A multifocal, vascular, malignant tumor derived from endothelial cells, that is often first apparent in the skin or mucous membranes, but may involve the viscera. It was once a rare disease seen in elderly men, but in recent years the incidence of Kaposi's sarcoma has risen dramatically along with the incidence of acquired immunodeficiency syndrome (AIDS) (see Diagnostic Code: 6351). Currently, it is the most common AIDS-related tumor.

The exact cause of Kaposi's sarcoma is unknown, but the disease may be due to immunosuppression. Genetic or hereditary predisposition is also suspected. Kaposi's sarcoma may be associated with other types of cancer, including leukemia or lymphoma, and in patients with acquired immunodeficiency syndrome (AIDS). Recent studies have shown that it may be caused by a new strain of the human herpes virus type 8 (HHV8). Kaposi's sarcoma may appear in three forms:

• aggressive which occurs both internally and externally, and occurs with AIDS;

• lymphadenopathic which is wide-spread and aggressive, involving lymph nodes, viscera, and occasionally the gastrointestinal (GI) tract; and

• indolent which causes dark tumors due to blood vessel involvement.

The tumors of the indolent form may spread to the hands and arms, may produce fungus-like growths, or both; and may also penetrate tissue and invade bone, lymph nodes, and internal organs.

Kaposi's sarcoma causes structural and functional damage. The condition is characterized by obvious, colorful lesions. When associated with AIDS, it progresses aggressively, involving the lymph nodes, the viscera, and possibly the GI tract. The early lesions, or nodules, are barely elevated purple, pink, or red papules, or round to oval brown or purple plaques that appear on the upper body or mucosa, and look like a birthmark (hemangioma). Older lesions become a brown to a black color, are flatter, and occur with scarring of the skin. In advanced disease, the lesions may fuse together becoming one large plaque. If the lesions are untreated, they may appear as large, ulcerative masses. The patient may exhibit shortness of breath, especially if there is pulmonary involvement present. Ausculatation of the lungs may reveal wheezing and decreased rate and depth of breathing. Respiratory distress may result from bronchial obstruction. Kaposi's sarcoma may also involve the mucosa of the GI tract, causing bleeding and upper GI distress.

Diagnosis requires a physical examination. Usually, the patient will undergo a biopsy of suspected tissue to determine the type of lesion and stage. A computed tomography (CT) scan may be performed to look for metastasis. Upper endoscopy may also be needed to identify lesions in the GI tract causing bleeding.

After a diagnosis of Kaposi's sarcoma has been confirmed, treatment may include

• electron beam radiotherapy;

• cryotherapy;

• electrocoagulation;

• chemotherapy; or

• x-ray therapy for lesions that are not responding to other treatments.

Radiation therapy may offer relief of symptoms, including pain from obstructing lesions in the oral cavity or extremities, and edema caused by lymphatic blockage.

Death due to Kaposi's sarcoma usually results from lung involvement. The treatment of Kaposi's sarcoma in AIDS patients does not prolong life because infections dominate the outcome of the condition due to immunosuppression.

• Consider service connection on a presumptive basis as a chronic condition (38 CFR 3.307; §3.309(a)).

• Consider service connection on a presumptive basis as a condition associated with ionizing radiation exposure (38 CFR 3.307; §3.311).

• May be entitled to special monthly compensation where the veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Total plus 60% – Special Monthly Compensation (SMC).

• For cancers with a high mortality rate, do not schedule a review examination unless the record clearly shows a long-term and stable remission.

• When Regional Offices (ROs) receive a claim identifying exposure to radiation, the existence of a radiogenic disease must be confirmed prior to transferring the claim to the Jackson Regional Office. The rating activity sends claims to Compensation Service for additional development only if there requirements under 38 CFR 3.311(b)(1) are met, and development is complete. 

• The American Cancer Society endorses the use of the Lugano classification, which is important when determining the grade, extent, and whether cancer has metastasized. It is also a factor when deciding whether a future exam is required or whether a total and permanent disability exists in accordance with 38 CFR 3.340 and 38 CFR 4.15.

• SC on a presumptive basis for cancer associated with an in-service exposure event may only be established if the cancer in consideration is the primary or originating site of the cancer. Most pathology reports will indicate if the cancer is primary or metastatic

• If SC is established for a primary cancer that metastasizes into a separate body system, then SC on a secondary basis should be established for the metastasized cancer in the separate body system. If doubt arises as to the medical cause or association of any symptoms or diagnosis, a medical opinion should be requested for clarification.

• A 100 percent evaluation shall continue beyond the cessation of any surgical therapy, radiation therapy, antineoplastic chemotherapy, or other therapeutic procedures. Six months after discontinuance of such treatment, the appropriate disability rating shall be determined by mandatory VA examination. Any reduction in evaluation based upon that or any subsequent examination shall be subject to the provisions of 38 CFR 3.105(e). If there has been no local recurrence or metastasis, rate on residuals under the appropriate diagnostic code(s).