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Updated: Jun 22 2021

Hemangioma of Soft Tissue

Images cavernous hemangioma x-ray.jpg image of hand hemangioma.jpg image of cavernous hemangioma.jpg mri forearm.jpg A - pelvis invasion - parsons_moved.png image of hemangioma.jpg
  • summary
    • Hemangioma of Soft Tissue is a benign vascular neoplasm that may be located in a cutaneous, subcutaneous, or intramuscular location. 
    • The condition is typically seen in patients < 30 years of age who present with a painful mass of variable size depending on activity level.
    • Diagnosis is made with MRI with contrast.
    • Treatment is usually a trial of observation, NSAIDs, vascular stockings, and activity modification. Embolization, sclerotherapy, or surgical resection are indicated for lesions associated with persistent symptoms not responsive to nonoperative management. 
  • Epidemiology
    • Incidence
      • males and females incidence is equal
      • <1% of all benign vascular tumors
    • Demographics
      • more common in patients <30 years of age
    • Anatomic location
      • intramuscular is commonly found deep in the lower extremities
      • most commonly found as isolated lesions, except for diffuse hemangioma, a rare childhood form which extensively involves a single limb
      • common in the hand
  • Etiology
    • Types
      • may be cutaneous, subcutaneous, or intramuscular
      • capillary (superficial) or cavernous (deep)
    • Pathophysiology
      • caused by an error in the new formation of a segment of the vascular system
      • hormones may play role in modulation
      • 20% are associated with trauma
  • Presentation
    • Symptoms
      • pain of variable intensity depending on activity level
      • symptoms of vascular engorgement if hemangioma is large
        • aching
        • heaviness
        • swelling
    • Physical exam
      • examine patient in both supine and standing position
        • lower extremity lesions will fill up after several minutes of standing
      • inspection
        • mass of variable size depending on activity level
  • Imaging
    • Radiograph
      • may show small phleboliths (calcifications) inside the lesion
      • erosion into adjacent bone
    • MRI with gadolinium
      • differentiates these benign lesions from arteriovenous malformations and angiosarcomas
      • increased signal on T1- and T2- weighted images
      • focal areas of low-signal are a sign of blood flow or calcifications
      • heterogeneous lesion with numerous small blood vessels and fatty infiltration (bag of worms)
  • Studies
    • Histology
      • gross
        • varies depending on whether it is capillary type or cavernous type
        • color spectrum varies from red to tan to yellow
      • micro
        • no malignant cells noted by cellular pleomorphism noted
        • many vascular dilations with large nuclei filled with erythrocytes
        • vascular lumens infiltrated with muscle fibers (intramuscular type)
        • cavernous shows large vessels with lots of fatty tissue
  • Differential
    • Other vascular malformations
      • arteriovenous malformations
      • cavernous hemangiomas
      • angiomatosis
      • vascular ectasia
    • Sarcoma
      • important to distinguish from sarcoma
  • Treatment
    • Nonoperative
      • observation, NSAIDS, vascular stockings, and activity modification
        • indications
          • first line of treatment
          • childhood lesions
      • sclerotherapy or embolization
        • performed by interventional radiology (IR)
        • indications
          • large, painful lesions that fail NSAID and vascular stockings therapy
    • Operative
      • marginal excision
        • indications
          • small lesions of the hand
      • wide surgical resection
        • indications
          • lesions resistant to nonoperative management
        • outcomes
          • high incidence of local recurrence
  • Complications
    • Kasabach-Merritt syndrome
      • rare complications caused by entrapped platelets leading to a possbly fatal coagulopathy
  • Prognosis
    • Infantile hemangiomas involute by age 7
    • No incidence of malignant transformation
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