Intramedullary inclusion cysts of the cervicothoracic junction

Report of two cases in adults and review of the literature

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✓Intramedullary inclusion cysts are extremely rare within the rostral spinal cord. In this case report the authors outline the clinical features and surgical treatment of one dermoid cyst and one epidermoid cyst of the cervicothoracic junction. The authors also include a relevant literature discussion regarding the treatment and the embryological origin of these lesions.

Abbreviation used in this paper:MR = magnetic resonance.

Article Information

Address reprint requests to: Paul McCormick, M.D., Neurological Institute, Columbia University, 710 West 168th Street, New York, New York. email: pcm6@columbia.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. a–c: Serial midsagittal MR images of the cervical spine demonstrating a well-circumscribed, intramedullary spinal cord mass lesion at the C7–T1 level, with multiple associated areas within the center of the cord up to C-2. The lesion is heterogeneous in signal intensity but predominantly bright on T1-weighted sequences (a) and relatively bright on T2-weighted sequences (b). The fat-suppressed T2-weighted sequence (c) confirms the lipid content in this lesion. d and e: Axial T2-weighted MR images confirming the intramedullary location of this complex mass and showing incompletely fused spinous processes at C-7 and T-1. Given the signal characteristics and the lack of any significant enhancement (images not shown), the most likely diagnosis is an intramedullary dermoid. If a diffusion weighted image of the cord were obtained, one would expect the lesion to be bright and demonstrate areas of restricted diffusion, a characteristic appearance of these true ectoderm-lined inclusion cysts. f: Postoperative image revealing a cystic cavity after removal of the dominant mass along with residual elements.

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    Case 1. Intraoperative photographs obtained in a 23-year-old man with an intramedullary tumor. a: Incompletely fused spinous processes at C-7 and T-1 (arrows) were seen after subperiosteal dissection. b: After laminectomy, a bulge within the dura mater was apparent. c: Following durotomy a swollen cord with dorsal indentation was noted together with an intact pial margin overlying neural tissue and some fatty elements. d and f: A midline myelotomy was performed, revealing an encapsulated lesion filled with yellow cheesy material and hair. e: The lesion was cored out internally and the tumor capsule, which was densely adherent to neural tissue, was left behind.

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    Case 2. a and b: Sagittal midline MR images of the cervical spine demonstrating a complex mass lesion involving the cervical spinal cord extending from C3–T1, with focal enlargement centered at the C-6 and C-7 vertebral body levels. The mass is mostly isointense to neural tissue on T1-weighted sequences (a) but hyperintense on T2-weighted sequences (b), with heterogeneously scattered foci of increased signal on the T1-weighted images, probably indicating fatty proteinaceous material, less likely blood products (methemoglobin). c–e: Fat-suppressed T2-weighted images showing small dark specks of high lipid content. The osseous spinal canal is focally expanded at C6–7 (chronic slow-growing lesion). The mass is intradural and extramedullary in location with a possible intramedullary component that is difficult to reconcile on axial imaging (d and e). Imaging also shows an interesting defect along the posterior aspect of the epidural space at C-7 in connection with the osseous spinous process at that level, possibly representing the site of the failure of surface ectoderm to separate from underlying structures. f: Postoperative image revealing resection of the dominant mass with residual capsular components and unresected satellite lesions.

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    Case 2. Intraoperative photographs obtained in a 61-year-old woman with an intradural lesion. a: A patch of hypertrichosis (arrow) was noted off-midline at around the T-4 spinous process level. b: After laminectomy and mid-line durotomy, a shiny, glistening intramedullary mass with a large exophytic component was readily apparent. c: The extramedullary portion was excised, and internal debulking was performed on the intramedullary portion of the lesion whose capsule proved to be densely adherent to neural tissue and could not be safely dissected free.

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