Podcast
Questions and Answers
What is a characteristic finding of classic Kaposi Sarcoma?
What is a characteristic finding of classic Kaposi Sarcoma?
Where are cavernous lymphangiomas most commonly located in children?
Where are cavernous lymphangiomas most commonly located in children?
What is the composition of the stroma in cavernous lymphangiomas?
What is the composition of the stroma in cavernous lymphangiomas?
Which condition is most commonly associated with AIDS-related Kaposi Sarcoma?
Which condition is most commonly associated with AIDS-related Kaposi Sarcoma?
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What type of cells primarily make up the nodules in Kaposi Sarcoma?
What type of cells primarily make up the nodules in Kaposi Sarcoma?
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What is the first stage in the morphology of Kaposi Sarcoma?
What is the first stage in the morphology of Kaposi Sarcoma?
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What is a major cause of aortic aneurysms associated with systemic hypertension?
What is a major cause of aortic aneurysms associated with systemic hypertension?
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Which cells primarily produce MMPs that contribute to the breakdown of the extracellular matrix in inflamed aorta?
Which cells primarily produce MMPs that contribute to the breakdown of the extracellular matrix in inflamed aorta?
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What effect does inflammation have on collagen synthesis in the context of cystic medial degeneration?
What effect does inflammation have on collagen synthesis in the context of cystic medial degeneration?
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What role do TIMPs play in the context of MMP production?
What role do TIMPs play in the context of MMP production?
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What is a result of systemic hypertension affecting arterioles of the vasa vasorum?
What is a result of systemic hypertension affecting arterioles of the vasa vasorum?
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Which cytokines are associated with the Th2 response that drives increased MMP production in macrophages?
Which cytokines are associated with the Th2 response that drives increased MMP production in macrophages?
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What type of aneurysm is commonly associated with atherosclerosis and affects the iliac arteries?
What type of aneurysm is commonly associated with atherosclerosis and affects the iliac arteries?
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Which condition is associated with obliterative endarteritis impacting vasa vasorum of the thoracic aorta?
Which condition is associated with obliterative endarteritis impacting vasa vasorum of the thoracic aorta?
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What is a potential origin of mycotic aneurysms?
What is a potential origin of mycotic aneurysms?
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What is the most common bacterial agent that causes lymphangitis?
What is the most common bacterial agent that causes lymphangitis?
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Which condition is characterized by a collection of lymph fluid in a body part?
Which condition is characterized by a collection of lymph fluid in a body part?
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Which of the following is NOT a cause of secondary lymphedema?
Which of the following is NOT a cause of secondary lymphedema?
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What is a major risk factor for aortic dissections?
What is a major risk factor for aortic dissections?
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What is a notable symptom of lymphangitis?
What is a notable symptom of lymphangitis?
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What characterizes the morphology of aorta in patients with dissections?
What characterizes the morphology of aorta in patients with dissections?
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Which of the following conditions is associated with congenital lymphedema?
Which of the following conditions is associated with congenital lymphedema?
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What usually initiates a spontaneous aortic dissection?
What usually initiates a spontaneous aortic dissection?
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What effect does postradiation fibrosis have on the lymphatic system?
What effect does postradiation fibrosis have on the lymphatic system?
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What type of pain is associated with classical symptoms of aortic dissection?
What type of pain is associated with classical symptoms of aortic dissection?
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What is a common complication associated with Sturge-Weber syndrome?
What is a common complication associated with Sturge-Weber syndrome?
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What is the most common cause of death in aortic dissections?
What is the most common cause of death in aortic dissections?
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What type of lymphatic disorder is lymphadenitis?
What type of lymphatic disorder is lymphadenitis?
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What is a characteristic finding in the aorta of hypertensive patients concerning dissection?
What is a characteristic finding in the aorta of hypertensive patients concerning dissection?
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Which statement accurately describes the histological feature of conditions involving lymphedema?
Which statement accurately describes the histological feature of conditions involving lymphedema?
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What is the effect of aggressive pressure-reducing therapy in the context of dissections?
What is the effect of aggressive pressure-reducing therapy in the context of dissections?
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Which of the following factors can cause secondary lymphedema?
Which of the following factors can cause secondary lymphedema?
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Where do most spontaneous aortic dissections occur?
