eMedicine Specialties > Oncology > Sarcomas of Soft Tissue and Bone
Kaposi Sarcoma: Differential Diagnoses & Workup
Updated: Aug 19, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Differential Diagnoses
Other Problems to Be Considered
Bacillary angiomatosis: This entity is often difficult to distinguish clinically from Kaposi sarcoma. It is caused by Rochalimaea species, a slow-growing, fastidious, gram-negative bacillus that is readily treated with antibiotics. Bacillary angiomatosis lesions typically possess capillary proliferation and neutrophilic inflammation. In contrast, Kaposi sarcoma lesions display slitlike vascular spaces containing lymphoplasmacytic infiltrates. A skin biopsy is required to establish diagnosis.
Hematoma
Hemangioma
Dermatofibroma
Pyogenic granuloma
Purpura
Workup
Laboratory Studies
CD4 lymphocyte counts and plasma HIV viral-load studies should be performed for patients with HIV infection.
Imaging Studies
- Chest radiographs: Radiographic findings in patients with Kaposi sarcoma (Kaposi's sarcoma, KS) are variable and nonspecific. They may include diffuse reticulonodular infiltrates, interstitial infiltrates, pleural effusions, hilar or mediastinal lymphadenopathy, or an isolated pulmonary nodule.
- Thallium or gallium scans: These studies may help to differentiate pulmonary Kaposi sarcoma from infection. Pulmonary Kaposi sarcoma lesions typically demonstrate intense thallium uptake and no gallium uptake, whereas infection is often gallium avid and thallium negative.
- This has become less of an issue in the era of HAART. Prior to that, with the decreasing incidence of Pneumocystis carinii pneumonia (PCP), there had been an increased incidence of tuberculosis (TB), Mycobacterium avium-intracellulare (MAI), Kaposi sarcoma, and malignant lymphoma.
- In a study performed in the mid 1990s, thallium-positive and gallium-negative pattern of scanning had a sensitivity of 63%, specificity of 95%, positive predictive value of 92%, and negative predictive value of 75%.40 Presently, this type of scanning has little clinical relevance, and diagnosis must be suspected on more clinical grounds.
Other Tests
Prevention
- Primary Kaposi sarcoma
- Prophylaxis against exposure to the etiology agents of HIV and HHV-8 is the most effective methods of preventing Kaposi sarcoma. Sexual abstinence, monogamy, condom use, avoidance of IV drug use, and use of sterile needles and syringes are the best methods.
- Similar measures are recommended for those who are already infected with HIV to prevent infecting others and to avoid co-infection with other sexually transmitted and hematogenous infections.
- Secondary Kaposi sarcoma
- The use of HAART decreases risk of Kaposi sarcoma in patients already infected with HIV and may be equally efficacious in treating Kaposi sarcoma.33
- As of 2003, an estimated 6 million people in developing nations were urgently in need of HAART. Major efforts are underway to provide such antiretroviral treatment to these people.41
Procedures
- Punch biopsy: A punch biopsy of the skin or an endoscopic, pleural, or transbronchial biopsy is necessary to establish a definitive diagnosis.
- Bronchoscopy: Pulmonary involvement is typically characterized by a slightly raised (submucosal) cherry-red lesion. Biopsy is generally avoided due to the risk of bleeding.
- Esophagogastroduodenoscopy (EGD) or colonoscopy: Gastrointestinal lesions are frequently observed. The yield of endoscopic biopsy may be low secondary to the submucosal location of many lesions.
Histologic Findings
Typical histologic findings include proliferation of spindle cells, prominent slitlike vascular spaces, and extravasated red blood cells.
Staging
Kaposi sarcoma does not lend itself to conventional tumor, node, metastases (TNM) classification. It should be individualized and customized for the type of Kaposi sarcoma. Patients with classic (sporadic) Kaposi sarcoma tend to have indolent disease and should not be subjected to extensive evaluation by imaging. A good physical examination, routine laboratories, and confirmatory biopsy may be all that is needed. If there is clinical suspicion for systemic disease, then appropriate imaging and even bronchoscopic or endoscopic procedures may be indicated.
