Central/intraosseous venous malformation/hemangioma

Central/intraosseous venous malformation/hemangioma of the jaws is a non-neoplastic, slow-flow vascular anomaly composed of dilated venous channels within medullary bone.[1] Under the International Society for the Study of Vascular Anomalies (ISSVA) classification, most lesions historically labeled "central hemangioma" of the jaws are reclassified as intraosseous venous malformations — developmental errors in vasculogenesis rather than true proliferative neoplasms.[2][3] The mandible is affected far more often than the maxilla, with a strong predilection for the posterior jaws.[4] The signature dental radiographic pattern is a multilocular radiolucency with honeycomb or soap-bubble internal architecture, although considerable variability exists.[4] The lesion carries a risk of life-threatening hemorrhage during tooth extraction or biopsy, making pre-procedural recognition critical.[5][6] Management ranges from conservative curettage to en-bloc resection, often preceded by embolization.[7][8]

The ISSVA classification (updated 2014/2018) divides vascular anomalies into vascular tumors (proliferative, GLUT-1–positive in infantile hemangiomas) and vascular malformations (structural, non-proliferative, GLUT-1–negative).[2][9] Under this framework, the vast majority of intraosseous jaw lesions historically called "central hemangioma" or "cavernous hemangioma" are venous malformations.[3][10] The WHO classification of bone tumors retains "hemangioma" as a diagnostic category, acknowledging microscopic overlap with vascular malformation; subtypes include cavernous, capillary, and mixed.[11][12]

• Preferred current terms: intraosseous venous malformation; central venous malformation of the jaws[1][7]
• Legacy terms still in wide use: central hemangioma, intraosseous hemangioma, cavernous hemangioma of the jaws[3][10]
• Bony venous malformation (BVM): recently proposed as a unified diagnostic category for intraosseous venous malformations of facial bones[7]

The persistent use of "hemangioma" in clinical, pathologic, and radiologic practice creates diagnostic confusion and may lead to inappropriate management; adoption of ISSVA-aligned terminology is recommended.[1][3]

• Frequency: Intraosseous vascular lesions account for approximately 0.5%–1% of all bone tumors; jaw involvement is uncommon relative to the vertebral column and calvarium.[13][14]
• Age: Diagnosis spans a wide range (0–74 years); mean age at presentation is approximately 23 years (median 17 years) in a pooled review of 86 cases.[4]
• Sex: Approximately equal distribution between males and females. Some smaller series report a slight female predominance.[4][13]
• Jaw predilection: Mandible-to-maxilla ratio of approximately 3.3:1. Roughly 69% of jaw lesions occur in the posterior region (premolar–molar–ramus).[4]

Presentation and symptoms

Many lesions are discovered incidentally on dental radiographs and may be entirely asymptomatic.[13][15] When symptomatic, common findings include:

• Painless swelling or facial asymmetry, often slowly progressive[7]
• Tooth mobility, displacement, or accelerated exfoliation[13][16]
• Bluish-purple discoloration of overlying mucosa or gingiva[13][15]
• Pulsatile or spontaneous gingival bleeding; periodontal oozing[16]
• Pain or discomfort (variable)[7]
• Malocclusion in larger lesions[7]

Red flags and complications

• Hemorrhage is the most critical complication. Tooth extraction, biopsy, or even primary tooth exfoliation in a child can provoke massive, life-threatening bleeding — including hemorrhagic shock and death. Spontaneous rupture with uncontrolled oral hemorrhage, though rare, has been reported.[5][16][17]
• Elevated cutaneous temperature, palpable pulsation, or an audible bruit over the lesion should raise suspicion for a high-flow (arteriovenous) component.[16][18]
• Gingival telangiectasia and erythema adjacent to the lesion are suggestive clinical clues.[16][19]

Location and distribution

The posterior mandibular body and ramus are the most common sites; maxillary lesions are less frequent and may involve the alveolar process and extend into the maxillary sinus.[4][20] Lesions are typically solitary and centered within medullary bone, often in a tooth-bearing region.[4]

Periphery and shape

• Borders are diffuse or poorly defined in approximately 68% of cases and well defined in roughly 32%.[4]
• Shape is variable — round, ovoid, or irregular — and does not reliably distinguish the lesion from other jaw pathology.[4][6]
• Fine, irregular margins on cross-sectional imaging are characteristic.[21]

