Acute Appendicitis Secondary to Kaposi’s Sarcoma - An Overview

Introduction:

Kaposi’s sarcoma is a cancer that develops in HIV (human immunodeficiency virus) patients. Its presence indicates that the person is immunocompromised (weakened immunity) and requires regular consumption of antiretroviral medications prescribed by the doctor. These patients develop conditions such as acute appendicitis due to gastrointestinal involvement caused by Kaposi's sarcoma. Hence, the timely identification of the condition by a proper diagnostic study and appropriate treatment can help provide better patient survival.

What Is Kaposi’s Sarcoma?

Kaposi’s sarcoma is a viral malignant (cancerous) condition caused by the HHV-8 (human herpesvirus-8). These viruses are rare, and even if they are present within the body, they do not cause many manifestations. The virus is usually triggered by a weak immune system, like in HIV-positive patients. They cause lesions on the skin, mucous membranes, lymph nodes, and internal organs. They also affect the gastrointestinal and respiratory tract. Gastrointestinal involvement causes abdominal pain in about 11 % of the patients. Among the various causes of abdominal pain, the most common is acute appendicitis which accounts for 30 %.

What Are the Clinical Manifestations of Gastrointestinal Involvement in Kaposi’s Sarcoma?

Kaposi’s sarcoma is the most frequent cause of gastrointestinal malignancy in patients with AIDS (acquired immunodeficiency syndrome). It constitutes 40 % of the cases, and sometimes they show no signs or symptoms. If the symptoms are present, they include the following:

• Presence of extensive bleeding in the oral cavity, esophagus (food pipe), stomach, and large intestine.

• Pain in the abdominal region.

• Weight loss.

• Nausea and vomiting.

• Diarrhea.

• Malabsorption.

• Bowel obstruction.

• Bowel perforation.

All of the above symptoms do not appear at the same time. Some of them appear at the initial stages, and others, like bowel obstruction, may happen later. Any changes noticed must be consulted with the doctor and get treated as soon as possible.

What Is Acute Appendicitis?

The appendix is a finger-like small tube about two to four inches long attached to the large intestine in the lower right abdomen. It does not have a specific role in digestion but may indirectly affect the process. The inflammation of the appendix is called appendicitis. The inflammation can be acute or chronic in nature. When appendicitis is left untreated, it may burst and lead to spillage of the bacterial contents into the abdominal cavity causing severe outcomes.

What Are the Symptoms of Acute Appendicitis?

The symptoms of appendicitis are initially mild. It starts gradually as a discomfort or pain in the lower abdominal region near the belly button and spreads to the lower right part of the abdomen. The pain is sudden, severe, aggravated by moving or coughing, and the intensity increases so much that the patient’s sleep is disturbed. The following are the few common symptoms of appendicitis:

• Nausea, vomiting, diarrhea, or constipation.

• Indigestion.

• Loss of appetite.

• Abdominal swelling.

• Abdominal pain.

• Persistent fever.

• Difficulty in passing gas.

How Does Acute Appendicitis Occur in Patients With Kaposi’s Sarcoma?

Kaposi’s sarcoma causes gastrointestinal malignancy in patients with HIV-AIDS. About 50 % of cases have been reported for Kaposi’s sarcoma of the gastrointestinal tract. These patients usually develop no symptoms at all initially. The cause by which the malignancy of the gastrointestinal tract due to Kaposi’s sarcoma develops is not known. It is a rare manifestation, but when it is not noticed and left untreated for a long time, the symptoms become severe and turn fatal for the patient.

What Are the Investigations to Be Performed for Gastrointestinal Kaposi’s Sarcoma?

The doctor first performs a complete medical assessment followed by laboratory and imaging tests to diagnose and confirm the presence of the gastrointestinal manifestations of Kaposi’s sarcoma.

• Medical History: A thorough medical history regarding the signs and symptoms, past medical conditions, underlying medical illnesses, and allergy must be noted. The doctor must get details about the history, diarrhea, and prior infections in the body. Patients who know about their HIV status must be asked about the last test performed to know the CD4 levels, which help determine the grade of immunosuppression. This is vital in estimating the risks and benefits of surgery in addition to the possibility of opportunistic infections, which may not present until CD4 counts are less than 200 cells per millimeter.

• Physical Examination: A physical examination of the abdomen is conducted to detect any tenderness, swelling, enlargement of lymph nodes, or any other signs in the lower right abdominal region that may indicate appendicitis. Kaposi’s sarcoma usually presents with skin lesions. So, the presence of any skin lesions is also noted down.

• Laboratory Tests: These include a complete blood count and differential white blood count to know the body's level of white blood cells. A CD4 count is also taken to know their current level as it is a measure to indicate the treatment prognosis in HIV patients.

The microbiological tests to assess the serum urea and electrolytes, amylase, liver function tests, and urinalysis are also performed.

• Imaging Tests: This includes an abdomen and chest x-ray. The x-ray of the abdomen shows extraluminal gas, indicating bowel perforation, or dilated loops of bowel, related to bowel obstruction. But, the accuracy of the plain x-ray is low, so a CT (computed tomography) scan of the abdomen is performed to have a more detailed view. The bowel necrosis (death of cells and tissue) and fluid collection within the abdomen can be visualized using a CT scan. Another technique that can be helpful is ultrasound imaging.

• Endoscopy: An upper endoscopy can be performed to examine the esophagus, stomach, and small intestine. An endoscope is a long, thin, flexible tube that has a camera at one end to visualize the objects inside the abdomen from the outside.

• Biopsy: Lastly, a biopsy (a sample of affected tissue is collected to examine under a microscope) is performed to confirm the presence of acute appendicitis with Kaposi’s sarcoma.

What Is the Treatment for Acute Appendicitis Secondary to Kaposi’s Sarcoma?

• The treatment is started with medications such as antibiotics to clear the bacterial flora due to appendicitis.

• The open-surgery or laparoscopy technique is considered the two main treatment modalities for these patients.

• In laparoscopy, the surgeon inserts a long thin tube into the abdomen through small incisions. It is a minimally invasive surgery that helps treat appendicitis without much discomfort. But, laparoscopy is not indicated for all patients. Only those with minimal damage may get relief from this technique.

• In open surgery, the removal of the appendix is performed. Since the appendix has no important role in the body, removing it can at least relieve the symptoms.

It is up to the doctor and the patient to discuss the condition and the severity of the disease and provide the appropriate treatment.

Conclusion:

Acute appendicitis is a rare but symptomatic finding in patients with Kaposi’s sarcoma. About half of the HIV-positive individuals may develop gastrointestinal symptoms due to Kaposi’s sarcoma. The doctor must be informed at the earliest to treat them minimally invasively. If left untreated, the condition might worsen, the doctor shifts to open surgery, or the condition may also turn fatal.