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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Month
Case of the Month
The Vascular Impostor : A Multifocal Osseous Hemangioma in an Adolescent
The Vascular Impostor : A Multifocal Osseous Hemangioma in an Adolescent
Contributed by : Rushikesh Jadhav
Contributed by : Rushikesh Jadhav
Introduction :
Introduction :
Osseous venous hemangiomas (slow-flow venous malformations) are rare benign vascular lesions accounting for less than 1% of primary bone tumors. They most commonly involve the vertebrae and skull, while multifocal involvement of the pelvis and long bones in children is exceptionally uncommon. Their expansile osteolytic appearance with cortical thinning and soft-tissue extension may closely mimic aggressive primary bone tumors, making clinicoradiological correlation and histopathological confirmation essential. We present a adolescent with multifocal osseous venous hemangioma involving the left hemipelvis, femur and tibia, highlighting the role of multimodality imaging and long-term follow-up in establishing the correct diagnosis.
Osseous venous hemangiomas (slow-flow venous malformations) are rare benign vascular lesions accounting for less than 1% of primary bone tumors. They most commonly involve the vertebrae and skull, while multifocal involvement of the pelvis and long bones in children is exceptionally uncommon. Their expansile osteolytic appearance with cortical thinning and soft-tissue extension may closely mimic aggressive primary bone tumors, making clinicoradiological correlation and histopathological confirmation essential. We present a adolescent with multifocal osseous venous hemangioma involving the left hemipelvis, femur and tibia, highlighting the role of multimodality imaging and long-term follow-up in establishing the correct diagnosis.
Case presentation :
Case presentation :
A 13-year-old boy presented with a gradually progressive swelling over the left inguinal and proximal thigh regions (Figure 1), first noticed during early childhood. Initially, the swelling was painless and slowly increased in size over several years. Subsequently, he developed intermittent pain around the left hip associated with limping, progressive difficulty in walking, and restriction of daily activities. There was no history of trauma, fever, weight loss, or other constitutional symptoms.
A 13-year-old boy presented with a gradually progressive swelling over the left inguinal and proximal thigh regions (Figure 1), first noticed during early childhood. Initially, the swelling was painless and slowly increased in size over several years. Subsequently, he developed intermittent pain around the left hip associated with limping, progressive difficulty in walking, and restriction of daily activities. There was no history of trauma, fever, weight loss, or other constitutional symptoms.
Fig 1
Clinical photograph demonstrating diffuse swelling over the proximal thigh (black arrow). The overlying skin shows prominent veins
Radiological Findings :
Radiological Findings :
Plain radiographs (Figure 2) demonstrated multifocal, expansile, osteolytic lesions involving the left hemipelvis, proximal femur, and proximal tibia, showing diffuse osseous expansion, cortical thinning, and coarse internal trabeculations producing a characteristic honeycomb/soap-bubble appearance. No aggressive periosteal reaction or pathological fracture are seen.
Plain radiographs (Figure 2) demonstrated multifocal, expansile, osteolytic lesions involving the left hemipelvis, proximal femur, and proximal tibia, showing diffuse osseous expansion, cortical thinning, and coarse internal trabeculations producing a characteristic honeycomb/soap-bubble appearance. No aggressive periosteal reaction or pathological fracture are seen.
Figure 2
A) Anteroposterior radiograph of the pelvis and both hips demonstrates an expansile osteolytic lesion involving both iliac bones, acetabulum, and left superior pubic ramus (black arrows) with diffuse osseous expansion and cortical thinning.
(B) Anteroposterior radiograph of the left femur demonstrates a diaphyseal lesion extending into the proximal femur with coarse internal trabeculations producing a honeycomb/soap-bubble appearance . The cortex is thinned out (white arrow) without aggressive periosteal reaction.
(C) Radiograph of the left tibia demonstrates a similar expansile osteolytic lesion involving the proximal tibial metaphysis with cortical remodeling and coarse internal trabecular pattern.
CT angiography (Figure 3) confirmed extensive intraosseous vascular channels with numerous dilated venous collaterals associated with extraosseous vascular components. The adjacent iliac and femoral arteries are displaced but remained patent without arterial encasement or evidence of a high-flow arteriovenous shunt. Serial follow-up imaging demonstrated gradual progression despite intervention while maintaining imaging features of a slow-flow vascular lesion.
CT angiography (Figure 3) confirmed extensive intraosseous vascular channels with numerous dilated venous collaterals associated with extraosseous vascular components. The adjacent iliac and femoral arteries are displaced but remained patent without arterial encasement or evidence of a high-flow arteriovenous shunt. Serial follow-up imaging demonstrated gradual progression despite intervention while maintaining imaging features of a slow-flow vascular lesion.
Figure 3
CT Angiography of both lower limbs
(A) Coronal CT angiographic image demonstrates extensive expansile osteolytic involvement of the diaphysis of the left femur and tibia with cortical expansion and coarse internal trabeculations.
(B) Axial CT image demonstrates multiple intraosseous vascular channels within the left iliac bone (white arrowheads) associated with adjacent extraosseous soft tissue extension (asterisk).
