Neuropathology

Paraganglioma of the cauda equina

Elisabeth Rushing
Armed Forces Institute of Pathology
Washington, DC


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Case History
The patient is a 29 year-old male who experienced low-back pain of increasing severity that radiated down both legs. Subsequently, he was discovered to have a mass in the region of the cauda equina.

 
Magnetic resonance (MR) imaging showed an intradural mass localized to the conus medullaris and proximal cauda equina. The mass was isointense with spinal cord on all pulse sequences and enhanced avidly after administration of contrast material. Flow voids were seen on both T1 and T2-weighted images.

 
At surgery, the tumor appeared as a soft, sausage-shaped and well-encapsulated vascular mass.


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Differential Diagnosis
  1. Myxopapillary ependymoma

  2. Schwannoma

  3. Meningioma (meningothelial)

Final Diagnosis and References
Paraganglioma of the cauda equina

Key words:
cauda equina, immunohistochemistry,myxopapillary ependymoma, paraganglioma,

Take-home points
  • Although rare, paragangliomas should always be considered in the differential diagnosis of cauda equina tumors.

  • Features such as "zellballen" or packeted architecture, composed of uniform, polygonal chief cells with "salt and pepper" should raise suspicion.

  • Paragangliomas may mimic the perivascular pseudorosette growth pattern of ependymomas.

  • Immunohistochemical studies with neuroendocrine markers substantiate the diagnosis

References
  1. Labrousse F, Leboutet MJ, Petit B, Paraf F, Boncoeur-Martel MP, Moreau JJ, Catanzano G. Cytokeratins expression in paragangliomas of the cauda equina. Clin Neuropathol . 1999; 18 :208-13.

  2. MoranCA, RushW, MenaH . Primary spinal paragangliomas. A clinicopathological and immunohistochemical study of 30 cases . Histopathology . 1997; 31 : 167-173 .

  3. Raftopoulos C, Flament-Durand J, Brucher JM, Stroobandt G, Chaskis C, Brotchi J. Paraganglioma of the cauda equina. Report of 2 cases and review of 59 cases from the literature. Clin Neurol Neurosurg. 1990;92:263-70.

  4. RochePH, Figarella-BrangerD, RegisJ, PeragutJC . Cauda equina paraganglioma with subsequent intracranial and intraspinal metastases . Acta Neurochir (Wien) . 1996; 138 : 475-479 .

  5. StrommerKN, BrandnerS, SariogluAC, SureU, YonekawaY . Symptomatic cerebellar metastasis and late local recurrence of a cauda equina paraganglioma. Case report. J Neurosurg . 1995; 83 : 166-169 .

  6. SonnelandPR, ScheithauerBW, LeChagoJ, CrawfordBG, OnofrioBM . Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology andultrastructure . Cancer 1986; 58 : 1720-1735 .

  7. Achilles E, Padberg BC, Holl K, Klöppel G, Schröder S. Immunocytochemistry of paragangliomas--value of staining for S-100 protein and glial fibrillary acid protein in diagnosis and prognosis. Histopathology.1991;18:453-8.