Kaposi’s Sarcoma

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Introduction   

Kaposi sarcoma is a soft-tissue tumor caused by an infectious agent that occurs in clients with immunosuppression, particularly those with acquired immunodeficiency syndrome (AIDS) or those undergoing organ transplantation [2, 4]. The disease was first described by a Hungarian dermatologist, Moritz Kaposi, of the University of Vienna, in 1872 when he assessed pigmented cutaneous sarcoma in the lower extremities of a client [5, 10].  

Kaposi sarcoma is an AIDS-defining cancer, as are liver, anal, lung and Hodgkin lymphoma [2, 4]. Male homosexuals who are HIV positive have a fivefold to tenfold increased risk of Kaposi sarcoma, and in HIV clients who are not taking high-activity antiretroviral therapy (HAART), up to 30% will develop Kaposi sarcoma [4, 5].  

Over five percent of clients who undergo an organ transplant, and who subsequently develop cancer for the first time, will develop Kaposi sarcoma at an increased risk of 500-fold over the general population [4, 5]. Clients who undergo solid organ transplantation have a higher risk of developing Kaposi sarcoma than their counterparts who have bone marrow or peripheral blood stem cell transplants [4].  

In the 1980s and 1990s, Kaposi sarcoma was punctuated by a five-year survival rate of roughly 10% and thus was associated with considerable morbidity and mortality [5]. Today, the survival rate ranges from approximately 70% to 95% [5]. The international incidence rate of soft tissue sarcoma ranges from roughly two to five cases per 100,000 individuals per year, with the annual rate of new cases in the United States being 3.4 per 100,000 people [9]. However, Kaposi sarcoma is a major public health concern in Eastern and Central Africa, and the challenge is compounded by the lower socioeconomic status of the region [14]. 

Kaposi sarcoma is a chronic condition marked by periods of relapse that require sustained systemic treatments that can lead to increased risk of toxicities, poor tolerability, and decreased quality of life [5]. Even though the disease is not common, it can cause disfigurement of the skin and death in clients, thus necessitating nursing knowledge of the condition. 

Pathophysiology 

Kaposi sarcoma is a condition of malignant tumors that can arise in any organ system; it is caused by immunosuppression that permits human herpesvirus-8 (HHV-8) to interfere with normal cell function [4,5,9]. However, the specific pathophysiology is unknown since not all clients who present with HHV-8 develop the condition [5]. HHV-8 is a large, double-stranded DNA virus that belongs to the herpesvirus family, and it expresses a protein that is necessary for its replication called latency-associated nuclear antigen (LANA).  

HHV-8 is transmitted via saliva, semen, intravenous drug use, and blood transfusion (Batash, Bishop). It infects cells, causing immune suppression and inflammation that permit the virus to proliferate [4]. Interestingly, the dermal lymphatic alterations associated with impaired immunity that predispose clients to HHV-8 infection can be due to exposure to volcanic soil, iron, aluminosilicate, clay, and some bloodsucking insects [5]. 

Conditions associated with Kaposi sarcoma are plasmablastic multicentric Castleman disease, primary effusion lymphoma, intravascular large B cell lymphoma, angiosarcoma, and inflammatory myofibroblastic tumor [4]. 

Kaposi sarcoma is delineated into four subtypes. These subtypes include [3,5,8,10,14]: 

• Classic—This type was originally described by Kaposi and presents in middle or old age, particularly in immunocompetent men of Mediterranean and Eastern European descent. 
• Endemic—This includes several forms found in individuals from sub-Saharan Africa before the AIDS epidemic. It also occurs in children in Africa, particularly in ages 4 to 9. Endemic Kaposi sarcoma is typically mild in adults, but in children, it is aggressive and can quickly metastasize to the visceral organs. 
• Iatrogenic—This type is associated with immunosuppressive medication therapy, particularly in clients who have undergone organ transplantation, and those who have gastrointestinal inflammatory disease and rheumatologic conditions. Clients who take systemic corticosteroids and chemotherapy agents are also at risk. 
• HIV/AIDS association—This type is linked mainly to homosexual men who are diagnosed with HIV or AIDS. 