(C) Coronal maximum-intensity projection (MIP) image demonstrates numerous dilated tortuous venous collaterals surrounding the pelvis and proximal thigh (black arrows) without evidence of early arterial filling.
(D) Axial CT angiographic image demonstrates displacement of the adjacent iliac and femoral vessels (white arrows) without vascular encasement or high-flow arteriovenous communication, consistent with a slow-flow venous vascular malformation.
Magnetic resonance imaging (Figure 4) revealed extensive marrow replacement involving the left hemipelvis, proximal femur, and proximal tibia with large lobulated extraosseous soft tissue components. The lesions demonstrated low T1 signal intensity, marked T2/STIR hyperintensity, internal low-signal fibrous septa, serpiginous vascular channels (flow voids), and heterogeneous post-contrast enhancement, consistent with a vascular lesion.
Magnetic resonance imaging (Figure 4) revealed extensive marrow replacement involving the left hemipelvis, proximal femur, and proximal tibia with large lobulated extraosseous soft tissue components. The lesions demonstrated low T1 signal intensity, marked T2/STIR hyperintensity, internal low-signal fibrous septa, serpiginous vascular channels (flow voids), and heterogeneous post-contrast enhancement, consistent with a vascular lesion.
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Figure 4
Magnetic Resonance Imaging
(A) Coronal T1-weighted image demonstrates diffuse replacement of normal marrow involving the left hemipelvis and proximal femur by a predominantly hypointense lesion.
(B&C) Coronal T2-weighted/STIR image demonstrates marked hyperintensity of the lesion with multiple internal low-signal fibrous septa (black arrowheads) and extensive extraosseous soft tissue extension (asterisk).
(D) Axial post-contrast fat-suppressed T1-weighted image demonstrates heterogeneous enhancement of the intraosseous and extraosseous components (white arrows) with enhancing fibrous septa and preservation of adjacent neurovascular planes.
Radiological Diagnosis :
Radiological Diagnosis :
Multifocal slow-flow intraosseous venous malformation (osseous venous hemangioma) involving the left hemipelvis, proximal femur, and proximal tibia with associated extraosseous soft tissue extension.
Multifocal slow-flow intraosseous venous malformation (osseous venous hemangioma) involving the left hemipelvis, proximal femur, and proximal tibia with associated extraosseous soft tissue extension.
Differential Diagnosis
Differential Diagnosis
Based on the initial imaging appearance, the differential diagnoses included:
Based on the initial imaging appearance, the differential diagnoses included:
• Ewing sarcoma – considered because of the patient's age, extensive marrow involvement, and associated soft tissue component.
• Ewing sarcoma – considered because of the patient's age, extensive marrow involvement, and associated soft tissue component.
• Telangiectatic osteosarcoma – owing to the expansile lytic appearance with cortical thinning.
• Telangiectatic osteosarcoma – owing to the expansile lytic appearance with cortical thinning.
• Aneurysmal bone cyst – because of the expansile multicystic appearance.
• Aneurysmal bone cyst – because of the expansile multicystic appearance.
• Fibrous dysplasia – due to extensive osseous expansion, although the absence of ground-glass matrix made this less likely.
• Fibrous dysplasia – due to extensive osseous expansion, although the absence of ground-glass matrix made this less likely.
• Primary bone angiosarcoma – considered because of the marked vascularity and extensive osseous involvement.
• Primary bone angiosarcoma – considered because of the marked vascularity and extensive osseous involvement.
The absence of aggressive periosteal reaction, preservation of cortical remodeling, serpiginous vascular channels, displacement rather than invasion of adjacent neurovascular structures, and indolent progression favored a benign vascular lesion.
The absence of aggressive periosteal reaction, preservation of cortical remodeling, serpiginous vascular channels, displacement rather than invasion of adjacent neurovascular structures, and indolent progression favored a benign vascular lesion.
Pathological Diagnosis :
Pathological Diagnosis :
Image-guided biopsy performed at a tertiary oncology center demonstrated numerous dilated venous vascular channels lined by bland endothelial cells within fibrovascular stroma without significant cytological atypia or increased mitotic activity, confirming the diagnosis of venous hemangioma (slow-flow venous vascular malformation).
Image-guided biopsy performed at a tertiary oncology center demonstrated numerous dilated venous vascular channels lined by bland endothelial cells within fibrovascular stroma without significant cytological atypia or increased mitotic activity, confirming the diagnosis of venous hemangioma (slow-flow venous vascular malformation).
Following histopathological confirmation, the patient was discussed in a multidisciplinary team comprising orthopedic oncology, interventional radiology, and musculoskeletal radiology specialists. Owing to the extensive skeletal involvement and high vascularity of the lesion, complete surgical excision was considered technically challenging and associated with a significant risk of hemorrhage.
Following histopathological confirmation, the patient was discussed in a multidisciplinary team comprising orthopedic oncology, interventional radiology, and musculoskeletal radiology specialists. Owing to the extensive skeletal involvement and high vascularity of the lesion, complete surgical excision was considered technically challenging and associated with a significant risk of hemorrhage.
