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Intradural Extramedullary Capillary Hemangioma

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Rare Diseases and Syndromes of the Spinal Cord
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Abstract

Capillary hemangiomas are reddish-purple lesions and microscopically by the lobules of capillary-sized channels that are tightly aggregated into nodules nourished by feeding vessels.

Abstracted from Kelleher et al. (2005)

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References

  • Choi BY, Chang KH, Choe G, et al. Spinal intradural extramedullary capillary hemangioma: MR imaging findings. AJNR Am J Neuroradiol. 2001;22(4):799–802.

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9.8 Updates

9.8 Updates

Miri et al. (2009)

The authors describe a case of a 20-year-old man complaining of back pain, radiating to both legs and paresthesia of the feet, weakness of the knee flexion and extension, urinary retention, impotence, and retrograde ejaculation. MRI showed an intradural mass, well defined at L3 level isointense at T1-weighted imaging and hyperintense at T2-weighted image with enhancement after gadolinium injection. Laminectomy at L3, after duratomy, a pinkish mass was found attached to one of the rootlets on the cauda equine and was ressected entirely. Histopathology showed capillary hemangioma. Postoperative recovery was attained without evidence of recurrence. The authors state vascular tumors account for 2–7 % of intraspinal tumors. Capillary hemangiomas within the boundries of the dura are extremely rare. They are usually distal in position, i.e., in the conus medullaris or in a root of cauda equine presenting with myelopathy or radiculopathy. As regards to the pathogenesis the capillary hemangioma arises from the vessels of nerve roots or from the subpial vessels. For the pathogenesis there are two hypotheses:

  • The lesion may evolve during the early somatic differentiation at the time of angoblastic differentiation (days 21–24 of embryogenesis) due to the impaired movement and differentiation of the primitive mesoderm for the embryonic mesodermal plate.

  • It may arise from the vascular structures within the epineurium of the nerve roots during individual during ontogenesis.

As regards to the histopathology the tumor is multilobular with pseudo-capsule. Its structure is made of capillaries lined with flattened endothelium. The lobules are separated by collagen partitions. Mild lymphocytic infiltrations may be found. Histochemical immune tests are positive against neuron-specific enolase and S-100 protein. The tumor cells stain strongly positive for endothelial markers CD31 and CD34, which are compatible with the diagnosis of capillary hemangioma.

Reference

Miri SM, Habibi Z, Hashemi M, Meybodi AT, Tabatabai SA. Capillary hemangioma of cauda equina: a case report. Cases J. 2009;2(1):80.

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Eltorai, I.M. (2016). Intradural Extramedullary Capillary Hemangioma. In: Rare Diseases and Syndromes of the Spinal Cord. Springer, Cham. https://doi.org/10.1007/978-3-319-45147-3_9

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  • DOI: https://doi.org/10.1007/978-3-319-45147-3_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-45146-6

  • Online ISBN: 978-3-319-45147-3

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