A 44-Year-Old Man With Progressive Weakness and Back Pain

Benjamin O. Cornwell, DO; Jignesh Modi, MD

Disclosures

November 26, 2018

Discussion

The diagnosis of epidural cavernous hemangioma was made by pathology following surgical removal of the mass. Cavernous hemangiomas within the epidural space of the spine are exceedingly rare and can only be confidently diagnosed in this region of the spine by histology. The patient's symptoms, backache, and progressive weakness and numbness in the lower extremities are a common presentation.[1] Additionally, the findings on MRI, including the homogeneous isointense signal intensity on T1, homogeneous high signal on T2, and uniform homogeneous postcontrast enhancement are typical for an epidural cavernous hemangioma.[1,2]

Pure epidural cavernous hemangiomas are rare and are believed to only comprise 4% of all epidural tumors and 12% of all intraspinal hemangiomas.[2] The cavernous hemangioma is a conglomeration of closely packed, dilated, thin-walled vessels that lack a true elastic lamina. The tumor has no malignant potential, but it causes problems secondary to mass effect on the spinal cord or nerve roots. Symptoms of spinal epidural cavernous hemangiomas include spinal cord syndromes and radiculopathies.[3] The affected part of the neuroaxis depends on where the tumor is located within the spine.

These tumors have been documented to originate in the cervical, thoracic, and lumbar spine.[3] They also may grow within the ventral, dorsal, or lateral epidural space and involve the adjacent neuroforamina. Cavernous hemangiomas also have a propensity to bleed, and acute hemorrhage can cause acute compression of the spinal cord leading to acute onset of symptoms. As in this case, patients can present with more chronic symptoms if the cavernous hemangioma grows slowly and is not accompanied by hemorrhage. Patients may also suffer from bladder dysfunction; however, this is not as common as pain syndromes or sensorimotor paresis.[3]

The differential diagnosis of an epidural cavernous hemangioma includes meningioma, lymphoma, schwannoma, angiolipoma, disk herniation, synovial cysts, granulomatous infection, pure epidural hematoma, and extramedullary hematopoiesis.[2] The combination of history, physical exam findings, laboratory data, and MRI can be used to narrow the differential. History can help distinguish epidural cavernous hemangioma from certain other lesions. For instance, a history of trauma, coagulopathy, or intervention would favor a pure epidural hematoma. If a patient reports pain with lifting or exertion, disk herniation would be more likely.

The physical examination may be helpful because patients with epidural infection may present with fever and tenderness to palpation. Patients who have conditions such as sickle cell disease may be more likely to develop extramedullary hematopoiesis, and there should be associated signal abnormalities in the adjacent bone marrow on MRI. Although pure epidural hemangiomas are rare, findings that may help to distinguish these lesions conditions, such as disk prolapse, more common meningiomas, and nerve sheath tumors include "its ovoid shape, uniform T2 hyperintense signal, and lack of anatomic connection with the neighboring intervertebral disk or the exiting nerve root."[4,5]

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