Vascular Tumors and Malformations

Vascular Tumors and Malformations

Vascular Tumors and Malformations

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Vascular Lesions: Intro & Classification - Flowing Forms

  • Broad Classification:
    • Vascular Tumors: Result from endothelial cell proliferation. Often show growth & potential involution.
      • Examples: Infantile Hemangioma, Cherry Angioma, Pyogenic Granuloma.
    • Vascular Malformations: Structural anomalies of capillaries, veins, arteries, or lymphatics. Normal endothelial turnover.
      • Present at birth; grow proportionally with the child.
  • Key Distinctions:
    • Tumors: Cellular hyperplasia.
    • Malformations: Dysmorphic vessels.

⭐ Infantile hemangiomas are GLUT-1 positive, distinguishing them from other vascular anomalies.

  • Malformation Subtypes (ISSVA Classification):
    • Low-flow: Capillary (e.g., Port-wine stain), Venous, Lymphatic.
    • High-flow: Arteriovenous malformations (AVMs), Arteriovenous fistulas (AVFs).
    • Combined-complex: Mixed types. Vascular Tumors and Malformationsoka

Benign Vascular Tumors - Common Spots

  • Infantile Hemangioma (IH)
    • Most common in infancy; head & neck (60%), trunk (25%).
    • Appear weeks post-birth; proliferate, then involute.
    • Types: Superficial ("strawberry"), deep, mixed.

    ⭐ Oral propranolol is the first-line treatment for problematic infantile hemangiomas. Infantile hemangioma before and after treatment

  • Cherry Angioma (Campbell de Morgan spots)
    • Adults >30 yrs; bright red papules.
    • Trunk, proximal extremities.
  • Pyogenic Granuloma (Lobular Capillary Hemangioma)
    • Rapid growth, friable, red nodule; often post-trauma.
    • Fingers, lips, oral mucosa (gingiva in pregnancy).
  • Spider Angioma (Nevus Araneus)
    • Central arteriole, radiating capillaries; blanches.
    • Face, neck, upper trunk; ↑estrogen states.
  • Glomus Tumor
    • Painful, blue-red subungual nodule.
    • 📌 Triad: Paroxysmal Pain, Point tenderness, Cold sensitivity.

Intermediate & Malignant Tumors - Sinister Streams

  • Kaposi Sarcoma (KS)
    • Locally aggressive vascular proliferation; endothelial cell origin.

    ⭐ Kaposi Sarcoma is strongly associated with Human Herpesvirus 8 (HHV-8) infection.

    • Types:
      • Classic: Elderly Mediterranean men; indolent, lower extremities.
      • Endemic (African): More aggressive; lymphadenopathic in children.
      • Iatrogenic: Immunosuppression (e.g., transplant recipients).
      • AIDS-associated: Most common type globally; aggressive, widespread mucocutaneous lesions.
    • Clinical: Violaceous (red-purple-brown) macules, papules, plaques, or nodules. Oral/GI involvement common.
    • Histo: Spindle cells forming slit-like vascular spaces, extravasated RBCs, hemosiderin, hyaline globules (PAS+).
  • Angiosarcoma
    • Rare, highly malignant tumor of endothelial differentiation.
    • Risk factors: Chronic lymphedema (e.g., Stewart-Treves syndrome post-mastectomy), prior radiation, vinyl chloride exposure.
    • Clinical: Often affects elderly; scalp, face, breast. Presents as ill-defined, bruise-like patches (ecchymotic) that may evolve into nodules or ulcers.
    • Prognosis: Poor; high rates of local recurrence and metastasis.

Angiosarcoma on scalp: dermoscopy, clinical, post-op

Vascular Malformations - Congenital Currents

  • Congenital lesions present at birth; grow proportionally with child, persist throughout life.
  • Result from developmental anomalies in vascular morphogenesis.
  • Classified by predominant vessel type and hemodynamic features (flow rate).
  • Low-Flow Malformations:
    • Capillary (CM): e.g., Port-wine stain (nevus flammeus).

      ⭐ Port-wine stains in the V1 trigeminal distribution are a key indicator for Sturge-Weber Syndrome (encephalotrigeminal angiomatosis).

    • Venous (VM): Soft, compressible, bluish; may contain phleboliths. Associated with Klippel-Trenaunay Syndrome (CM + VM + limb overgrowth).
    • Lymphatic (LM): Macrocystic (cystic hygroma) or microcystic (lymphangioma circumscriptum).
  • High-Flow Malformations:
    • Arteriovenous (AVM/AVF): Direct artery-to-vein shunts; warm, pulsatile, thrill/bruit. Risk of high-output cardiac failure. Clinical examples of vascular malformations

High‑Yield Points - ⚡ Biggest Takeaways

  • Infantile Hemangiomas: Most common; proliferate then involute. Propranolol for complicated cases.
  • Port-Wine Stains: Congenital, persistent capillary malformations. Risk of Sturge-Weber syndrome (V1/V2).
  • Pyogenic Granuloma: Rapidly growing, friable papule; often post-trauma or in pregnancy.
  • Kaposi Sarcoma: HHV-8 linked vascular tumor; purplish lesions, common in AIDS.
  • Angiosarcoma: Aggressive; elderly (head/neck) or chronic lymphedema (Stewart-Treves).
  • Glomus Tumor: Painful subungual nodule; classic triad (pain, cold sensitivity, tenderness).
  • Kasabach-Merritt Syndrome: Consumptive coagulopathy with specific vascular tumors.

Practice Questions: Vascular Tumors and Malformations

Test your understanding with these related questions

Which of the following statements about cavernous hemangioma is false?

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Flashcards: Vascular Tumors and Malformations

1/10

The underlying abnormality of keloid scars is the excessive accumulation of _____ with hypervascularity.

TAP TO REVEAL ANSWER

The underlying abnormality of keloid scars is the excessive accumulation of _____ with hypervascularity.

collagen

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