Intramedullary cervical spinal cord teratoma

Abstract

Background: Intramedullary cervical spinal cord teratomas (ICTs) are extremely rare, and diagnosis and treatment are challenging. We conducted a systematic review of the literature on the diagnosis and treatment of ICT.

Method: The presentation, imaging manifestations, diagnosis, management, surgery findings, prognosis and histology were reviewed following Preferred Reporting Items for Systematic Reviews and Meta Analyses guidelines. English-language studies and case reports published from inception to 2018 were retrieved. Data on presentation, imaging characteristics, diagnosis, management, surgery findings, outcomes, and histopathology were extracted.

Results: Ten articles involving 10 patients were selected. The lesions were located in the upper cervical vertebrae in 4 cases, whereas in the lower cervical vertebrae in the remaining 6 cases. In 5 cases, the lesions were located on the dorsal side of the spinal cord, and in the center of the spinal cord in the remaining 5 cases. Quadriparesis (60%), paraplegia (30%), monoplegia (10%), and neck pain (50%) were the main presentations. The lesion appeared as a intramedullary heterogeneous signal during an MRI scan, and the lesion signal would be partially enhanced after the contrast medium was applied. All patients underwent surgical intervention through a posterior approach. Neurological function improved postoperatively in all patients. Two patients with pathology confirmed to be immature teratomas experienced recurrence.

Conclusion: ICTs are extremely rare entities that are mainly located in the center or dorsal part of the spinal cord which mainly manifest as quadriplegia and neck pain. MRI is a useful modality that provides diagnostic clues. Surgery from a posterior approach is the primary treatment, and the effect of adjuvant therapy remains uncertain. The prognosis is mainly related to the pathological nature of the tumor and not the method of resection.

Figure 1

Flow diagram showing selection of studies.

Figure 2

Preoperative plain computed tomography scan shows a hypodense lesion located at C1–C2 levels without calcification (A). Preoperative axial T1-weighted MRI shows the bad-delineated mass of hypointense signal located exclusively in the cervical spinal cord (B). Sagittal MRI (C and D) depicts the fusiform-shaped and eccentrically located intramedullary tumor at the C1–C2 levels, appearing hypointense on T1-weighted and mixed signal intensity on T2-weighted images with adjacent spinal cord edema. The cystic lesion within the spinal cord distal to the mass is quite visible (see white dovetail arrow). (E and H) Immediately postoperative Gd-enhanced T1-weighted sagittal and axial MRI shows gross-total removal of the tumor (black arrowhead). Sagittal (F and G) and axial (I and J) on the 10th month follow-up MRI images with contrast reveal two metastatic extramedullary lesions at C4–C6 and T11–12, respectively, appearing highly enhanced with heterogeneous features. No changes are observed in the previous operation site (see white double arrow). (K) Postoperative T1-weighted sagittal MRI with contrast depicts near-total resection of the cranial and caudal tumors, respectively. Of note, numerous additional disseminated enhancing foci nodules are seen along the leptomeninges and dura, likely representing metastatic drop lesions (long white arrows). (Han Z, Du Y, Qi H, Zheng S, Yin W. Cervical intramedullary immature teratoma with metastatic recurrence in an adult. Spinal Cord Ser Cases. 2015;1:15006.).

Figure 3

Hematoxylin-eosin stained section showing typical histological appearances. (A) Overview demonstrating cartilage (top left) adipose tissue, vessels, and nerves (center) and neurological tissue (bottom right) (Magnification [mag.]) 12.5×); (B) cartilage, adipose tissue, and pacini corpuscles (mag. 100×);(C) blood vessels and nerves (mag. 100×); (D) neuroglial tissue (mag. 200×); (E) large mature ganglion cells (mag. 200×); (F) serous/mucus glands (mag. 400×); and (G) cyst with cuboidal epithelium (mag. 400×). (Arvin B, Pohl U, David K. Intramedullary cervical teratoma in an adult. The Spine J. 2009;9(5):e14-8.).