Original Article

Modern Pathology (2005) 18, 710–718, advance online publication, 3 December 2004; doi:10.1038/modpathol.3800350

Venous congestive myelopathy: a mimic of neoplasia

Fausto J Rodriguez1, Brian A Crum2, William E Krauss3, Bernd W Scheithauer1 and Caterina Giannini1

  1. 1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
  2. 2Department of Neurology, Mayo Clinic, Rochester, MN, USA
  3. 3Department of Neurosurgery, Mayo Clinic, Rochester, MN, USA

Correspondence: Dr C Giannini, MD, PhD, Department of Laboratory Medicine and Pathology, 200 First Street SW, Mayo Clinic, Rochester, MN 55905, USA. E-mail: Giannini.Caterina@Mayo.edu

Received 25 August 2004; Revised 7 October 2004; Accepted 10 October 2004; Published online 3 December 2004.

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Abstract

Venous congestive myelopathy is a progressive disorder frequently associated with arteriovenous fistulas, usually dural. By causing diffuse spinal cord enlargement and enhancement on imaging, it may simulate a neoplasm and prompt a biopsy. We evaluated the biopsy findings in seven such patients (M=5, F=2, mean age 59plusminus11 years) who presented variably with progressive lower extremity weakness (86%), bowel and bladder dysfunction (86%), sensory changes (86%) or pain (29%). Preoperative magnetic resonance imaging showed spinal cord enlargement with T2-hyperintensity (86%) and contrast enhancement (57%) at the cervical (14%), thoracolumbar (57%), and/or conus medullaris (57%) level. Prebiopsy spinal angiogram, performed in two patients, was negative. Spinal cord biopsy showed architecturally distorted parenchyma with gliosis and thick hyalinized vessels (100%), variable myelin loss (71%), mild glial atypia (57%), hemosiderin deposition (71%), Rosenthal fibers (43%), vascular thrombosis (29%), and necrosis (29%), features highly suggestive of venous congestive myelopathy. Postbiopsy spinal angiograms were performed in five patients. A dural arteriovenous fistula was identified by selective angiography in three patients, including the two with a negative preoperative angiogram. Additional postbiopsy angiographic studies in two patients were negative, and two patients were followed up without angiography. Mean follow-up after biopsy was 13.6 months. Histologic changes characteristic of venous congestive myelopathy may be seen in spinal cord biopsies with or without an associated fistula. Recognition of this entity by surgical pathologists is important, leading to the correct identification of a non-neoplastic lesion as well as of a surgically treatable disease.

Keywords:

venous congestive myelopathy, Foix–Alajounine syndrome, spinal dural arteriovenous fistula, spinal cord tumor, progressive myelopathy

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