A more recent subtype of Kaposi sarcoma has developed due to increased cases of men who have sex with men (MSM) without HIV infection [5,8]. This type differs from classic Kaposi sarcoma because it develops at a younger age and has a milder presentation that does not require systemic treatment [5].  

Risk Factors  

Kaposi sarcoma occurs mainly in male clients of Eastern European and Mediterranean descent who are over 50 years of age [3,8,9]. The median age at diagnosis is 70 years, and men of Italian, Greek, and Jewish heritage are affected more than clients of other cultures [5]. The incidence of the condition has a male-to-female ratio of 17:1 [4]. 

Soft tissue sarcomas like Kaposi sarcomas have several risk factors. These include [3,9]: 

• Contact with salicaceous volcanic soil 
• Bloodsucking insect bites 
• Chronic lymphedema 
• Previous radiation therapy 
• Arsenic, thorium dioxide, and vinyl chloride exposure 
• HHV-8 infection 
• HIV 
• Li-Fraumeni syndrome  
• von Recklinghausen disease  
• Gardner syndrome  
• Nevoid basal cell carcinoma syndrome  
• Tuberous sclerosis  
• Werner syndrome  

Signs and Symptoms 

Kaposi sarcoma is a vascular lesion that forms pinkish purple to reddish blue to brownish-black macules, plaques, and nodules on the skin’s surface or mucocutaneous surfaces [3,8,10]. These lesions can range in size from very small to several centimeters in diameter; they can grow slowly over months to years, or quickly within weeks, in some cases [8].  

The three main skin presentations of Kaposi sarcoma are [4,12]: 

• Patch: Flat, reddish-violaceous, well-defined margins, scaly surface 
• Plaque: Violaceous-brown in color with underlying vascularity, well-defined margins, whitish scales 
• Nodule: Deep purple with underlying active vascularity, rainbow pattern of colors, whitish scales 

Various terms used to describe Kaposi sarcoma growths include patch, plaque, nodule, lymphadenopathic, exophytic, infiltrative, ecchymotic, telangiectatic, keloidal, cavernous, and lymphangioma-like [8]. 

Anatomical distribution of the lesions in adults includes the extremities (45%), intra-abdominal organs (38%), trunk (10%), and head and neck (5%) [9]. The lesions can be painful and cause lymphedema and secondary infection. They can also lead to hemorrhage. Additionally, the lesions can ulcerate and invade nearby tissues, including the lymph nodes, lungs, gastrointestinal system, and other visceral organs [4,5]. When the lungs are infected, respiratory compromise can lead to death [4].  

When the lesions invade the mucous membranes of the mouth and throat, swelling in the hard palate, protruding lips, tumors in the oral cavity, and swallowing difficulties can be present [6]. Oral lesions can also grow on the gums and cause tooth displacement. 

Since Kaposi sarcoma is considered a rare condition, the lesions can be misdiagnosed as arterial insufficiency or venous stasis [3]. Kaposi sarcoma can also be confused as hemangioma, venous lake, glomus tumor, pyogenic granuloma, purpura, nodular melanoma, angiosarcoma, Merkel cell carcinoma, or bacillary angiomatosis [5,12]. 

Signs and symptoms that accompany the dermatologic presentation of lesions in Kaposi sarcoma are unexplained fever, weight loss, and lymphadenopathy [5]. 

Nursing Assessment 

The nurse should take the client’s health history and perform a body systems assessment that emphasizes the skin and mucocutaneous surfaces in clients suspected of or diagnosed with Kaposi sarcoma. Purplish lesions and enlarged lymph nodes should be biopsied and sent to pathology for evaluation [4]. The nurse should also assess the client for fever and weight loss, as well as lesions in the oral mucosa. 

