DEFINITION AND PATHOGENESIS
- Capillary hemangioma
- Cavernous hemangioma
- Arteriovenous hemangioma
- Venous hemangioma
- Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia)
- Pyogenic granuloma (granulation tissue type capillary hemangioma)
- Intramuscular hemangioma
- Synovial hemangioma
- Hemangioma of peripheral nerve (perineural hemangioma)
- The most common tumor of childhood
- 7% of all benign tumors, the most common tumor in infancy and childhood; may present as a painless mass, variation in size
- 60% of synovial hemangiomas occur in the knee
- Swelling and joint pain
- Limitation of motion reported in hip location
- Spontaneous hemarthrosis/monarthritis in children and adolescents
- Overlying skin discoloration may be present
- May become clinically apparent during pregnancy
- Pulsatile or audible bruit rarely
- Tender, spongy mass that may decrease in size with palpation or elevation
- Contractures have been reported
- Bilateral 1st dorsal compartment hemangiomas reported causing bilateral deQuervain's stenosing tenosynovitis
- Locking of a MCPJ by a volar plate hemangioma reported
- Neuropathies have been reported: PIN, ulnar nerve at the cubital tunnel
- FDP flexor sheath location reported as a cause of CTS
- Unusual location
- Mineralization types
- Phleboliths (49%) (on radiographs or CT)
- Nonspecific amorphous or curvilinear
- Metaplastic ossification
- ± cortical erosions or sclerosis in long-standing lesions
- LLD has been reported
- MRIreveals worrisome indistinct margins; MRA diagnostic
- T1-weighted images reveal poorly marginated mass isointense with muscle
- Some areas isointense with fat can be seen
- T2-weighted images show marked ? signal
- Serpiginous regions
- Robust contrast enhancement
- ± effusion in joint locations
- High flow signal, vascular channels
- The combination of lobulation, septation, and central low-signal-intensity dots (not seen on gadolinium-enhanced images) on MRI specific for hemangioma
- Tortuous blood vessels within subcutaneous or muscular tissues
- Depends on type, but with a myriad of dilatedvessels with plump nuclei within the cells
- Lobular capillary
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Soft tissue sarcomas, esp angiosarcoma
- Vascular malformation
- Intravascular papillary endothelial hyperplasia
- Kaposi's sarcoma
- PVNS or synovial chondromatosis in the joint location
- Synovial sarcoma
DISEASE COURSE AND TREATMENT
- Observation in asymptomatic pts after adequate bx
- Intramuscular hemangiomas must be suspected prior to bx or bleeding may be a problem
- Large unresectable hemangioma tx is controversial
- Percutaneous embolization may require several txs
- LR often because of incomplete excision
- Thrombocytopenia purpura associated usually with a cavernous hemangioma of an extremity and trauma such as a femoral fx
- Surgery can be complicated by bleeding due to platelet consumption from sequestration in sinusoids, secondary fibrinolysis, hypofibrinogenemia, ? fibrin split products, and DIC
- Resuscitation includes FFP, cryoprecipitate, platelets
- Right posterior triangle kaposiform hemangiomaextending to skull base resulting in respiratory compromise and spinal cord compression in an infant reported
- Corticosteroids, medical managment, embolization and vincristine reported
- Hematology consultation!
- Multiple hemangiomas and enchondromas with a high rate of malignant degeneration of either type lesion
- Gigantism (bone and soft tissue hypertrophy with LLD), massive cavernous hemangiomas and varicosities (laterally in a 'lumbar-to-foot' pattern)
- (Also at risk for Wilms' tumor)
- High output cardiac failure can occur necessitating tx
- Total circulatory arrest in infants for surgical resection has been described
- May require amputation for functional or cosmetic reasons (pre-prosthetic consultations)
- Thrombophlebitis, DVT/PE
- 95% LE, 85% unilateral
EPIDURAL SPINAL CAVERNOUS HEMANGIOMA
- Pure epidural lesion rare
- Low to intermediate signal on T1-weighted and high signal on T2-weighted and proton density images
- Laminectomy and microsurgical resection when symptomatically indicated
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