Where do most spontaneous aortic dissections occur?
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What is notably absent in the inflammation related to aortic dissections?
What is notably absent in the inflammation related to aortic dissections?
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What type of signaling defect can lead to aortic dissections?
What type of signaling defect can lead to aortic dissections?
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What type of mutation is primarily associated with port wine stains in Sturge-Weber Syndrome?
What type of mutation is primarily associated with port wine stains in Sturge-Weber Syndrome?
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Which statement about juvenile hemangiomas is true?
Which statement about juvenile hemangiomas is true?
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Spider telangiectasias are most commonly associated with which condition?
Spider telangiectasias are most commonly associated with which condition?
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What characteristic distinguishes cavernous hemangiomas from capillary hemangiomas?
What characteristic distinguishes cavernous hemangiomas from capillary hemangiomas?
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What genetic inheritance pattern does hereditary hemorrhagic telangiectasias follow?
What genetic inheritance pattern does hereditary hemorrhagic telangiectasias follow?
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Which of the following is a potential complication of hereditary hemorrhagic telangiectasias?
Which of the following is a potential complication of hereditary hemorrhagic telangiectasias?
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Angiomatosis refers to which of the following?
Angiomatosis refers to which of the following?
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Malignant transformation of hemangiomas is:
Malignant transformation of hemangiomas is:
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Which vascular malformation resembles a spider and blanches with pressure?
Which vascular malformation resembles a spider and blanches with pressure?
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Which statement correctly describes juvenile hemangiomas?
Which statement correctly describes juvenile hemangiomas?
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Study Notes
Cystic Medial Degeneration
- TGF-B3 itself can contribute to the degeneration.
- Aneurysms in this area are prone to rupture even at small sizes.
- Atherosclerosis and hypertension are the most important causes of aortic aneurysms.
- Atherosclerosis is most common in abdominal aortic aneurysms (AAA) and common iliac arteries.
- Hypertension is most common in ascending aortic aneurysms.
- Other factors include advanced age, smoking, trauma, vasculitis, congenital defects, dysplasia and berry aneurysms, infections (mycotic aneurysms).
- Mycotic aneurysms can originate from embolization of a septic embolus, extension of an adjacent suppurative process, or circulating organisms directly infecting the arterial wall.
Pathogenesis
- The balance of collagen degradation and synthesis is altered by inflammation and associated proteases.
- Inflammation or atherosclerosis of the aorta leads to inflammatory cells, mainly macrophages, producing MMPs.
- MMPs contribute to the breakdown of extracellular matrix components.
- There is also loss of TIMP, tissue inhibitors of metalloproteinases.
- This may involve Th2 production of cytokines, like IL-4 and IL-10, which drive macrophages to produce increased amounts of MMPs.
- The vascular wall is weakened through loss of smooth muscle cells or the inappropriate synthesis of non-collagenous or non-elastic extracellular matrix (ECM).
- Systemic hypertension can significantly narrow the arterioles of the vasa vasorum leading to outer medial ischemia.
- Tertiary syphilis can cause obliterative endarteritis in the vasa vasorum of the thoracic aorta, leading to medial ischemia, SMC loss, elastic fiber loss, and inadequate or inappropriate ECM production.
- Cystic medial degeneration can be iatrogenic.
Thoracic Aortic Aneurysm
- Hypertension is a major risk factor.
- Aortas have decreased smooth muscle cell and ECM content.
- Medial degenerative changes are seen in hypertensive patients.
- Defective TGF-B signaling can also contribute.
- Aggressive pressure-reducing therapy may be effective in limiting evolving dissection.
Morphology
- Elastic fiber fragmentation and loss is present.
- Mucoid ECM accumulation occurs.
- There is attrition of smooth muscle cells.
- Inflammation is characteristically absent.
- Dissections usually start with an intimal tear.
- Most spontaneous dissections occur in the ascending aorta.
Classical Symptoms of Dissection
- Sudden onset of excruciating pain, usually in the anterior chest, radiating to the back between the scapulae.
- Pain may move downwards as dissection progresses.
Rupture
- Rupture of the dissection is the most common cause of death.
- Rupture can lead to bleeding into the pericardial, pleural, or peritoneal cavities.
Lymphangitis
- Acute inflammation of the lymphatics caused by bacterial infection.