Standard classifications like Tumor, Node, and Metastasis (TNM) are not beneficial for clients with Kaposi sarcoma since the condition typically progresses slowly and most clients present with the skin disease without other symptoms [5]. However, clients with AIDS-related Kaposi sarcoma are staged according to the AIDS Clinical Trials Group (ACTG) system.  

The ACTG staging system is as follows [1,5]: 

Tumor (T) 

• T0: Limited disease involving localized regions of the skin, lymph nodes, or oral mucosa 
• T1: The presence of edema and ulceration that indicates extensive mucosal and visceral disease 

Immune System (I) 

• I0: CD4 cell count greater than, or equal to, 200/µL (normal range, 600–1500/µL) 
• I1: CD4 cell count less than 200/µL 

Systemic Illness (SI) 

• S0: No systemic illness or history of opportunistic infection or thrush 
• S1: Systemic illness or history of opportunistic infection or thrush 

There are some non-standardized proposed staging systems for Kaposi sarcoma, one of which is as follows [5]: 

• Stage I: Greater than 15 cutaneous lesions, or involvement restricted to one bilateral anatomic site and few gut nodules 
• Stage II: Both exophytic destructive lesions and locally infiltrative cutaneous lesions present 
• Stage III: Widespread lymph node involvement with or without skin lesions, but no visceral organ involvement 
• Stage IV: Widespread disease with multiple visceral organ involvement

Another non-standardized staging system includes [1,3,10]: 

• Stage 1: Maculonodules on the lower extremities 
• Stage 2: Plaques and small nodules on the lower extremities 
• Stage 3: Ulcerated multiple angiomatous plaques and nodules on the lower extremities 
• Stage 4: Multiple plaques and angiomatous nodules extending beyond the lower extremities 

Stages 1 and 2 typically have a slow clinical course, while stages 3 and 4 can be aggressive. Stages 3 and 4 involve the viscera and gastrointestinal tract, with the lesions in stage 3 remaining on the limbs, but in stage 4 they progress to the trunk and head. Organ damage can occur during stage 3 when the viscera become involved [1]. 

Pediatric-specific staging of Kaposi sarcoma, called Lilongwe, includes [1]: 

• Stage 1: Mild Kaposi sarcoma with lesions on the skin and mouth 
• Stage 2: Lymphadenopathic Kaposi sarcoma that involves the lymph nodes with facial edema, oral nodular lesions, and lesions across the body 
• Stage 3: Called woody edema Kaposi sarcoma, this stage divides clients into two groups: edema in less than ten percent of the body and edema in greater than ten percent of the body. 
• Stage 4: Disseminated skin and/or visceral Kaposi sarcoma is present. 

Nurses can use digital photography during the initial assessment to establish a baseline and during treatment to track the effectiveness of the therapeutic modalities [5].  

Diagnostics and Treatment 

Diagnosis and treatment of Kaposi sarcoma are considered difficult by many healthcare professionals because there is not a standard set of therapeutic recommendations to support the rarity of the disease since it mainly affects a smaller population, namely HIV-positive clients [5]. All clients suspected of Kaposi sarcoma should be tested for HIV infection, and those with confirmed HIV should undergo CD4 lymphocyte count and plasma HIV viral load testing [5]. 

Additional tests include complete blood count (CBC), serum protein electrophoresis, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and fecal occult blood testing (preferably three samples) to rule out concomitant gastrointestinal involvement. HHV-8 serology helps confirm the diagnosis, but not many laboratories perform this test [5]. However, plasma HHV-8 load testing can be a cost-effective measure for determining which clients should undergo more costly diagnostics, such as computed tomography (CT), positron emission tomography (PET), and biopsy. This testing can also help exclude other related disorders like Castleman’s disease and primary effusion lymphoma. 

Dermatoscopy can reveal lesions with a scaly surface and rainbow pattern where several colors of the rainbow are juxtaposed. Doppler ultrasonography reveals increased vascularity within the well-defined lesions [5,12].  

