PMC8492414_01
Patient with congenital cutaneous hemangioma of thorax, arm and forearm (left) (a) anterior and (b) posterior aspect.
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F1
Notably, he had a large cherry red cutaneous lesion on the left side of the thorax, (i.e. left side of the chest from sternum to mid line at the back), also involving the left arm/forearm (i.e. from the nape of neck to just below the scapula involving the lateral/medial arm/forearm) since birth [Figures 1a and b].
CT of thoracic vertebrae axial view showing "polkadot" appearance.
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F2
Axial studies showed the typical "polka-dot" pattern of vertebral involvement [Figure 2].
MRI dorsal spine Sagittal view (a) T1W image (b) T2W image (c) STIR image showing altered bone marrow signal intensity involving D1-D7 vertebrae appearing hypointense on both T1W and T2W images with contiguous involvement of posterior element. Patchy residual normal bone marrow signal noted in D1-D7 vertebrae. Extra dural/epidural lesion arising from C7 to D8 vertebrae appearing hypointense on T1W and hyperintense on T2W/STIR causing significant mass effect on cervicodorsal spinal cord. Intracord T2W/STIR hyperintense signal at D2-D3 level.
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F3
T1W and T2W images and enhanced with contrast (i.e., encasing the cervicodorsal spinal cord from C7 to D8 with transforaminal extension). There was also a subtle increased intracord T2W hyperintensities seen at the D2-D3 vertebral levels [Figures 3-5].
MRI dorsal spine coronal view/STIR image showing contiguous involvement of costovertebral joints at multiple levels noted on left side.
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F4
T1W and T2W images and enhanced with contrast (i.e., encasing the cervicodorsal spinal cord from C7 to D8 with transforaminal extension). There was also a subtle increased intracord T2W hyperintensities seen at the D2-D3 vertebral levels [Figures 3-5].
MRI dorsal spine axial view T2W image showing contiguous infiltration into left costovertebral joint and adjacent rib with evident altered bone marrow signal. Infiltrative extradural/ epidural lesion causing significant mass effect on spinal cord and shift of cord to right side depicted.
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F5
T1W and T2W images and enhanced with contrast (i.e., encasing the cervicodorsal spinal cord from C7 to D8 with transforaminal extension). There was also a subtle increased intracord T2W hyperintensities seen at the D2-D3 vertebral levels [Figures 3-5].
Intraoperative pictures (a) hemangiomatous lesion in para spinal soft tissue above dorsal vertebra (b) post laminectomy exposing epidural lesion encasing posterior aspect of cord (c) decompression of cord and instrumentation using pedicle screws and interconnecting rods.
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F6
Tumor was adherent to the dorsal thecal sac and demonstrated lateral, foraminal, and left-sided paraspinal extension [Figure 6a]. The blood vessels were coagulated and removed [Figure 6b]. Finally, the patient underwent a pedicle screw fusion from C7 to D5-D8 [Figure 6c and Figure 7].
Post op X-ray Dorsal spine (a) AP, (b) Lateral view.
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F7
Finally, the patient underwent a pedicle screw fusion from C7 to D5-D8 [Figure 6c and Figure 7].
Histopathological examination showing Cavernous hemangioma (a) dilated and congested vascular spaces of varying size (b) dermis show dilated and congested blood vessels (arrow). Dermis show hemorrhage (c) bone with marrow showing closely packed dilated vascular spaces filled with blood.
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F8