Although no staging system has been accepted, several have been proposed. Some have included laboratory parameters as well as clinical features. One must remember that most patients with epidemic Kaposi sarcoma, the most common form of the disease in the United States, do not die of the disease. Therefore, factors other than tumor burden may have a role to play in survival. Because of this, the AIDS Clinical Trials Group (ACTG) has proposed a system based on immune status, extent of tumor involvement, and presence of systemic illnesses. The staging system has not been evaluated prospectively; however, some evidence suggests that it may have prognostic significance.
- ACTG Kaposi sarcoma staging classification42,1,43
- Good risk
- Tumor (T) - Confined to skin and/or lymph nodes and/or minimal nonnodular oral disease limited to the palate
- Immune system (I) - CD4 greater than 150/mm3
- Systemic illness (S) - No history of opportunistic infection, thrush, unexplained fevers, more than 10% involuntary weight loss, or diarrhea persisting more than 2 weeks
- Karnofsky performance status greater than 70
- Poor risk
- Tumor (T) - Tumor-associated edema or ulceration, extensive oral Kaposi sarcoma, gastrointestinal Kaposi sarcoma, Kaposi sarcoma in other non-nodal viscera
- Immune system (I) - CD4 less than 150/mm3
- Systemic illness (S) - History of opportunistic infection, thrush, unexplained fever, night sweats, more than 10% involuntary weight loss, or other HIV-related illness (ie, lymphoma, neurologic disease)
- Karnofsky performance status less than 70
- Other HIV-related illness (concurrent malignancy such as lymphoma or neurologic disease)
- Good risk is shown with a 0 subscript and poor risk with a 1 subscript. For example, T0, S0, and I0 are good risk, and T1, S1, and I1 are poor risk. In the pre-HAART era, any category with poor risk designated the patient as an overall poor risk. One must remember that previous studies were conducted in the pre-HAART era. HAART has had a clear impact on survival by pushing many patients into the good risk category. Now it appears as though CD4 levels do not carry the same prognostic significance. Two broad categories have been defined, good risk (T0 S0, T1 S0, and T0 S1) and poor risk (T1 S1) in HAART responders. The original TIS classification may instead be more applicable to multidrug-resistant HIV.
- Pre-HAART date, confirm the use of the original TIS staging system. Analysis of 294 patients placed on ACTG clinical trials for Kaposi sarcoma in the period from 1989 to 1995 confirmed that each of the variables of tumor, immune system, and systemic illness were independently associated with survival.44 Immune system impairment is the single most important predictor of survival by multivariate analysis. Three adverse prognostic factors for survival include prior or coexistent opportunistic infection (OI), the presence of B symptoms (weight loss, fever, and night sweats), and an absolute CD4 count of less than 300/m L. The most important appears to be opportunistic infection (OI), with a survival of only 7 months versus 20 months without an OI.45
- An accurate assessment of response to treatment for Kaposi sarcoma is often difficult because the lesions may not be amenable to conventional bidimensional tumor measurements.
- Standard response evaluation criteria in solid tumors (RECIST) are not very useful for evaluating treatment effect in Kaposi sarcoma. New Kaposi sarcoma response criteria, using an assessment of the impact of treatment on tumor-related symptoms, are currently being evaluated prospectively.
- ACTG response criteria establish a cutaneous lesion response as a 50% decrease in total body lesion count and a flattening of 50% of previously raised lesions. Evaluation of visceral and oral Kaposi sarcoma is more difficult because lesions in this type do not lend themselves to measurement by either imaging or physical assessment.
More on Kaposi Sarcoma |
| Overview: Kaposi Sarcoma |
Differential Diagnoses & Workup: Kaposi Sarcoma |
| Treatment & Medication: Kaposi Sarcoma |
| Follow-up: Kaposi Sarcoma |
| Multimedia: Kaposi Sarcoma |
| References |
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Further Reading
Keywords
Kaposi's sarcoma, KS, Kaposi tumor, Kaposi's tumor, Kaposi malignancy, Kaposi's malignancy, HIV, AIDS, human immunodeficiency virus, acquired immune deficiency syndrome, epidemic AIDS-related Kaposi sarcoma, immunocompromised Kaposi sarcoma, classic Kaposi sarcoma, endemic African Kaposi sarcoma
Differential Diagnoses & Workup: Kaposi Sarcoma