Intradural Intramedullary and Intradural Extramedullary Lipomas Unassociated with Spinal Dysraphism

Spinal cord intradural, intramedullary lipomas are rare entities, usually found along the dorsal aspect of the thoracic spine and in the midline.12,23 In one early report, they were found in decreasing frequency over the cervical-thoracic spine and over the cervical spine. In addition, there are eight reports of intramedullary spinal cord lipomas affecting nearly all levels of the spinal cord, known as holocord lipomas.12

Patients with spinal cord lipomas most often have slowly progressive signs of spinal cord compression. The most commonly reported presenting symptoms are those of an ascending spastic motor weakness in one or both legs.10,12,23 The clinical presentation and examination findings reflect the location and size of the spinal cord lipoma.

Spinal cord lipomas are uncommon, with approximately 200 reported cases, predominantly of the intradural or extra-medullary type, having been identified in the cervical and upper thoracic spine since the first report by Gowers in 1876.12 Spinal cord lipomas account for 0.6% to 1% of all intraspinal tumors.10,12 They are found with equal frequency in males and females and are presumed to be present from birth. However, because of their benign, slow-growing nature, these lesions often do not present until the second or third decades of life.12,23 As with other lipomatous tumors, the diagnosis may be suggested with the use of plain radiographs, but CT or MRI is the study of choice.

The pathogenesis of spinal cord lipomas may be related to the incorporation of multipotential mesodermal cells present between days 18 and 27 of embryogenesis.23 Like intracranial lipomas, spinal cord lipomas are composed of mature adipocytes with a collagenous matrix and capsule, which is reflected in their appearance on CT and mRi. Spinal cord lipomas have also been noted to enlarge and regress with changes in total body fat.23 Dietary modification has been proposed as a treatment modality for spinal cord lipomas, but surgical debulking remains the mainstay of therapy, if needed.

Given their characteristic location, spinal cord lipomas are commonly accessible by way of a posterior approach. The goal of surgery is not total resection, because multiple authors have noted the lack of identifiable planes between the lipoma and the spinal cord tissue. Instead, the goal should be decompression and subtotal resection, with careful attention to the spinal cord elements. Multilevel laminectomies, covering the area of involvement, are performed. There are no reports of radiation therapy or chemotherapy used as adjuncts in the treatment of spinal cord lipomas. Recurrence is rare, because these tumors do not contain neoplastic elements, and there are no reports of malignant transformation.

Outcome data are limited, but in a report on eight patients with spinal cord lipomas who underwent surgical decompression, all patients demonstrated improved muscle strength, one third had improved sensation, one fourth had improved pain, and one fourth had improved bladder function.3 These improvements stand in sharp contrast to the postoperative outcomes seen in patients with lipomyelomeningoceles, discussed later.

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