Dear Editor, Nerve root compression secondary to a combination of lumbosacral disc herniation and intrathecal space-occupying lesion is a rare co-incidence, particularly when histology reveals a paraganglioma. Cauda equina paraganglioma is a rare tumour that can be hypervascular and is associated with neuroendocrine disturbance during intraoperative tumour manipulation. Dual extraand intra-thecal pathology is unusual in the spine but can be addressed by a single operation to good effect. A 33-year-old gentleman was referred with a 12month history of persistent left leg sciatica associated with troublesome paraesthesiae in the left S1 dermatome. Neurological examination revealed 4+/5 left plantar flexion, an absent left ankle reflex and reduced pinprick sensation in the S1 dermatome. MR imaging of the lumbar spine revealed an avidly enhancing, intrathecal space-occupying lesion at the L5/S1 level (1.5×2 cm) as well as a large posterolateral L5/S1 disc protusion, resulting in S1 nerve root compression (Fig. 1). The patient underwent L5 and S1 laminectomies followed by intrathecal resection of a dark red, encapsulated lesion. Bipolar coagulation of the attached filum terminale arrested the tumour blood supply prior to manipulation. After dural closure, the left S1 root was retracted and a microdiscectomy was performed (Fig. 2). The patient was pain-free postoperatively with resolution of his antalgic gait, regaining the ability to walk on tiptoes. Histology revealed paraganglioma (WHO I) (Fig. 3). Followup imaging at 12 months did not reveal any evidence of recurrence, and the patient remained asymptomatic. Cauda equina paraganglioma (CEP) accounts for 3–4 % of all tumours in the region of the cauda equina [6]. There are no particular radiological features that reliably distinguish this entity from other more commonly encountered tumours at this site, such as ependymoma or nerve sheath tumour. The literature classifies CEP as a distinct sub-category of paraganglioma since its first description in 1970 by Miller and Torack [4]. CEP is thought to derive from primitive neural crest cells; however, whether the pathogenesis is due to aberrant migration of paraganglioma cells or the non-regression of paraganglioma cell nests is unclear [3]. The most common presenting symptoms of CEP are low back pain (51 %) and sciatica (19 %) [2]. CEP is found as commonly attached to the filum terminale as it is to nerve roots. Spinal paraganglioma has a characteristic vascular and endocrine phenotype. Typically, histopathology reveals small, rounded, polyhedral cells arranged in nests or ‘Zellballen’. Immunohistochemistry is positive for a variety of markers for both chief and sustenacular cells, including synaptophysin, chromogranins A and B, and S-100 protein [3]. CEP can demonstrate hormonal activity; however, in the largest review in the literature to date, this was found in only 3/174 cases (approximately 2 %) [2]. Intraoperative release of catecholaminergic hormones during tumour manipulation can result in haemodynamic instability, and we advise early vascular detachment. CEP can be highly vascular, and some authors advocate preoperative vascular imaging [1]. Gross R. Bhatia (*) : L. Zrinzo Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK e-mail: robbhatia@yahoo.com
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