Primary Lumbar Paraganglioma: Clinical, Radiologic, Surgical, and Histopathologic Characteristics from a Case Series of 13 Patients.

Abstract:

BACKGROUND:Paragangliomas are uncommon neuroendocrine tumors, rarely occurring in the lumbar spine. Primary lumbar paragangliomas are prominently vascularized, can present variably, and pose both diagnostic and surgical challenges. We report on a large case series with long-term follow-up and intraoperative footage to characterize the natural history, diagnostic approach, and operative approach to this rare surgical disease. METHODS:This is a single-center, retrospective cohort study including all patients with histologically confirmed primary lumbar paraganglioma treated at our tertiary neurosurgical center between 1997 and 2018. Clinical, radiologic, surgical, and histologic data were collected from medical records. RESULTS:There were 13 cases of primary lumbar paraganglioma (8 men [61.5%], 5 women [38.5%]; mean age, 51.3 years; range, 33.2-68.9 years). Symptom duration correlated with tumor size (Spearman r = 0.735, P = 0.01). The main presenting symptoms were lower back pain and radiculopathy, often long-standing with recent deterioration. Seven patients (53.8%) were admitted as emergency cases, including 3 with cauda equina syndrome. Preoperative differential diagnoses included nerve sheath tumor, ependymoma, meningioma, and disk herniation. The mean Ki-67 mitotic index was 5.7% (range, 1%-10%). Surgical resection improved pain in 8 of 13 patients (61.5%) and weakness improved in 5 of 5 patients (100%). CONCLUSIONS:Primary lumbar paragangliomas are rare neoplasms of the cauda equina that typically progress slowly but may also present acutely. They are often related to the filum terminale, which should be resected prior to other attachments intraoperatively to prevent displacement of the tumor out of view. Total resection can be curative, and long-term follow-up in this series found no recurrence.

journal_name

World Neurosurg

journal_title

World neurosurgery

authors

Fiorini F,Lavrador JP,Vergani F,Bhangoo R,Gullan R,Reisz Z,Al-Sarraj S,Ashkan K

doi

10.1016/j.wneu.2020.05.144

subject

Has Abstract

pub_date

2020-10-01 00:00:00

pages

e66-e72

eissn

1878-8750

issn

1878-8769

pii

S1878-8750(20)31115-3

journal_volume

142

pub_type

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