The patient subsequently underwent multiple sessions of image-guided percutaneous sclerotherapy along with selective transarterial embolization to reduce lesion vascularity and improve symptoms. Clinical and imaging follow-up over nearly twelve years demonstrated persistent but slowly progressive disease without imaging evidence of malignant transformation. The patient continues to remain under regular multidisciplinary surveillance with periodic MRI and CT angiographic evaluation.
The patient subsequently underwent multiple sessions of image-guided percutaneous sclerotherapy along with selective transarterial embolization to reduce lesion vascularity and improve symptoms. Clinical and imaging follow-up over nearly twelve years demonstrated persistent but slowly progressive disease without imaging evidence of malignant transformation. The patient continues to remain under regular multidisciplinary surveillance with periodic MRI and CT angiographic evaluation.
Timeline
Timeline
Discussion :
Discussion :
Venous hemangiomas, currently classified under the spectrum of slow-flow venous malformations by the ISSVA, are benign congenital vascular anomalies resulting from aberrant embryological development rather than true neoplastic proliferation [1]. Primary intraosseous lesions account for less than 1% of primary bone tumors and most commonly involve the vertebral column and calvarium; appendicular multifocal disease is exceedingly rare [2,3].
Venous hemangiomas, currently classified under the spectrum of slow-flow venous malformations by the ISSVA, are benign congenital vascular anomalies resulting from aberrant embryological development rather than true neoplastic proliferation [1]. Primary intraosseous lesions account for less than 1% of primary bone tumors and most commonly involve the vertebral column and calvarium; appendicular multifocal disease is exceedingly rare [2,3].
Radiographs typically demonstrate expansile osteolytic lesions with coarse trabeculation producing honeycomb or soap-bubble appearances, whereas CT depicts cortical remodeling and vascular channels [1,3,4]. MRI is the modality of choice, showing T1 hypointensity, marked T2/STIR hyperintensity, internal fibrous septa, serpiginous vascular channels and progressive heterogeneous enhancement, while accurately assessing marrow involvement and soft-tissue extension [4,5,6]. CT angiography is valuable for defining vascular anatomy before intervention [7,8].
Radiographs typically demonstrate expansile osteolytic lesions with coarse trabeculation producing honeycomb or soap-bubble appearances, whereas CT depicts cortical remodeling and vascular channels [1,3,4]. MRI is the modality of choice, showing T1 hypointensity, marked T2/STIR hyperintensity, internal fibrous septa, serpiginous vascular channels and progressive heterogeneous enhancement, while accurately assessing marrow involvement and soft-tissue extension [4,5,6]. CT angiography is valuable for defining vascular anatomy before intervention [7,8].
The principal differentials include Ewing sarcoma, telangiectatic osteosarcoma, aneurysmal bone cyst, fibrous dysplasia and primary angiosarcoma [9–13]. In our patient, the absence of aggressive periosteal reaction, preserved cortical remodeling, displacement rather than invasion of adjacent neurovascular structures, and indolent progression favored a benign vascular lesion despite its extensive appearance.
The principal differentials include Ewing sarcoma, telangiectatic osteosarcoma, aneurysmal bone cyst, fibrous dysplasia and primary angiosarcoma [9–13]. In our patient, the absence of aggressive periosteal reaction, preserved cortical remodeling, displacement rather than invasion of adjacent neurovascular structures, and indolent progression favored a benign vascular lesion despite its extensive appearance.
Histopathology remains the diagnostic gold standard and demonstrates dilated venous channels lined by bland endothelial cells within fibrovascular stroma without significant atypia [1,13]. Management is individualized and includes observation, sclerotherapy, embolization and selected surgical intervention depending on lesion extent and symptoms [3,7,8]. This case emphasizes that careful multimodality imaging combined with histopathological confirmation can prevent misdiagnosis and unnecessary radical oncological treatment.
Histopathology remains the diagnostic gold standard and demonstrates dilated venous channels lined by bland endothelial cells within fibrovascular stroma without significant atypia [1,13]. Management is individualized and includes observation, sclerotherapy, embolization and selected surgical intervention depending on lesion extent and symptoms [3,7,8]. This case emphasizes that careful multimodality imaging combined with histopathological confirmation can prevent misdiagnosis and unnecessary radical oncological treatment.
Conclusion :
Conclusion :
Multifocal osseous venous hemangioma is an exceptionally rare benign vascular lesion that can closely mimic primary bone malignancy. Recognition of its characteristic multimodality imaging features, supported by histopathology, is essential for accurate diagnosis and appropriate multidisciplinary management.
Multifocal osseous venous hemangioma is an exceptionally rare benign vascular lesion that can closely mimic primary bone malignancy. Recognition of its characteristic multimodality imaging features, supported by histopathology, is essential for accurate diagnosis and appropriate multidisciplinary management.
References:
References:
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1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children. Plast Reconstr Surg. 1982;69(3):412-422. PMID:7063565.
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7 Gaudino S, Martucci M, et al. A systematic approach to vertebral hemangioma. Skeletal Radiol. 2015;44(1):25-36.
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