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
- 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)
- Infantile hemangioma
- Epithelioid hemangioma
- Spindle cell hemangioma
- Hemangioendothelioma NOS
- Epithelioid hemangioendothelioma
- Other tumors
- Arteriovenous malformation
- Capillary-venous malformation
- Capillary-lymphatic-venous malformation
- Lymphatic-venous malformation
- Capillary-arteriovenous malformation
- Capillary-lymphatic-arteriovenous malformation
- 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
- "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
- 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
- En face:
- Long Bones
- ? bony expansion
- Mixed sclerotic, lucent lesion
- Both T1- and T2-weighted images with ? uptake due to intralesional fat and blood
- 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
- 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 +
- 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
- When confined to bones, affects a region, such as multiple vertebrae
- May be associated with Osler-Weber-Rendu Disease
- 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
- 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
- An intermediate (locally aggressive, rarely metastasizing) vascular tumor of bone
- Long tubular bones (40%)
- Short tubular bones of the distal lower extremity (18%)
- Flat bones (18%)
- Vertebrae (16%)
- Small bones of the hands (8%)
- Contiguous bone involvement has been reported
- Left posterior 10th rib and T9-10 reported
- Intralesional resection, laminectomy, and spinal fusion with stable disease at 3 years reported
- Left posterior 10th rib and T9-10 reported
- Radiologic evaltion
- Radiographs reveal well marginated lucency
- Successfully txd with curettage or marginal en bloc excision
- Disease course
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