Acute onset of paraganglioma of filum terminale: A case report and surgical treatment

Abstract

Introduction: Paragangliomas of filum terminale are rare benign tumors, arising from the adrenal medulla or extra-adrenal paraganglia. These lesions usually present with chronic back pain and radiculopathy and only two cases of acute neurological deficit have been reported in literature.

Presentation of case: A case with an acute paraplegia and cauda equina syndrome due to an hemorrhagic paraganglioma of the filum terminale is described. Magnetic resonance imaging showed an intradural tumor extending from L1 to L2 compressing the cauda equina, with an intralesional and intradural bleed. An emergent laminectomy with total removal of the tumor was performed allowing a post-operative partial sensory recovery. Histopathological examination diagnosed paraganglioma.

Discussion: Paragangliomas are solid, slow growing tumors arising from specialized neural crest cells, mostly occurring in the head and neck and rarely in cauda equina or filum terminale. MRI is gold standard radiological for diagnosis and follow-up of these lesions. They have no pathognomonic radiological and clinical features and are frequently misdiagnosed as other spinal lesions. No significant correlation was observed between the duration of symptoms and tumor dimension. Acute presentation is unusual and emergent surgical treatment is fondamental. The outcome is very good after complete excision and radiotherapical treatment is recommended after an incomplete resection.

Conclusion: Early radiological assessment and timely surgery are mandatory to avoid progressive neurological deficits in case of acute clinical manifestation of paraganglioma of filum terminale.

Keywords: Case report; Cauda equina; Filum terminale; Paraganglioma; Spinal tumor.

Fig 1

Sagittal (B) T1-weighted (A), T2-weighted FrFSE (B) and T2-weighted FrFSE fat sat (C) magnetic resonance images revealing a large omogeneously hyperintense intradural lesion (yellow arrow) extending from L1 to L2, with enhancement after gadolinium and flow voids cranial to the mass indicative of venous congestion or high vascularity of the tumor.

Fig. 2

Axial T1 (A) and T2-weighted (B) and coronal T1-weighted (C) images showing a large lesion (yellow arrow) taking up the vast majority of the cross-sectional area at L1-L2 level, obscuring the visibility of the cauda equina. Photograph of the hemorrhagic paraganglioma (4 × 2 cm) after excision en bloc (D). One year post-operative, sagittal T1-weighted image after gadolinim (E) demonstring no residual or recurrent contrast enhancing tumor.