Pathology of Vascular and Cardiac Tumors

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?

Hyperestrinism in pregnancy. Signup and view all the answers

Which type of nevus flammeus usually regresses spontaneously?

Stork bite. Signup and view all the answers

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

They disappear. Signup and view all the answers

What distinguishes generalized hereditary hemorrhagic telangiectasia from localized types?

It typically appears as multiple lesions. Signup and view all the answers

Which statement about nevus flammeus is true?

Port wine stains are always red and rough. Signup and view all the answers

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

HHV-8 Signup and view all the answers

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

Pink-red-purple patch that progresses to nodules Signup and view all the answers

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

AIDS-associated KS Signup and view all the answers

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

Pleomorphic spindle-shaped cells Signup and view all the answers

What is the primary route of transmission for Kaposi Sarcoma?

Sexual intercourse and blood transfusion Signup and view all the answers

Which clinical behavior is NOT typically associated with Kaposi Sarcoma?

Always causes severe pain Signup and view all the answers

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

Rows of RBCs, hemosiderin, and macrophages Signup and view all the answers

Which statement regarding Kaposi Sarcoma is accurate?

It may invade and metastasize despite good treatment response. Signup and view all the answers

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

Fever Signup and view all the answers

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

Systemic embolization with brain infarcts Signup and view all the answers

Which genetic condition is commonly associated with cardiac rhabdomyoma?

Tuberous sclerosis complex Signup and view all the answers

What is the typical histological appearance of cardiac rhabdomyoma?

Spider cells laden with glycogen granules Signup and view all the answers

What is a common feature of cardiac myxoma presentation?

Valvular obstruction Signup and view all the answers

In what location are cardiac rhabdomyomas usually found?

Ventricular wall Signup and view all the answers

Which of the following best describes cardiac myxoma?

Benign myocardial lesion associated with systemic inflammatory response Signup and view all the answers

What is the prognosis for cardiac rhabdomyoma?

Typically resolves without treatment Signup and view all the answers

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

Rhabdomyoma Signup and view all the answers

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

Vascular ectasia Signup and view all the answers

Which vascular tumor is associated with intermediate malignancy?

Kaposi sarcoma Signup and view all the answers

Which of the following tumors is considered malignant?

Lymphangiosarcoma Signup and view all the answers

Which benign vascular tumor primarily affects capillary structures?

Hemangioma Signup and view all the answers

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

They can arise from various organ systems Signup and view all the answers

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

Predisposing factors Signup and view all the answers

What is a known treatment approach for benign cardiac tumors?

Surgical excision Signup and view all the answers

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

Bartonella species Signup and view all the answers

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

A focal vascular proliferation Signup and view all the answers

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

Wartin-Starry (silver) stain Signup and view all the answers

Which of the following statements about hemangiomas is correct?

Juvenile hemangiomas often regress spontaneously by age 7. Signup and view all the answers

What is the most common affected location for hemangiomas?

Skin Signup and view all the answers

Which condition is characterized by inflamed hemangioma?

Pyogenic granuloma Signup and view all the answers

Which Bartonella species is responsible for cat-scratch disease?

B.henselae Signup and view all the answers

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

Vascular proliferation Signup and view all the answers

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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Description

This quiz focuses on the classification and pathology of vascular and cardiac tumors. Participants will explore different types of tumors, including benign and tumor-like conditions. Gain insights into specific conditions such as bacillary angiomatosis and cardiac myxoma.