kaposi. Idiopathisches multiples pigmentsarkom der haut. Archiv für Dermatologie und Syphilis. 1872 Jun;4:265-73.
- ↑ Mariggiò G, Koch S, Schulz TF. Kaposi sarcoma herpesvirus pathogenesis. Philosophical Transactions of the Royal Society B: Biological Sciences. 2017 Oct 19;372(1732):20160275.
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 Lebbe C, Garbe C, Stratigos AJ, Harwood C, Peris K, Del Marmol V, Malvehy J, Zalaudek I, Hoeller C, Dummer R, Forsea AM. Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC). European Journal of Cancer. 2019 Jun 1;114:117-27.
