Multiple intradural-extramedullary ependymomas: proven dissemination by genetic analysis

Abstract

This 45-year-old woman was admitted with neck and back pain and difficulty in ambulation that had been progressively worsening for 2 years. Admission MR imaging revealed a cervicomedullary junction tumor and 2 intradural-extramedullary spinal tumors located separately at the levels of T5-6 and T8-9. All masses were successfully resected in a 2-stage operation. Histopathological examination of the surgical specimens revealed that all the lesions were ependymomas. Genetic analysis was performed to determine if the tumors were related. Conventional cytogenetics, multiplex fluorescence in situ hybridization (M-FISH), interphase-FISH specific to 22q11, and epidermal growth factor receptor loci analyses of the tumor samples revealed that the lesions originated from the same primary tumor. Although 3 simultaneous tumors in different compartments of the neural axis were diagnosed as ependymoma by histopathological examination, it was not possible to be sure if their multiplicity was due to spread of tumor cells via CSF or if it was due to multicentric foci. Thus, genetic analysis of the tumor samples is essential to confirm the exact mechanism of development of multiple ependymomas.

Intradural Extramedullary Ependymoma with Spinal Root Attachment: A Case Report

“…Spinal cord ependymoma is an intradural intramedullary lesion most frequently located at the conus medullaris or filum terminale. Only rare reports exist of spinal cord ependymomas in IDEM locations; only 17 cases of spinal cord ependymomas in IDEM locations exist in the relevant literature 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 11 , 12 , 13 , 15 , 16 , 18 , 19) .…”

“…Spinal cord ependymoma comprises less than 2% of all central nervous system neoplasms, 15% of spinal cord tumors, and up to 60% of spinal cord gliomas 20) . It is an intramedullary lesion and there have been reports of only 17 cases of ependymomas arising in IDEM locations worldwide 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 11 , 12 , 13 , 15 , 16 , 18 , 19) . This is the fourth-reported case of primary IDEM ependymoma in South Korea 10 , 14 , 17) .…”

Multifocal intradural extramedullary anaplastic ependymoma of the spine

“…Non-myxopapillary IDEM ependymomas are very rare tumours with only 33 cases reported (25 female, 7 male). Only five cases were multifocal ( Table 1), and only one of these was anaplastic (1,(4)(5)(6). The most common site for IDEM ependymomas is the thoracic spine (n=16), followed by cervical (n=8) and lumbosacral (n=4).…”

Isolated Lumbar Spinal Nerve Root Myxopapillary Ependymoma: Case Report

“…The majority of these are of the myxopapillary subtype [1][2][6][7]. However, the overall incidence of primary intradural extramedullary ependymomas is quite low with less than 20 cases reported in the literature [1][2][8][9][10][11][12][13]. Some of these were multifocal, while others were myxopapillary ependymomas.…”