- Group A streptococcus is the most common agent.
- Symptoms include red, painful subcutaneous streaks with painful enlargement of the draining lymph nodes (lymphadenitis).
Lymphedema
- Collection of lymph fluid in a body part.
- Primary lymphedema can be congenital or familial.
- Secondary lymphedema is more common and is caused by tumors, surgical procedures, post-radiation fibrosis, filariasis, post-inflammatory thrombosis, and scarring.
Sturge-Weber Syndrome
- If lesions are distributed according to the trigeminal nerve innervation, it is also known as encephalotrgeminal angiomatosis.
- Characterized by facial port wine nevi.
- Ipsilateral venous angiomas in the cortical leptomeninges are present.
- Associated with intellectual disability, seizures, hemiplegia, and skull radiopacities.
- Can also occur in the liver, spleen, and kidneys.
Hemangiomas
- Tumors composed of blood-filled vessels.
- Most are present from birth and initially increase in size, but regress spontaneously.
- Angiomatosis is a more extensive and diffuse form of hemangiomas.
Capillary Hemangiomas
- Histologically, they are thin-walled capillaries with scant stroma.
Juvenile Hemangiomas
- Extremely common and can be multiple.
- Completely regress by age 7 in most cases.
Spider Telangiectasias
- Nonneoplastic vascular malformations that resemble a spider.
- Associated with hyperestrogenic states, such as pregnancy or patients with cirrhosis.
Cavernous Hemangiomas
- Large, dilated vascular channels.
- More infiltrative and frequently involve deep structures.
- Do not spontaneously regress.
- Unencapsulated and composed of large, cavernous blood-filled vascular spaces.
- Can be seen in von Hippel-Lindau disease.
Hereditary Hemorrhagic Telangiectasias
- Also known as Osler-Weber-Rendu disease.
- Autosomal dominant inheritance.
- Mutations in genes encoding components of the TGF-B signaling pathway of endothelial cells.
- Lesions can spontaneously rupture and may lead to serious epistaxis, gastrointestinal bleeding, and hematuria.
Glomus Tumor (Glomangioma)
- Exquisitely painful tumors.
- Arise from modified smooth muscle cells of the glomus bodies, which are arteriovenous structures involved in thermoregulation.
- Commonly found in the distal portion of digits, under the fingernails.
- Excision is curative.
Kaposi Sarcoma
- Solid, vascular tumors that are almost always benign.
- Classified as endothelial tumors by the presence of endothelial markers or the absence of erythrocytes, including VEGFR-3 and LYVE-1.
- Can occur in the skin, mouth, lungs, and lymph nodes, and can be acquired or inherited.
Classic KS
- Not associated with HIV infection.
- Involves multiple red-purple skin plaques or nodules, usually in the distal lower extremities.
- Tumors are asymptomatic and remain localized.
Transplant-Associated KS
- Occurs in those who have had a solid organ transplant with T-cell immunosuppression.
AIDS-Associated (Epidemic) KS
- Most common HIV-related malignancy.
- Often involves lymph nodes and disseminates widely to viscera.
- Morphology progresses through patches, raised plaques, and nodules.
Patches
- Red-purple macules.
- Histologically, they show dilated, irregular endothelial cell-lined vascular spaces with interspersed lymphocytes, plasma cells, and macrophages.
Raised Plaques
- Dermal accumulations of dilated, jagged vascular channels, lined and surrounded by plump spindle cells.
Nodules
- Sheets of plump, proliferating spindle cells, mostly in the dermis or subcutaneous tissues.
- This stage often heralds lymph node and visceral involvement.
Cavernous Lymphangiomas (Cystic Hygromas)
- Typically on the neck or axilla of children.
- Rarely in the retroperitoneum, but when they do occur there, they tend to be large.
- Massively dilated lymphatic spaces, lined by endothelial cells and separated by connective tissue stroma containing lymphoid aggregates.
- Cavernous lymphangiomas in the neck are common in Turner Syndrome.
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Description
Test your knowledge on cystic medial degeneration and its connection to aortic aneurysms. This quiz covers key factors contributing to degeneration, including TGF-B3, atherosclerosis, and hypertension. Explore the pathogenesis and various factors influencing aneurysm formation.
