Resection of Bilateral Symmetrical Multiple Level Cervical Ganglioneuroma in a 43-Year-Old Man, a Probable Case of Neurofibromatosis Type-1: Report of a Case and Review of Literature

Abstract

Ganglioneuroma is a benign tumor, originating from sympathetic nervous system. Intradural and dumbbell shape spinal ganglioneuroma has been reported in the literature. In this study, we intend to present our case, a 43-year-old man with multiple cutaneous dimples-probably a Neurofibromatosis type-1 (NF-1) case-and subacute myelopathy, who presented with bilateral symmetric dumbbell shape C2/C3 and C4/C5 intradural extramedullary tumors. After resection, the pathologic feature was revealed as ganglioneuroma. We also reviewed the literature for similar cases, which revealed our case to be the 9th bilateral and symmetrical spinal GN, all of which in cervical region; the 5th involving multiple level (the 3rd multiple bilateral symmetrical involvement), the 3rd extending intradurally, and the first case of involving all cervical nerve root ganglions in different sizes. Bilateral symmetrical spinal GNs have also appeared to have different body location, geographic, and gender distribution.

Figure 1

Diffuse cutaneous nodules and café-au-lait spots.

Figure 2

Bilateral symmetrical C2 GN- axial T2WI at C1-C2.

Figure 3

Sagittal T2WI showing expanded intervertebral foramina mostly at C1-C2 level (a) and dorsal intradural part of bilateral C2 ganlion GN, compressing spinal cord and causing signal change at highest cervical spinal cord segments.

Figure 4

Sagittal-axial T2WI cross view of C4-C5 level showing bilateral symmetrical intradural ventrolateral C4 GN, compressing spinal cord.

Figure 5

Sagittal spiral CT scan of cervical spine, showing C3-C4 partial fusion (a) and expanded intervertebral foramina mostly at C1-C2 level (b).

Figure 6

Sagittal-axial cross-view of postoperative T2WI of C1-C2 level showing dorsal CSF collection (a), hemovaccum tube (b), Gelfoam covering dura (c), and no remnant of bilateral C1-C2 GNs.

Figure 7

Sagittal-axial cross-view of postoperative T2WI of C4-C5 level showing spinal cord decompression and no remnant of bilateral intradural C4-C5 GNs.

Figure 8

Histopathology of the tumor. (a, b) Histopathological sections of the tumor show bipopulation of the tumor cells, sheet of spindle shape bland looking cells mixed with scattered large spherical ganglion cells (^∗). Neither necrosis nor mitosis is seen in the tumor (×100, ×400, hematoxylin and eosin). (c) Immunohistochemical stain for S-100 shows diffuse, strong immunopositivity in both neuroma and ganglion cell component (^∗) (×100). (d) Immunohistochemical stain for chromogranin shows immunostaining in ganglion cell component (^∗) only (×100).