Pathology of Vascular and Cardiac Tumors

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Questions and Answers

What is the primary characteristic of localized vascular ectasia?

  • It involutes spontaneously.
  • It is a neoplasm.
  • It involves dilation of preexisting blood vessels. (correct)
  • It is a form of cancer.

Which condition is a type of nevus flammeus known as a port-wine stain?

  • A type of spider telangiectasia.
  • A benign tumor.
  • A hereditary hemorrhagic telangiectasia.
  • A congenital capillary malformation. (correct)

What is the typical location for spider telangiectasia to appear?

  • Palms and soles.
  • Lower extremities.
  • Face, neck, and upper chest. (correct)
  • Scalp and abdomen.

What is a common etiology for spider telangiectasia?

<p>Hyperestrinism in pregnancy. (B)</p> Signup and view all the answers

Which type of nevus flammeus usually regresses spontaneously?

<p>Stork bite. (A)</p> Signup and view all the answers

What happens to the tentacles of spider telangiectasia when pressure is applied to the central core?

<p>They disappear. (A)</p> Signup and view all the answers

What distinguishes generalized hereditary hemorrhagic telangiectasia from localized types?

<p>It typically appears as multiple lesions. (B)</p> Signup and view all the answers

Which statement about nevus flammeus is true?

<p>Port wine stains are always red and rough. (B)</p> Signup and view all the answers

Which virus is primarily associated with the etiology of Kaposi Sarcoma?

<p>HHV-8 (D)</p> Signup and view all the answers

What is a characteristic gross appearance of a Kaposi Sarcoma skin lesion?

<p>Pink-red-purple patch that progresses to nodules (A)</p> Signup and view all the answers

Which variant of Kaposi Sarcoma is associated with a dramatic decline in incidence due to antiretroviral treatment?

<p>AIDS-associated KS (C)</p> Signup and view all the answers

What type of cells are predominant in the histology of Kaposi Sarcoma?

<p>Pleomorphic spindle-shaped cells (C)</p> Signup and view all the answers

What is the primary route of transmission for Kaposi Sarcoma?

<p>Sexual intercourse and blood transfusion (B)</p> Signup and view all the answers

Which clinical behavior is NOT typically associated with Kaposi Sarcoma?

<p>Always causes severe pain (D)</p> Signup and view all the answers

What does the histological analysis of Kaposi Sarcoma commonly reveal in the interstitium?

<p>Rows of RBCs, hemosiderin, and macrophages (A)</p> Signup and view all the answers

Which statement regarding Kaposi Sarcoma is accurate?

<p>It may invade and metastasize despite good treatment response. (A)</p> Signup and view all the answers

What is the primary constitutional symptom associated with cardiac myxoma due to IL-6 production?

<p>Fever (C)</p> Signup and view all the answers

What complication can arise from cardiac myxoma that involves the brain?

<p>Systemic embolization with brain infarcts (D)</p> Signup and view all the answers

Which genetic condition is commonly associated with cardiac rhabdomyoma?

<p>Tuberous sclerosis complex (D)</p> Signup and view all the answers

What is the typical histological appearance of cardiac rhabdomyoma?

<p>Spider cells laden with glycogen granules (D)</p> Signup and view all the answers

What is a common feature of cardiac myxoma presentation?

<p>Valvular obstruction (C)</p> Signup and view all the answers

In what location are cardiac rhabdomyomas usually found?

<p>Ventricular wall (A)</p> Signup and view all the answers

Which of the following best describes cardiac myxoma?

<p>Benign myocardial lesion associated with systemic inflammatory response (A)</p> Signup and view all the answers

What is the prognosis for cardiac rhabdomyoma?

<p>Typically resolves without treatment (B)</p> Signup and view all the answers

Which of the following is classified as a benign cardiac tumor?

<p>Rhabdomyoma (C)</p> Signup and view all the answers

What condition is classified as a tumor-like vascular condition?

<p>Vascular ectasia (A)</p> Signup and view all the answers

Which vascular tumor is associated with intermediate malignancy?

<p>Kaposi sarcoma (A)</p> Signup and view all the answers

Which of the following tumors is considered malignant?

<p>Lymphangiosarcoma (A)</p> Signup and view all the answers

Which benign vascular tumor primarily affects capillary structures?

<p>Hemangioma (C)</p> Signup and view all the answers

What is a common feature of metastatic tumors in the context of cardiac pathology?

