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Intraosseous Hemangioma

DEFINITION AND PATHOGENESIS

  • Benign lesion with thin-walled capillary to cavernous vessels within bone, occasionally associated with a soft tissue mass which probable represents a hamartomatous condition

IMPORTANCE

  • Variable biologic activity resulting in little or no sxs to an aggressive clinical picture necessitating differentiation from angiosarcoma
  • Classification of vascular anomalies according to the International Society for the Study of Vascular Anomalies (ISSVA)
    • Hemangioma
      • Infantile hemangioma
      • Epithelioid hemangioma
      • Spindle cell hemangioma
      • Other
    • Hemangioendothelioma
      • Hemangioendothelioma NOS
      • Epithelioid hemangioendothelioma
    • Angiosarcoma
    • Other tumors
    • Malformations
      • Simple
        • Capillary
        • Lymphatic
        • Venous
        • Arterial
      • Combined
        • Arteriovenous malformation
        • Capillary-venous malformation
        • Capillary-lymphatic-venous malformation
        • Lymphatic-venous malformation
        • Capillary-arteriovenous malformation
        • Capillary-lymphatic-arteriovenous malformation

Other tumors

Malformations

Simple

Capillary

Lymphatic

Venous

Arterial

Combined

Arteriovenous malformation

Capillary

Capillary

Lymphatic

Capillary

Capillary

CLINICAL FEATURES

  • 75% between 20-60 years
  • M:F = 1.2
  • 1% of biopsied primary bone tumors
  • Skull and mandible (47%) and spine (28%), also femur, humerus
  • 1-7cm in size
  • Incidental finding (10.7% autopsied spines), spinal cord compressive sxs with epidural extension, loosening of teeth and bleeding
  • Complete paraplegia has been reported, with near complete recovery with decompression and fixation
  • Preoperative embolization has been utilized

RADIOLOGIC FEATURES

  • Spine
    • "Corduroy cloth", "jailhouse", or "honeycombing" pathognomonic pattern
    • "Polka dot" sign on CT
    • Spine lesions centered in body, may extend to arch or disk
    • Contiguous spinal bones or ribs may be involved
    • Extradural mass from tumor or hemorrhage ? soft tissue phleboliths
  • Skull
    • En face:
      • Round to oval focus of lucency
    • Honeycombed reticulated pattern
    • "Sunburst" pattern
    • Tangential projection:Dome shaped outer table
    • "Sunray"/"spokewheel" pattern
    • Inner table may bulge inward
  • Long Bones
    • ? bony expansion
    • Mixed sclerotic, lucent lesion
  • MRI
    • Both T1- and T2-weighted images with ? uptake due to intralesional fat and blood

GROSS PATHOLOGY

  • Honeycombed trabecular arrangement in spine and long bones, and "sun ray" bone pattern on cut surface of the bone in skull lesions
  • Gross hemorrhage, cystic areas

HISTOLOGIC FEATURES

  • Blood filled vascular channels/vessel proliferation; - anaplasia
  • Residual bone trabeculae along lines of stress (spokes of a wheel in the skull)
  • Capillary, cavernous, mixed, cellular, histiocytoid
  • Special stains:
    • Factor VIII +
  • EM:
    • Weibel-Palade bodies
    • Sclerosing hemangioma associated with hypophosphatemic rickets

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Fibrous dysplasia
  • Meningioma (skull lesions)
  • Malignant hemangioendothelioma
  • R/O other causes of pain, i.e., HNP
  • Metastatic bone disease
  • Lymphoma of bone in densely sclerotic vertebral lesions
  • Paget's disease

DISEASE COURSE AND TREATMENT

  • Requires accurate dx, usually no tx unless associated with fx or soft tissue mass compressing cord
  • Plain radiographs, MRI, or CT usually diagnostic without further w/u
  • Pathologic fx may complicate diagnostic w/u and may necessitate bx
  • Resection of a thoracic vertebral hemangioma under deep hypothermia and circulatory arrest has been reported (resection under routine measures usually adequate when neurologic deficit or pathologic fx has occurred)
  • XRT has been advocated for spinal lesions in the past, although complete resection and decompression has been recently used
  • Embolization to ? intraoperative bleeding (avoid the artery of Adamkiewicz!)
  • Brown-Sequard syndrome has been reported after intralesional absolute alcohol injection into a T8 vertebral hemangioma

SPECIAL CONSIDERATIONS

HEMANGIOMATOSIS

  • When confined to bones, affects a region, such as multiple vertebrae
  • May be associated with Osler-Weber-Rendu Disease

GLOMUS TUMOR

  • Tumor of the glomus body (probably regulates blood flow in response to temperature)
  • Often occurs subungually eroding into bone and causing a geographic lucent lesion, an innervated AV anastomotic lesion of the perivascular muscular glomus cell
  • Unusual locations include middle phalanx, proximal ulna, sacrum, coccyx
  • Highly vascular with round to cuboidal cells in sheets or nests
  • Immunoreactive for (nerve fiber) substance p
  • Curettage curative

PERIOSTEAL GLOMUS TUMOR

  • Has been reported in a periosteal location of a long bone (femur)

PERIOSTEAL (SURFACE) and INTRACORTICAL HEMANGIOMA

  • May appear radiographically identical to osteoid osteoma or Brodie's abscess
  • Cortical thickening or erosion around a small lucent region; uptake on bone scan. MR with intermediate signal on T1 and T2
  • Tibia 45%, fibula 36%, femur 9%, ulna 9%
  • May be associated with significant pain and excision of the soft-tissue component (not the sclerosed bone) has been advocated

SKELETAL-EXTRASKELETAL ANGIOMATOSIS

  • Vascular proliferation of the medullary cavity and at least one other type of tissue
  • Usually, multiple bones are involved
  • Extraskeletal sites include GI tract, mediastinum, lung, spleen, paraspinal soft tissue, soft tissue adjacent to bone
  • Histologic patterns:
    • Cavernous lymphangioma
    • Cavernous hemangioma
    • Arteriovenous hemangioma
  •  

DISAPPEARING BONE DISEASE OF GORHAM

  • (Gorham-Stout syndrome/disease, vanishing bone disease, acute spontaneous absorption fo bone, phantom bone)
  • Unpredictably progressive disease affecting children and young adults related to hemangioma whcih radiographically casues bony disappearance, even crossing joints
  • Histologcial findings of # of stimulated osteoclasts
  • LR is the rule despite curettage, bone grafting, and multiple modalities reported
  • Death may occur when disease localization leads to respiratory failure, airway obstruction, or spinal cord compression

EPITHELIOID HEMANGIOMA

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