Internal architecture

• Multilocular radiolucency is the most common pattern (approximately 66%), producing a honeycomb or soap-bubble appearance.[4][13]
• Unilocular radiolucency occurs in roughly 33% of cases.[4]
• Lesions are radiolucent in 96% of cases; a mixed or predominantly radiopaque pattern is rare.[4]
• Internal bony spicules or coarsened trabeculae may be seen, corresponding to residual trabecular bone between dilated vascular channels.[1]
• The classic sunburst (sunray) or spoke-wheel pattern, although frequently cited in textbooks, is actually extremely rare in jaw lesions.[4]
• Phleboliths (calcified thrombi) are a hallmark of venous malformations in soft tissue but are uncommonly identified within intraosseous jaw lesions on dental radiographs.[18]

Effects on teeth and adjacent structures

• Root resorption is described in approximately 23% of cases and is more common in mandibular lesions.[4]
• Tooth displacement occurs in roughly 16%.[4]
• The inferior alveolar nerve canal is involved in approximately 15% of mandibular lesions; continuity between the lesion and the mandibular canal is a useful diagnostic clue on cross-sectional imaging.[4][21]
• Maxillary lesions may opacify or extend into the maxillary sinus (approximately 35% of maxillary cases).[4]

Extent and cortical involvement

• Expansion of the cortical plates is common in larger lesions; cortical thinning or perforation may occur.[7][13]
• Bone overgrowth (periosteal new bone) has been observed in a minority of cases.[21]
• CBCT and cross-sectional imaging are superior to conventional radiography for delineating the three-dimensional extent of the lesion, identifying cortical integrity, and detecting enlarged feeding or draining vessels within or adjacent to the lesion.[7][21]

Variants

• Rare multicentric intraosseous involvement (multiple bones) has been reported.[14]
• Combined intraosseous and extraosseous (soft-tissue) extension may occur, particularly when cortical integrity is lost.[16]

CT (medical)

Medical CT demonstrates a low-to-intermediate-density lesion (approximately 30–53 HU) with fine, irregular borders.[21] Multilocularity, enlarged feeding and outflow vessels, and continuity with the mandibular canal are characteristic findings.[21] Contrast-enhanced CT shows heterogeneous enhancement within the cancellous bone.[20] Phleboliths, when present, appear as small calcified foci and are more readily detected on CT than on MRI.[18][22]

MRI

MRI is the most sensitive modality for delineating lesion extent and soft-tissue involvement:[23][24]

• T1-weighted imaging: low to intermediate signal intensity[21]
• T2-weighted / STIR: intermediate to high signal intensity; heterogeneous high signal on STIR[18][21]
• Flow voids are consistently identified and help confirm the vascular nature of the lesion[21]
• Contrast-enhanced sequences: progressive, heterogeneous enhancement[18][24]
• Dynamic contrast-enhanced MRI (DCE-MRI): venous malformations typically show early weak enhancement followed by a plateau, a pattern that can aid differentiation from other vascular and non-vascular lesions[24]
• DWI/ADC: reported ADC values range from 0.69 to 1.74 × 10⁻³ mm²/s[21]

MRI is particularly valuable for distinguishing low-flow (venous) from high-flow (arteriovenous) lesions; the latter demonstrate prominent flow voids with enlarged feeding arteries and early draining veins.[18][25] MR angiography can further characterize feeding vessels when a high-flow component is suspected.[21]

Angiography

Conventional angiography (digital subtraction angiography) remains the reference standard for characterizing flow dynamics, identifying feeder vessels, and guiding preoperative embolization.[5][26]

Central/intraosseous venous malformation produces a nonspecific multilocular or unilocular radiolucency that overlaps with many odontogenic and non-odontogenic jaw lesions. The key discriminating features are clinical signs of vascularity (mucosal discoloration, bleeding, pulsation), characteristic cross-sectional imaging findings (internal bony spicules, flow voids, phleboliths, vessel continuity), and the critical importance of aspiration before biopsy.