<p>They can arise from various organ systems (D)</p> Signup and view all the answers

Which of these characteristics can vary among vascular and cardiac tumors?

<p>Predisposing factors (B), Age at onset (C)</p> Signup and view all the answers

What is a known treatment approach for benign cardiac tumors?

<p>Surgical excision (C)</p> Signup and view all the answers

What type of infection primarily causes bacillary angiomatosis in immunocompromised hosts?

<p>Bartonella species (B)</p> Signup and view all the answers

Which of the following best describes the nature of bacillary angiomatosis?

<p>A focal vascular proliferation (C)</p> Signup and view all the answers

What is the primary method for identifying Bartonella in cases of bacillary angiomatosis?

<p>Wartin-Starry (silver) stain (B)</p> Signup and view all the answers

Which of the following statements about hemangiomas is correct?

<p>Juvenile hemangiomas often regress spontaneously by age 7. (A)</p> Signup and view all the answers

What is the most common affected location for hemangiomas?

<p>Skin (A)</p> Signup and view all the answers

Which condition is characterized by inflamed hemangioma?

<p>Pyogenic granuloma (A)</p> Signup and view all the answers

Which Bartonella species is responsible for cat-scratch disease?

<p>B.henselae (C)</p> Signup and view all the answers

In the pathogenesis of bacillary angiomatosis, HIF-1α production leads to what outcome?

<p>Vascular proliferation (A)</p> Signup and view all the answers

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Study Notes

Classification of Tumors

  • Vascular Tumors

    • Tumor-like conditions: vascular ectasias, bacillary angiomatosis
    • Benign tumors: hemangioma, hemangioblastoma, lymphangioma, glomus tumors
    • Intermediate malignancy: Kaposi sarcoma
    • Malignant tumors: hemangiosarcoma, lymphangiosarcoma
  • Cardiac Tumors

    • Benign tumors: cardiac myxoma, rhabdomyoma
    • Malignant tumors: sarcomas, metastatic tumors

Tumor-like Vascular Conditions

  • Vascular Ectasia

    • Dilation of existing blood vessels; not a neoplasm
    • Types include telangiectasia (includes spider telangiectasia) and nevus flammeus
    • Generalized hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
  • Telangiectasia

    • Characterized by small blood vessel dilation in skin or mucosal areas
    • Spider telangiectasia presents as a radial array of dilated subcutaneous vessels; often associated with liver issues or pregnancy
  • Nevus Flammeus

    • Congenital capillary malformation; common birthmark seen in up to 50% of newborns
    • Variants include "stork bite" (on nape) and "angel kiss" (on face), usually regresses, while port-wine stain persists
  • Bacillary Angiomatosis

    • Focal vascular proliferation due to Bartonella infection
    • Most commonly affects skin, bone, and brain, primarily in immunocompromised hosts
    • Treatment involves antibiotics

Benign Vascular Tumors

  • Hemangioma
    • Most common vascular tumor; can occur at any age
    • Classified into capillary (juvenile, often regresses) and cavernous types, with pyogenic granuloma as an inflamed version

Vascular Tumors of Intermediate Malignancy

  • Kaposi Sarcoma (KS)
    • Originates from lymphatic endothelium; presents with skin lesions that can invade and metastasize
    • Four variants: chronic (European), lymphadenopathic (African), transplant-associated, and AIDS-associated; incidence declines with antiretroviral treatment
    • Histology shows pleomorphic spindle-shaped cells and small blood vessels

Benign Cardiac Tumors

  • Cardiac Myxoma

    • Most common primary cardiac tumor in adults; can present with systemic inflammatory symptoms due to IL-6 production
    • Complications include obstruction of heart valves and risk of systemic embolization
  • Cardiac Rhabdomyoma

    • Most prevalent cardiac tumor in childhood, often associated with tuberous sclerosis
    • Multiple tumors may be present, located typically in the ventricular wall, characterized by large glycogen-laden "spider cells"

Summary of Key Tumor Presentations

  • Spider telangiectasia: dilation from liver disease or pregnancy
  • Port-wine stain: persistent congenital vascular malformation
  • Bacillary angiomatosis: opportunistic infection linked to Bartonella
  • Hemangioma: common benign vascular tumor in skin and visceral organs
  • Kaposi sarcoma: HIV and immunosuppression-related vascular malignancy
  • Cardiac rhabdomyoma: associated with genetic predisposition and regresses spontaneously
  • Cardiac myxoma: significant complications, often requiring surgical intervention

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