Core-needle or incisional biopsy is the main diagnostic mode for Kaposi sarcoma. Incisional biopsy is reserved for clients whose previous core-needle biopsies were nondiagnostic or when anatomical constraints prevent the safe performance of a core-needle biopsy [9]. The nurse should plan the initial biopsy with the surgeon, radiation oncologist, and interventional radiologist. The extracted tissues should be evaluated by a pathologist with experience diagnosing sarcomas. 

Imaging is conducted before the biopsy to determine metastases. Plain radiography, CT, magnetic resonance imaging (MRI), PET, and bone scans are used. In recurrent sarcoma, CT and PET imaging have higher sensitivity than contrast-enhanced CT imaging [9]. A whole-body CT scan is used in aggressive cases to determine lymph node, viscera, bone, and liver involvement, which is common. MRI helps delineate soft tissue and bone involvement while PET scans help with whole-body staging and disease response to treatment [5]. 

Clients diagnosed with Kaposi sarcoma should undergo esophagogastroduodenoscopy and otorhinolaryngological evaluation, including flexible endoscopy, chest X-ray, and complete abdominal ultrasound to determine extracutaneous involvement. Endoscopy is the gold standard for diagnosing gastrointestinal lesions since they are often difficult to detect due to their small size and submucosal presentation [5]. Clients with a positive fecal occult blood test should have a colonoscopy performed, and those with clinical lymphadenopathy should undergo ultrasound testing. 

The stage and severity of Kaposi sarcoma determines the treatment method. Treatment also takes into consideration the client’s medical history, physical and psychological characteristics, and comorbidities. The interdisciplinary team that guides treatment should include dermatologists, oncologists, radiation oncologists, infectious disease specialists, mental health professionals, and nurses. Surgery is not the typical treatment for Kaposi sarcoma since it is a systemic disease with multi-site involvement; therefore, surgery is relegated to palliative therapy or cosmetic purposes [3]. 

HAART can delay or prevent HIV from progressing to AIDS and is largely responsible for the drop in the incidence of Kaposi sarcoma [2]. In some cases, clients are treated with a combination of HAART and chemotherapy. Radiation is used to treat early stages and can be used palliatively in late stages to reduce bleeding, edema, and pain. Chemotherapy includes bleomycin, doxorubicin, etoposide, gemcitabine, paclitaxel, vinblastine, vincristine, and vinorelbine treatments. Additionally, chemotherapy is the preferred treatment for children [3,4,5,7,10]. Paclitaxel is effective in treating non-HIV/AIDS-related Kaposi sarcoma as both a first- and second-line treatment option [10,11]. Side effects of chemotherapy include alopecia, anemia, arthralgia, myalgia, chronic fatigue syndrome, onycholysis, nausea and vomiting, leukopenia, neutropenia, hand-feet syndrome, and peripheral neuropathy. 

Vincristine can be injected into a large number of lesions in the same session, making it a cost-friendly treatment that is 95% effective after a single injection [5]. Bleomycin can also be used in this manner, but it is associated with a higher incidence of pain at the injection site compared to vincristine. Interferon alpha-2 can be injected into lesions twice a week and has been shown to reduce lesion size, thickness, and consistency [5].  

Antiangiogenic agents pazopanib, thalidomide, pomalidomide, lenalidomide, bevacizumab, and imatinib interfere with the network of blood vessels that tumors rely on to grow and metastasize [3]. Immunotherapy agents nivolumab and ipilimumab are effective in treating Kaposi sarcoma but can carry severe complications, such as colitis, pneumonia, and lipase elevation. 

Neodymium: YAG (Nd: YAG) laser treatments have an effectiveness rate of 80% in HIV-positive clients with cutaneous localized lesions [5]. Lesion size has been reduced, and elevated lesions flattened due to the Nd: YAG laser [5]. 