The highest-risk diagnostic pitfall is failure to suspect a vascular lesion before biopsy or extraction. When any jaw radiolucency is accompanied by unexplained gingival bleeding, mucosal discoloration, tooth mobility, or pulsation, needle aspiration should precede incisional biopsy to detect blood return and avert potentially fatal hemorrhage.[5][6][28] Cross-sectional imaging (CT with contrast and/or MRI) should be obtained before any invasive procedure when a vascular lesion is in the differential.[21][28]

Microscopic features

The lesion consists of dilated, thin-walled vascular channels lined by a single layer of flat endothelial cells, separated by fibrous connective tissue and residual bony trabeculae.[11][13] Two histologic patterns are recognized:

• Cavernous type: large, dilated, blood-filled sinusoidal spaces — the most common pattern in jaw lesions[11][14]
• Capillary type: smaller, more uniform vascular channels; less common and may coexist with cavernous areas[11]

Thrombus formation and phlebolith development may be seen within vascular spaces.[18]

Immunohistochemistry

• GLUT-1: negative in intraosseous venous malformations, distinguishing them from true infantile hemangiomas (which are GLUT-1–positive)[3][10][29]
• D2-40: negative, excluding lymphatic differentiation[3][29]
• CD31 and ERG: positive, confirming endothelial differentiation[11][29]
• α-smooth muscle actin (α-SMA): highlights smooth muscle cells and pericytes in vessel walls[29]

GLUT-1 negativity is the single most important immunohistochemical finding supporting classification as a vascular malformation rather than a hemangioma under the ISSVA framework.[3][10]

Molecular findings

Somatic activating mutations in TEK (TIE2) are found in over 50% of sporadic venous malformations; PIK3CA mutations account for 30%–50% of TEK-negative cases.[30][31][32] Both converge on the PI3K/AKT/mTOR signaling pathway.[18] In the WHO bone tumor classification, recurrent EWSR1::NFATC2 fusions have been identified in intraosseous hemangioma/vascular malformation of bone, though data on jaw-specific lesions remain limited.[11]

Workup and referral

• Any jaw radiolucency with clinical or imaging features suggestive of a vascular lesion warrants contrast-enhanced CT and/or MRI before biopsy or extraction.[21][28]
• Needle aspiration (with appropriate hemostatic preparation) should be performed before incisional biopsy to detect frank blood return.[5][6]
• Referral to an oral and maxillofacial surgeon experienced in vascular lesions and, when indicated, to interventional radiology for angiographic assessment and embolization planning.[8][26]

Treatment

• Surgical resection (en-bloc resection or segmental mandibulectomy/maxillectomy) remains the definitive treatment for large or symptomatic lesions and provides the lowest recurrence risk.[7][8][15]
• Aggressive curettage may be appropriate for selected smaller or favorably located lesions, potentially sparing teeth and avoiding extensive reconstruction.[8]
• Preoperative embolization (transarterial and/or direct-puncture) reduces intraoperative hemorrhage and is recommended for lesions with significant vascularity or high-flow components.[5][26][33]
• Sclerotherapy (sodium tetradecyl sulfate, ethanol, bleomycin) is the mainstay for soft-tissue venous malformations and may be used as primary or adjunctive treatment for intraosseous lesions with extraosseous extension.[18]
• Medical therapy with sirolimus (mTOR inhibitor) has been used for refractory pain, swelling, and coagulopathy in venous malformations, reflecting the PI3K/AKT/mTOR pathway involvement.[18][31]
• Temporary external carotid artery ligation has been described as an adjunct to minimize bleeding during resection of large maxillary lesions.[33]

Recurrence and prognosis

• Prognosis after complete surgical excision is excellent, with low recurrence rates.[7][8]
• Recanalization has been reported in approximately 14% of surgically treated venous malformations of facial bones.[8]
• Incomplete treatment, trauma, infection, and hormonal fluctuations (puberty, pregnancy) can stimulate lesion expansion.[18]
• Long-term clinical and imaging surveillance is recommended, particularly after conservative treatment.[7][8]

Pediatric considerations

Central vascular malformations may present during the mixed dentition period with spontaneous gingival bleeding or accelerated primary tooth exfoliation, mimicking physiologic tooth loss.[15][16] Life-threatening hemorrhage during the dental transitional period has been reported; awareness among pediatric dentists is essential.[16][17]

Hormonal influences

Venous malformations may enlarge during puberty or pregnancy due to hormonal fluctuations, potentially converting a previously asymptomatic lesion into a symptomatic or dangerous one.[18] More than 60% of venous malformations reportedly progress during adolescence, supporting consideration of earlier treatment even for asymptomatic lesions.[18]