Giant invasive spinal schwannoma in children: a case report and review of the literature

Abstract

IntroductionGiant invasive spinal schwannoma is defined as a tumor that extends over two or more vertebral levels, erodes vertebral bodies, and extends into the extraspinal space disrupting myofascial planes. Because of its rarity, there have been few published reports describing clinical features and surgical outcomes, especially in the pediatric patient population.Case presentationWe analyzed the medical record, pathologic findings, and radiographic studies of a 14-year-old Hispanic boy who presented to Texas Children’s Hospital with a three-month history of progressive spastic paraparesis. Preoperative computed tomography and magnetic resonance imaging reports showed a large mass lesion centered at the left T7-8 neural foramen with intra- and extraspinal extension, resulting in severe spinal cord compression and vertebral body erosion, and protrusion into the retropleural space and descending aorta. Our patient underwent a single-stage posterior approach for complete resection of the tumor with reconstruction and stabilization of the vertebral column. The pathological examination was consistent with schwannoma. At the six-month follow-up, our patient had resolution of preoperative symptoms and remains neurologically intact without any radiographic evidence of recurrent tumor.ConclusionTo the best of our knowledge, our case represents the fourth child with giant invasive spinal schwannoma reported in the literature. We describe our case and review the literature to discuss the aggregate clinical features, surgical strategies, and operative outcomes for giant invasive spinal schwannoma in the pediatric age group.

The association of a hemivertebra with a large dumbbell‐shaped tumor: A potential embryological explanation

“…Despite the absence of NF1 features in our patient and the rarity of schwannomas in NF1 cases, schwannoma is likely a consequence of an increased recruitment of neural crests cells during embryogenesis and/or loss of tumor suppressor activity of one or both genes (Brannan et al, 1994;Reith and Goldblum, 1996). Moreover, giant spinal dumbbell schwannomas reported in pediatric age groups further support our hypothesis of a shared pathogenesis with a hemivertebra (Inaoka et al, 2001;Sridhar et al, 2001;Yu et al, 2012;Vadivelu et al, 2013). Lastly, MRI has proven to be a valuable tool for detection of neural abnormalities in patients with congenital scoliosis secondary to an isolated hemivertebra with an accuracy of 65%, sensitivity of 59% and specificity of 87% increasing the prevalence of such anomalies with hemivertebra from 8% to 28% (Belmont et al, 2004).…”

Giant schwannoma of thoracic vertebra: A case report

“…Therefore, doctors should fully inform patients and families to be prepared for all aspects of multiple surgeries when they are facing this type of disease. For example, some surgeries are too difficult and too challenging to try to remove the entire tumor completely within a single surgery, and incomplete resection cases tend to increase the chances of recurrence[ 13 ].…”

Giant lumbar dumbbell extradural schwannoma in a child

“…[1] Contrast-enhanced MRI images of the spine will delineate most of the details of the tumor and its relationship to the surrounding structures including neural elements. [1912131415] Radiographs and computed tomography scan with bone window will further provide greater details of any bony erosion or destruction and thus potential to develop spinal stability. [11415] In addition, those patients whose imaging showed considerable vertebral erosion are at greater risk for spinal instability and may need spinal instrumentation to address the instability.…”