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Vascular tumors

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A 58-year-old Caucasian man comes to the office because of dull abdominal pain and nausea for the past 2 months. The pain is located at the right side of the abdomen, and the patient rates it 4/10 in intensity. The patient has worked as an engineer in a PVC pipe factory for the past 35 years. Medical history is relevant for hypertension, obesity, and recent travel to China. His temperature is 37.0°C (98.6°F), pulse is 80/min, and blood pressure is 135/85 mmHg. Physical examination shows pale skin and conjunctiva, hepatomegaly, and shifting dullness to percussion. Abdominal computed tomography reveals a hypoattenuating, solitary mass with nodular enhancement in the liver. Immunostaining is positive for CD31. Serum alpha-fetoprotein is 8 ng/mL (normal value <10 ng/mL). Serology is negative for hepatitis B and C. Which of the following is the most likely cause for this patient’s presentation?  

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Content Reviewers:

Rishi Desai, MD, MPH

Vascular tumors are tumors of the blood vessels, and there actually a lot of different types, so this is just a quick overview of three types of tumors: Kaposi sarcoma, hemangioma, and angiosarcoma. Kaposi sarcoma is a malignant vascular tumor that is linked with an infection of the human herpesvirus 8 (otherwise known as HHV-8). And it’s a cancer of the blood vessel endothelial cells. This virus is thought to get inside of the cells, and cause the cells to replicate uncontrollably. This type of cancer is seen in people who have suppressed immune systems. That’s why it’s one of the common diseases you’re at risk of getting if you have AIDS, and is a complication of organ transplant patients.

The most common symptoms of Kaposi sarcoma affect the skin, causing purple and red lesions. These lesions look somewhat like a bruise, but unlike a bruise they don’t blanch, or turn pale, when pressed. That’s because a Kaposi sarcoma has blood filled blood vessels whereas bruises are caused by blood leaking outside of blood vessels into the skin. Initially these lesions start off flat, but over time they may become raised and more painful. In people who have a compromised immune systems, the disease can also cause lesions in other tissues like the mouth, the nose, the throat, the lymph nodes, the lungs, and the gastrointestinal tract.

You can sometimes treat affected skin by surgically removing it or freezing it using cryotherapy, however treating the disease in immunocompromised patients is a little more difficult. If someone’s immune system is compromised because of drugs such as corticosteroids, it might be necessary to adjust immunosuppressants and allow the immune system to recover. It’s a lot harder to treat the disease in an AIDS patient whose immune system is severely compromised, so antiretroviral therapy is commonly used by patients to restore immunity. Radiation and chemotherapy are also treatment options.

Summary
Kaposi sarcoma is an angioproliferative disorder that requires infection with human herpes virus 8, also known as Kaposi sarcoma-associated herpes virus, for its development. It is classified into four types based upon the clinical circumstances in which it develops: classic, endemic, iatrogenic and AIDS-associated.
Sources
  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "Kaposi Sarcoma" Archives of Pathology & Laboratory Medicine (2013)
  5. "Hemangioendothelioma" Seminars in Diagnostic Pathology (2013)
  6. "Angiosarcoma: clinical and imaging features from head to toe" The British Journal of Radiology (2017)