Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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November 12, 2020
Cauda Equina Paraganglioma
- Background:
- Paragangliomas are benign, slow-growing, vascular neoplasms of neuroendocrine origin.
- The most common locations are an adrenal gland (pheochromocytomas), the carotid body at the common carotid artery bifurcation (carotid body tumors), the jugular foramen (glomus jugulare tumors), or the immediate proximity of the vagus nerve (vagal paragangliomas).
- Spinal paragangliomas are rare and mostly located in the intradural extramedullary compartment, usually involving the cauda equina and filum terminale.
- Clinical Presentation:
- Low back pain, sciatica or slowly progressive gait ataxia with cerebellar dysarthria, sensorineural hearing impairment, and headache if superficial siderosis is present
- Key Diagnostic Features:
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MRI shows a well-circumscribed mass that is isointense to the spinal cord on T1WI and iso- to hyperintense on T2WI and shows internal hemorrhage, T2WI low-signal-intensity rim (cap sign), and intense postcontrast enhancement.
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Some lesions may show a classical “salt and pepper” appearance.
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Serpentine flow voids along the surface and within the tumor nodule are typical.
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- Differential Diagnoses:
- Cauda equina paraganglioma is a rare condition and requires histologic correlation for definitive diagnosis.
- Hemangioblastoma: Iso- to hyperintense on T1WI, hyperintense on T2WI, cap sign present, and homogeneous intense enhancement of the mural nodule
- Myxopapillary ependymoma: Isointense on T1WI, hyperintense on T2WI, can be hyperintense on T1WI and T2WI due to mucinous content, predilection for cauda equina, may also show hemorrhage
- Intradural schwannoma: Isointense on T1WI, hyperintense on T2WI, moderate postcontrast enhancement with absence of vascular pedicle
- Cauda equina paraganglioma is a rare condition and requires histologic correlation for definitive diagnosis.
- Treatment:
- Complete surgical resection is curative.
- Radiotherapy is reserved for incompletely resected tumors.
- Tumor may relapse late following remote surgery; hence, prolonged postoperative follow-up is required.
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