Abstract
Simple SummaryKaposi’s sarcoma, a rare opportunistic tumor, is observed in four epidemiological conditions (AIDS-related, iatrogenic, endemic or classic KS). Although in most cases KS is an indolent disease, it can be locally aggressive and/or it can invade other organs than the skin, resulting in more severe presentations, especially in patients with severe immunosuppression. There is no consensus on the imaging workup that is necessary for either the initial staging of the disease or the follow-up. Future perspectives include the use of certain non-invasive imaging tools that may help to evaluate the clinical response to treatment, as well as certain new histological markers that may help in guiding the treatment planning for this atypical neoplasm.Kaposi’s sarcoma (KS) is a rare, atypical malignancy associated with immunosuppression and can be qualified as an opportunistic tumor, which responds to immune modulation or restoration. Four different epidemiological forms have been individualized (AIDS-related, iatrogenic, endemic or classic KS). Although clinical examination is sufficient to diagnose cutaneous lesions of KS, additional explorations are necessary in order to detect lesions involving other organs. New histological markers have been developed in recent years concerning the detection of HHV-8 latent or lytic proteins in the lesions, helping to confirm the diagnosis when it is clinically doubtful. More recently, the evaluation of the local immune response has also been shown to provide some guidance in choosing the appropriate therapeutic option when necessary. We also review the indication and the results of conventional radiological imaging and of non-invasive imaging tools such as 18F-fluoro-deoxy-glucose positron emission tomography, thermography and laser Doppler imaging for the diagnosis of KS and for the follow-up of therapeutic response in patients requiring systemic treatment.
Highlights
Kaposi’ sarcoma (KS) is an atypical tumor that is associated with immunosuppression.Kaposi’s sarcoma (KS) can be qualified as an opportunistic tumor, and it is well demonstrated that in patients with immunodeficiency, the restoration of immunity can be sufficient to cure KS
There are four major epidemiological forms of KS: classic KS (CKS), which mostly affects elderly men of Mediterranean, east European, or Jewish heritage with a peak incidence after the sixth decade of life; endemic African KS, which occurs in sub-Saharan Africa among children and adults; iatrogenic immunosuppressive KS, which affects patients who are receiving chronic immunosuppressive therapy, mostly organtransplant recipients; and Epidemic KS or acquired immunodeficiency syndrome (AIDS)-KS, which affects people living with HIV
Amongst AIDS-KS, a sub-entity of KS has emerged in the form of long-term HIV-aviremic PLHIV
Summary
Kaposi’ sarcoma (KS) is an atypical tumor that is associated with immunosuppression. KS can be qualified as an opportunistic tumor, and it is well demonstrated that in patients with immunodeficiency, the restoration of immunity can be sufficient to cure KS. Depending on the KS lesion localization, additional examinations are very variable, and it is generally necessary to combine several tools. Another challenging aspect is the assessment of the therapeutic response under cART in HIV+ patients and/or under specific treatment (cytotoxic drugs, antiangiogenic drugs, targeted therapies, immunotherapy, etc.). For skin lesions, this response is often difficult to determine, and there are non-invasive imaging options that could help distinguish scarring from lesions that are still active.
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