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      Intradural extramedullary capillary hemangioma of the cauda equina: Case report and literature review

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          Abstract

          Background:

          Capillary hemangiomas are benign vascular tumors that rarely occur in the neuraxis. When encountered in the spine, prompt diagnosis and complete resection is crucial. On rare instances, these lesions can acutely hemorrhage, leading to sudden neurological decline. To date, there are only 16 reported cases of intradural capillary hemangiomas in the cauda equina.

          Case Description:

          We report a case of an intradural extramedullary cauda equina capillary hemangioma that resulted in back pain and lower extremity motor deficit. Initial magnetic resonance (MR) imaging demonstrated a bilobular intradural L3-4 cauda equina lesion. The lesion was isointense on T1-weighted imaging, mildly hyperintense on T2-weighted images and avidly enhancing after gadolinium administration. Pathology confirmed the diagnosis of capillary hemangioma.

          Conclusion:

          Early diagnosis and treatment of this patient resulted in complete resection of the tumor and return of lower extremity motor function. Capillary hemangiomas should be considered in the differential diagnosis of cauda equina lesions. En bloc resection of these lesions is the mainstay of treatment.

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          Most cited references34

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          Capillary hemangioma of the central nervous system.

          Capillary hemangiomas are benign tumors or tumorlike lesions that originate from blood vessels and have rarely been reported to develop in the brain or spinal cord. The authors summarize the clinical and histological features of capillary hemangiomas of the central nervous system (CNS). The clinical features, imaging characteristics, and outcomes in 10 patients with CNS capillary hemangiomas were reviewed. Histological studies included immunostaining with CD31, alpha-smooth muscle actin, vascular endothelial growth factor, and Ki-67 antigen. Three patients with lesions in the brain presented with symptoms of increased intracranial pressure or seizures. Seven patients with lesions in the spinal cord presented with progressive sensorimotor disturbances of the lower limbs. Computerized tomography and magnetic resonance imaging demonstrated well-defined, enhancing lesions associated with marked perifocal edema. Angiography demonstrated hypervascular lesions, which have not recurred after resection. In two cases, multiple satellite lesions resolved after the systemic administration of steroid drugs or interferon-alpha. Histologically, all lesions were consistent with findings of capillary hemangioma of the skin or soft tissues. The CNS lesions differed significantly from other vascular neoplasms, such as hemangioendotheliomas, hemangiopericytomas, and hemangioblastomas. Capillary hemangiomas of the CNS are benign lesions that can be surgically removed and cured without adjuvant therapy.
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            Mesenchymal stem cells and adipogenesis in hemangioma involution.

            Hemangioma is a benign tumor of infancy whose hallmark is rapid growth during the first year of life followed by slow regression during early childhood. The proliferating phase is characterized by abundant immature endothelial cells, the involuting phase by prominent endothelial-lined vascular channels and endothelial apoptosis, and the involuted phase by few remaining capillary-like vessels surrounded by loose fibrofatty tissue. Nothing is known about the mechanisms that contribute to the adipogenesis during this spontaneous regression. We postulated that mesenchymal stem cells (MSCs) reside in the tumor and preferentially differentiate into adipocytes. To test this hypothesis, we isolated MSCs from 14 proliferating and five involuting hemangiomas by taking advantage of the well known selective adhesion of MSCs to bacteriologic dishes. These hemangioma-derived MSCs (Hem-MSCs) are similar to MSCs obtained from human bone marrow, expressing the cell surface markers SH2 (CD105), SH3, SH4, CD90, CD29, smooth muscle alpha-actin, and CD133 but not the hematopoietic markers CD45 and CD14 or the hematopoietic/endothelial markers CD34, CD31, and kinase insert domain receptor (KDR). Hem-MSCs exhibited multilineage differentiation with robust adipogenic potential that correlated with the proliferating phase. The numbers of adipogenic Hem-MSCs were higher in proliferating-phase than in involuting-phase tumors and higher than in normal infantile skin. Furthermore, Hem-MSCs exhibited a random pattern of X-chromosomal inactivation, indicating that these cells are not clonally derived. In summary, we have identified MSCs as a novel cellular constituent in infantile hemangioma. These MSCs may contribute to the adipogenesis during hemangioma involution.
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              Spinal intradural capillary haemangioma: a review.

              Capillary haemangioma is a benign tumour frequently encountered in the skin and other soft tissues. Histologically, these vascular lesions are characterised by nodules of capillary-sized vessels lined by flattened endothelium, each of which is subserved by a feeding vessel. Capillary haemangioma of the central and peripheral nervous system is extremely rare. Less than 20 of these lesions have been described as occurring within the confines of the spinal dura mater, in close relation to the conus medullaris and nerve roots of the cauda equina. The presenting symptoms are similar to those of more common intradural tumours at the conus-cauda region. Magnetic resonance imaging is the imaging modality of choice, and homogeneous enhancement following administration of Gd-DTPA is a useful clue to the diagnosis. Complete resection is the treatment of choice, and during surgery the vascular tumour is usually found encapsulated and sharply bordered from the surrounding parenchyma of the spinal cord and affected nerve roots. In the present account we give an overview of the clinical features, neuroradiological findings, therapeutic options and histopathological differential diagnostic aspects of spinal intradural capillary haemangioma. In general, vascular lesions of this entity are preoperatively misdiagnosed as neoplasms, and a higher level of clinical and radiological suspicion may avoid surgical overtreatment of these benign tumours.
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                Author and article information

                Contributors
                Journal
                Surg Neurol Int
                Surg Neurol Int
                SNI
                Surgical Neurology International
                Medknow Publications & Media Pvt Ltd (India )
                2229-5097
                2152-7806
                2015
                22 April 2015
                : 6
                : Suppl 3 , SNI: Unique Case Observations, a supplement to Surgical Neurology International
                : S127-S131
                Affiliations
                [1]Department of Neurological Surgery, University of California-Davis, 4860 Y. Street, Suite 3740, USA
                [1 ]Department of Pathology, University of California-Davis, 2805 50 th Street, Sacramento, CA. 95817, USA
                Author notes
                [* ]Corresponding author
                Article
                SNI-6-127
                10.4103/2152-7806.155701
                4408617
                25949855
                5a2685e2-5c37-41a7-9d07-f863bdaa3377
                Copyright: © 2015 Liu JJ

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 30 September 2014
                : 16 December 2014
                Categories
                Surgical Neurology International: Unique Case Observations

                Surgery
                capillary hemangioma,cauda equine,intradural,lumbar
                Surgery
                capillary hemangioma, cauda equine, intradural, lumbar

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