Paraganglioma of the cauda equina region

Abstract

Background context: Cauda equina paragangliomas (CEPs) are rare neuroendocrine tumors. The difficulty in differential diagnosis with other tumors of this region may be misleading for surgical planning and prognostic expectations.

Purpose: To report on a rare case of CEP and review the most current information regarding the diagnosis, treatment options, and outcomes.

Study design: Case report and literature review.

Patient sample: One patient affected by CEP.

Methods: We report on a 33-year-old woman with a 2-month history of worsening low back pain, aggravated by sitting, bending, and coughing. Neurological examination revealed normal power and muscular tone, no sensory or sphincter abnormality, and normal reflex. Magnetic resonance imaging of the lumbar spine demonstrated an intradural extramedullary lesion at L3, with homogeneous contrast enhancement and hypointense punctate foci. The patient underwent an L3 laminectomy and tumor removal. Relevant articles covering CEPs from 1970 to the present were reviewed.

Results: The histopathological examinations described paraganglioma features. The postoperative course was uneventful, and all the symptoms resolved, with no tumor recurrence after 3 years' follow-up.

Conclusions: Cauda equina paragangliomas are rare, benign, and slow-growing tumors. Except for its secreting tumor characteristics, preoperative CEP diagnosis is very difficult. Magnetic resonance imaging is important and may suggest specific radiological features for these tumors; however, these are only relative, and it is rare that diagnosis is made before surgery. Diagnosis is established by histological examination and electron microscopy, and immunohistochemical techniques must be used to achieve a correct diagnosis. Cauda equina paragangliomas are well-encapsulated tumors that may be cured by surgery alone, whereas radiotherapy is reserved for incompletely resected tumors. Overall, prolonged postoperative observation is mandatory because of the slow tumor evolution and the possibility of tumor relapse even up to 30 years after surgery.

Keywords: Cauda equina; Filum terminalis; Paraganglioma; Radiotherapy; Recurrence; Surgery.