Topical therapy is used in clients with mild, localized Kaposi sarcoma and who are not good candidates for more aggressive treatments. Alitretinoin, imiquimod, and timolol creams are used, and alitretinoin is FDA-approved for use in Kaposi sarcoma [3,4,5]. Topical treatments are especially effective in mucosal lesions or when combined with other local modalities [5]. Silver nitrate cauterization and nicotine patches are also used, but with less efficacy [5]. Cryotherapy with liquid nitrogen and carbon dioxide lasers is used to treat superficial, small lesions. Minor side effects include blistering, depigmentation, and scarring of the skin. 

Several new treatment options for Kaposi sarcoma are currently being studied, such as nelfinavir, valganciclovir, selumetinib, intra-lesional nivolumab, pomalidomide with liposomal doxorubicin for HIV-related neoplasms, ipilimumab and nivolumab for advanced HIV-related neoplasms, and recombinant EphB4-HAS fusion protein [4,5,7]. 

Complications and Prognosis 

The larger lesions of Kaposi sarcoma can cause painful edema and disfigurement of the skin while lung involvement can cause respiratory distress and death [4]. The side effects of chemotherapy include cardiac toxicity, neuropathy, neurotoxicity, and infertility, while radiation therapy causes skin dryness, avascularity, impaired wound healing, lymphedema, and additional malignancies. 

Poor prognosis is associated with age older than 60 years, tumors larger than five centimeters, high histological grade of the tumor, advanced pathological stage of the tumor, and positive tumor margins after surgery. Surgery alone is typically sufficient to cure small, low-grade tumors of the extremities or trunk while higher-grade sarcomas with higher local treatment failure rates are associated with increased metastatic potential [9]. Death occurs in 10% to 20% of clients diagnosed with Kaposi sarcoma, but a higher percentage develops fatal secondary malignancy [4]. CD4 counts and opportunistic infections are the most significant contributing factors to prognosis in Kaposi sarcoma, with lung involvement having the lowest positive outcomes [4]. 

Nursing Interventions  

Since Kaposi sarcoma is mainly associated with HIV/AIDS in the United States, the nurse needs to provide client education about the prevention and control of retroviral infection. Suppressing HHV-8 infection might also play a role in controlling Kaposi sarcoma growth, so providing education about HHV-8 is also important [7]. HIV is a preventable disease, and nurses should teach clients effective measures to reduce their risk of infection. 

HIV risk can be decreased by [13]: 

• Using a male or female condom during sex 
• Being tested for HIV and sexually transmitted infections 
• Undergoing a voluntary medical male circumcision 
• Using harm reduction services for people who inject and use illicit drugs 

The nurse should encourage clients with HIV/AIDS to start HAART since it strengthens the immune system. Current HAART does not cure HIV infection, but it does allow the client’s immune system to grow stronger. The nurse should remind the client that HAART must be taken every day for the remainder of the client’s life. 

Nurses can also implement the following interventions: 

• Listen to the client’s concerns and fears and utilize therapeutic communication. 
• Assist the client in a more comfortable position if the lesions are causing pain. 
• Administer pain medication, if indicated. 
• Provide high-calorie, high-protein meals to assist with wound healing. 
• Apply compression stockings to control edema of the lower extremities. Stockings can also decrease the severity of the lesions.  
• Provide rest periods and distraction techniques to promote relaxation. 
• Inspect the client’s skin and mucous membranes every shift. Document new lesions. 
• Monitor for side effects of chemotherapy and radiation. 
• Monitor gastrointestinal and respiratory function. 
• Offer emotional support to the client and their loved ones. 

Conclusion

Kaposi sarcoma is a rare type of skin cancer that is mainly associated with HIV/AIDS. The skin, mucous membranes, and major organs, such as the liver and lungs can be involved. Even though the condition has declined significantly since the 1980s and 1990s, nurses need to recognize the lesions during assessment and implement effective interventions since the condition can be fatal in some cases. 

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