Sporadic hemangioblastoma of cauda equina: A case-report and brief literature review

Hemangioblastomas are rare lesions accounting for 1–5% of all spinal cord tumors and are mostly associated with von Hippel–Lindau syndrome. Localization in the cauda equina is uncommon. In this manuscript we aim to describe a rare case of sporadic intradural extramedullary hemangioblastoma of the ca...

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Veröffentlicht in:Neurocirugía (Barcelona. Internet. English ed.) 2023-05, Vol.34 (3), p.153-158
Hauptverfasser: D’Oria, Salvatore, Giraldi, David, Fanelli, Vincenzo, D’Angelo, Vincenzo
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Sprache:eng
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Zusammenfassung:Hemangioblastomas are rare lesions accounting for 1–5% of all spinal cord tumors and are mostly associated with von Hippel–Lindau syndrome. Localization in the cauda equina is uncommon. In this manuscript we aim to describe a rare case of sporadic intradural extramedullary hemangioblastoma of the cauda equina and present a literature review. A systematic research was performed on Pubmed, MEDLINE, and Google Scholar, using as keywords “spinal hemangioblastoma” and “cauda equina tumors”. The previous literature is integrated by the description of the present case. A 49 year-old female, presented on August 2020 to our institution suffering from claudication neurogena, right sciatica and paraesthesia in right L5 radicular dermatome for more than 3 months. Neurological examination revealed hypoesthesia on right L5 dermatome and weakness of right anterior tibialis muscle. An MRI which showed an intradural mass at L1/2 level and an angiography that showing a nidus of serpiginous vessels inside the lesion. Microsurgical en bloc resection of lesion was performed with adjuvant neurophisological intra operative monitorings. Histological examination provided the diagnosis of hemangioblastoma. After surgery symptoms and neurological impairment gradually improved. A 10 months post-operative MRI showed no residual tumor. Although intradural extramedullary hemangioblastoma of the cauda equina without von Hippel–Lindau syndrome it is a rare pathological entity, this diagnosis must be taken in for cauda equina masses. Preoperative embolization is an option to minimize intraoperative bleeding. Radiosurgery seems to prevent recurrences when the tumor is not completely excised. Complete surgical removal of the lesion is usually possible and lead to a low likelihood of recurrence. Los hemangioblastomas son lesiones raras que representan del 1 al 5% de todos los tumores de la médula espinal y se asocian principalmente al síndrome de von Hippel-Lindau. La localización en la cauda equine es infrecuente. En este manuscrito nuestro objetivo es describir un caso raro de hemangioblastoma intradural extramedular esporádico de la cauda equina y presentar una revisión de la literatura. Se realizó una bùsqueda sistemática en Pubmed, MEDLINE y Google Scholar, utilizando como palabras clave «spinal hemangioblastoma» y «cauda equina tumors». Presentamos el caso clínico y se discute, y se compara con la literatura previamente publicada al respecto. Mujer de 49 años, se presentó en agosto del
ISSN:2529-8496
2529-8496
DOI:10.1016/j.neucie.2022.11.019