Flashcards in Vascular Neoplasms Deck (17):
What is a cavernous hemangioma?
large cavernous benign tumor comprised of blood vessels. Often occur on skin and mucosal surfaces and may bleed.
Look like red-blue spongy lesions with sharply defined margins and large cavernous blood filled spaces.
With what disease is a cavernous hemangioma associated?
Von Hippel-Lindau disease. May occur in cerebellum, brainstem, or retina.
What is a capillary hemangioma?
Closely packed capillaries with connective tissue usually in the skin and subcutaneous tissue and very small.
form well circumscribe lesions.
Most common type of hemangioma and commonly present at birth.
What is a Glomus tumor?
painful vascular tumor found in distal fingers and toes and under nails. Has branching vascular channels.
What is a hemangioendothelioma?
intermediate grade between benign and malignant tumors (can occur if not completely excised). Has discernible vascular channels with well differentiated endothelial cells.
What is an angiosarcoma?
The bad ones. These are malignant proliferations of endothelial cells; highly aggressive, poorly differentiated and form anaplastic spindle cells.
* Associated with VAT= Vinyl chloride, Arsenic, and Thorotrast (older contrast medium)
*Stain for CD 31 marker
Remember to look for high pleomorphism (high N/C ratio), and different sized cells.
How would you describe the blood vessels of an angiosarcoma macroscopically?
Tortuous, irregular, and poorly formed.
What is Kaposi's Sarcoma?
low-grade malignant proliferation of endothelial cells; associated with HHV-8. It presents as a patch, plaque, or nodular stages (purple color) and lymphadenopathy.
In what group is Kaposi's sarcoma classically seen?
older men of Ashkenazi-Jewish or Mediterranean descent
In what group is Kaposi's sarcoma endemic?
African men and children
In what group is Kaposi's sarcoma an epidemic?
HHV-8 associated with AIDS pts.
What will you see histologically with Kaposi's sarcoma?
Hemosiderin (brownish-gold breakdown of hemoglobin), degenerating RBCs, plump endothelial cells, proliferating spindle cells, and defined vascular channels.
What is Von-Hippel-Lindau Disease?
rare autosomal dominant genetic disorder characterized by multiple hemangioblastomas and cysts in the body (especially cerebellum, brain stem and retina), due to deletion of the tumor suppressor (VHL gene on chromosome 3).
For what does Von-Hippel Lindau disease increase your risk?
renal cell carcinoma and pheochromocytoma
What is Osler-Weber-Rendu syndrome?
autosomal dominant genetic disorder that causes dilated and convoluted venules and capillaries of the skin, mucous membrane of the oral cavity, respiratory tract, GI tract, and urinary tract, due to mutation in TGF beta binding proteins. Higher frequency in Utah Mormons.
Normocytic normochromic anemia
How do patients with Osler-Weber-Rendu syndrome present?
with epistaxis (nose bleeds) and GI bleeding