Updated: 6/22/2021

Hemangioma of Soft Tissue

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https://upload.orthobullets.com/topic/8069/images/benign cavernous hemangioma x-ray.jpg
https://upload.orthobullets.com/topic/8069/images/clinical image of hand hemangioma.jpg
https://upload.orthobullets.com/topic/8069/images/clinical image of cavernous hemangioma.jpg
https://upload.orthobullets.com/topic/8069/images/hemangioma mri forearm.jpg
https://upload.orthobullets.com/topic/8069/images/Case A - pelvis invasion - parsons_moved.png
https://upload.orthobullets.com/topic/8069/images/gross 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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Flashcards (3)
Cards
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Questions (7)

(OBQ19.19) A 29-year old male patient presents to your clinic after 3 months of medial left knee pain that is worse when standing for a long period of time and after his weekly workgroup kickball games. Initially, he thought it was arthritis in his knee, but there is associated swelling on the inside of his knee that made him concerned. He denies any neurologic symptoms or unexplained weight loss. Figures A are X-rays and select MRI cuts performed in your clinic. Figure B is a biopsy specimen. What is the tissue of origin for this lesion?

QID: 213921
FIGURES:

Cartilage

17%

(188/1130)

Bone

9%

(107/1130)

Vascular

56%

(631/1130)

Smooth Muscle

5%

(54/1130)

Skeletal Muscle

12%

(139/1130)

L 4 E

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(OBQ17.197) A 17-year-old female presents to your office with a mass on her calf that has been present for 3 years. It does not cause pain and has not been growing in size overall but does enlarge towards the end of the day. Physical examination demonstrates a 2cm non-tender mass near the Achilles tendon without skin discoloration. The patient has a normal neurovascular examination of the extremity, and no other masses are found elsewhere. Advanced imaging is shown in Figures A-C. A biopsy was previously performed by an outside surgeon. What does the biopsy most likely demonstrate and what is the best treatment at this time?

QID: 210284
FIGURES:

Spindle cells in a wavy arrangement with positive S100 staining, wide resection and radiation

11%

(186/1745)

Vascular dilations with erythrocytes and some striated muscle cells, observation

50%

(876/1745)

Small round monotonous blue cells in pseudo-rosettes, chemotherapy followed by marginal excision

2%

(43/1745)

Angiofibroblastic hyperplasia with disorganized collagen, observation

26%

(457/1745)

Hemosiderin stained multinucleated giant cells, marginal excision

10%

(166/1745)

L 4 A

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(OBQ06.119) A 17-year-old male presents to your office with hand swelling and intermittent pain. His pain occurs when his hand is held in a dependent position or when he is swimming. While he was born with a birthmark on his hand, he has noticed it is progressively getting bigger. Clinical photograph, radiographs, and histology are shown. What is the most likely diagnosis?

QID: 305
FIGURES:

Synovial sarcoma

1%

(21/3241)

Epitheliod sarcoma

5%

(169/3241)

Angiosarcoma

19%

(629/3241)

Benign hemangioma

74%

(2387/3241)

Giant cell tumor of tendon sheath

1%

(25/3241)

L 2 D

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(OBQ04.13) A 30-year-old female presents with a painful posterior knee mass. The mass gets larger and more painful with activity. Examination reveals a boggy soft tissue mass about her knee. Radiograph and MRI are shown in Figures A and B. What is the most likely diagnosis?

QID: 124
FIGURES:

Synovial sarcoma

21%

(422/2043)

Hemangioma

55%

(1128/2043)

Lipoma

2%

(42/2043)

Parosteal osteosarcoma

2%

(37/2043)

Pigmented villonodular synovitis (PVNS)

20%

(405/2043)

L 2 C

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