Vascular Tumors and Malformations

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. oka

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.

• 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.

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.

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.