Original articleMalignant peripheral nerve sheath tumor in spine: imaging manifestations☆
Introduction
Malignant peripheral nerve sheath tumor (MPNST) is also called nerve sarcoma or nerve fiber sarcoma. It is a relatively rare soft tissue malignant tumor with low incidence. The incidence is 0.001% in common clinical population and 4.6% in multiple neurofibromas [1], [2]. Prognosis of MPNST is poor, and 60% of the MPNST patients will die. In particular, the mortality is 80% if MPNST occurs at the spinal column. Most of reports currently are pathological articles and clinical case reports, and most of them are MPNST in the thorax, abdomen or extremities. Imaging report of spinal MPNST is very rare, so there is no sufficient understanding about it. Moreover, large tumor size, conspicuous bone destruction and mass center not limited to the neural foramen lead to great difficulty in defining the origin of the tumor, whether from soft tissue or vertebral body, and is thus easy to be misdiagnosed. In this study, we reviewed the computed tomography (CT) and magnetic resonance imaging (MRI) manifestations of spinal MPNSTs aiming to improve the accuracy of preoperative diagnosis. One case of an unusual child MPNST and one case of a rare subarachnoid MPNST are included in this article.
Section snippets
Patient data
From March 2004 to June 2009, 12 patients with MPNST in spine that had been proven by pathological results postoperatively visited the Department of Radiology in our hospital for CT and/or MRI scan, including six males and six females with age ranging from 7 to 71 years (median age of 40.5 years). There were five cases in the cervical spine, three cases in the thoracic spine, two cases in the lumbar spine and two cases in the lumbar–sacral spine. Three of the patients visited a doctor due to
Distribution
There were five cases of MPNST in the cervical spine, three cases in the thoracic spine, two cases in the lumbar spine and two cases in the sacral spine.
Shape
Ten cases were irregularly lobulated and two cases were circular.
Size
The maximal diameter of 10 cases was larger than 5 cm (the maximal one was 130×130×170 mm) and was less than 5 cm in two cases.
Boundary
Except for one case that was well demarcated by surrounding celiac fat, another case by surrounding bone and one tumor inside the vertebral canal (Fig. 1
Clinical and epidemiological characteristics
Malignant peripheral nerve sheath tumor, also called malignant nerve sheath tumor (malignant schwannoma), neural sarcoma or nerve fiber sarcoma, is a malignant tumor derived from peripheral nerve or malignant tumor manifested as nerve sheath differentiation, except for tumor originated from epineurium and peripheral vessel system. Based on the WHO classification of tumors, it belongs to III–IV [1]. MPNST usually affects adults ranging in age from 20 to 50 years, without sex difference, and it
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