Signs and symptoms of Kaposi sarcoma

The first sign of Kaposi sarcoma (KS) is usually the development of skin lesions. Typical lesions begin as flat or slightly raised colored spots. Sometimes they develop under the skin and do not have any unusual coloring. The lesions can develop anywhere on the body, but most often they arise on the legs, nose (especially the tip), the feet, outside parts of the ears, mouth, or genitals. In time the lesions progress to larger nodules that range in color from pink or red to blue, purple, or brown. In dark-skinned people, the lesions may be dark brown or black. Sometimes a greenish "halo" appears around the lesion. Usually the lesions are not tender or sore.

About 1 person in 3 with AIDS-related KS will develop mouth and throat lesions. These are most often on the roof of the mouth (palate) but are also found on the gums, tongue, tonsils, voice box (larynx), or windpipe (trachea). Lesions on the roof of the mouth may not cause any symptoms and may only be noticed at the dentist's office. Sometimes lesions elsewhere in the mouth or throat can cause problems - they may become tender and sore, and they may ooze or bleed. KS lesions in the mouth and throat can make it hard to eat, swallow, or even breathe. Lesions in the mouth can also lead to tooth loss.

KS lesions are often present in the gastrointestinal (GI) tract when the patient is first diagnosed. This is more likely if the immune system is severely suppressed, as it is when the CD4 cell count is low. CD4 cells are a special kind of T-cell (a type of white blood cell that fights infection) whose numbers go down in AIDS patients. Gastrointestinal KS lesions become more common with time. Sometimes lesions can develop in the GI tract even though no lesions are found on the skin. Usually GI lesions do not cause symptoms, but they may slowly bleed. Over time, blood loss can lead to anemia (a low red blood cell count) and fatigue. In some cases GI lesions can cause abdominal pain or discomfort as well as more severe bleeding. The lesions can also make it hard to digest and absorb nutrients from food. This can lead to diarrhea and weight loss. In rare cases, the lesions can completely block the digestive tract, preventing the bowels from moving. This is called a bowel obstruction, and it may require surgery to unblock the intestine.

Some people with KS develop swelling resulting from blockage of the lymph nodes or lymph vessels. This is called lymphedema. This can occur even when there are no (or only a few) lesions visible on the skin. Most often the swelling is seen in the legs or feet, but it can also be present in the groin, the genitals, or the skin around the eyes. Only rarely does the chest or arms swell. When lymphedema in the legs is severe, the swelling can be painful or make it difficult to walk. Sometimes bacterial infections develop in areas with severe lymphedema.

When KS develops in the lungs, it can cause problems like coughing (sometimes with bloody sputum), shortness of breath, and fever.

How is Kaposi sarcoma diagnosed?

Medical history and physical exam

Your doctor will ask about your medical history to learn about any illnesses, operations, your sexual activity, and other possible exposures to Kaposi sarcoma herpesvirus and HIV. The doctor will ask you about symptoms and about any skin tumors you have noticed. The doctor will examine your skin thoroughly and give you a complete physical exam. Sometimes Kaposi sarcoma (KS) lesions develop inside the rectum (the part of the large intestine just inside the anus). A doctor may be able to detect such lesions during an exam with a gloved finger. The doctor may also check the stool for occult blood to see if KS is in the intestines.

Biopsy

To be sure that a lesion is caused by KS, the doctor will need to take a small sample of tissue from the lesion and send it to a lab to be analyzed. This is called a biopsy. Looked at under a microscope, KS cells usually are arranged in a distinctive shape and pattern. Sometimes, though, early lesions may not show the characteristic cell patterns needed to positively diagnose KS.

For skin lesions, the doctor will usually perform a punch biopsy, which removes a tiny round piece of tissue. If the entire lesion is removed, it is called an excisional biopsy.

Chest x-ray

An x-ray of the lungs is often the first test used to see if KS is there. If changes in the lung are seen, it is not possible to determine if they are KS or some other condition. So, additional tests such as bronchoscopy are generally needed to determine if it is KS in the lung. Once it is determined that it is KS, x-rays can be used to watch the patient’s response to treatment.

Bronchoscopy

This procedure lets the doctor look into the lungs. It is often done if the patient has signs or symptoms of KS in the lungs (such as an abnormal finding on a chest x-ray or is coughing up blood). Before starting the bronchoscopy, the patient is put to sleep with a light anesthesia. Then the doctor inserts the bronchoscope (a thin, flexible lighted tube) through the mouth, down the windpipe, and then into the lungs. If the doctor sees a KS lesion, it can be biopsied through the bronchoscope. Bronchoscopy with biopsies can also be used to help diagnose other lung problems, such as pneumonia. Bronchial "washings" are sometimes used instead of, or together,with biopsies. In this procedure, a salt solution is put into an area of the lung, and then removed and tested for infections.

Gastrointestinal endoscopy

This is used when the doctor suspects that KS is in the stomach or intestines and is causing problems.

Three types of endoscopy are used to look at the stomach and intestines.

• Upper endoscopy (also called esophogastroduodenoscopy, or EGD) is used to examine the esophagus, the stomach, and the first part of the small intestine. For this procedure, the patient is first given medicines for sedation. Then, the doctor guides an endoscope (a thin, flexible, lighted tube) through the mouth and esophagus and into the stomach. This allows the doctor to see things like ulcers, infections, and KS lesions. If a lesion is seen, the doctor can use small surgical instruments through the endoscope to biopsy it..

• Colonoscopy is used to look inside the large intestine (colon). The stool in the colon must be cleaned out before the procedure using laxatives and possibly enemas - usually this is done the night before. Just before the procedure, the patient is given a sedative in a vein. Then a colonoscope (a long, flexible, tube with a light or camera on the end) is inserted through the rectum to look at the colon. Any lesion seen can be biopsied.

• Capsule endoscopy is a way to look at the small intestine. It is most often used to look for an area that is bleeding. In this procedure, a person swallows a capsule (about the size of a large vitamin pill) that contains a light source and a very small camera. Like any other pill, the capsule goes through the stomach and into the small intestine. As it travels through the small intestine (usually over the course of about 8 hours), it takes thousands of pictures. These images are transmitted electronically to a device worn around the person's waist, while he or she goes on with normal daily activities. The pictures can then be downloaded onto a computer, where the doctor can look at them as a video. The capsule passes out of the body during a normal bowel movement and is flushed away.

KS can also affect other organs, such as the liver, spleen, heart, or bone marrow. These areas do not often need to be biopsied to know if a patient has KS, as the disease can usually be diagnosed from biopsies of other tissues, such as skin, lungs, or intestines.