Single staged complete length excision of the holocord ependymoma: Team work.

The authors present a case of a 15-year-old male patient who presented with gradually progressive quadriparesis for 3 years. Magnetic resonance imaging of the spine was suggestive of heterogeneously enhancing mass lesion extending from cervicomedullary junction to conus. This holocord spinal tumor was excised in a single stage with standard microsurgical technique. In immediate postoperative period, the patient had deterioration in power in both lower limbs which improved in follow-up at 6 months. Histopathology of the tumor was suggestive of ependymoma. Holocord ependymoma is a rare entity; until now, only six cases have been described in the literature. To the author's best knowledge, this is only the second case of holocord ependymoma excised in a single stage.

Introduction

Ependymoma is the most common spinal cord tumor in adults but in pediatric population astrocytoma is 2–4 times more common. Spinal cord tumor which extends from cervicomedullary junction to conus is known as holocord tumor or pan spinal tumor.[1] The first case of holocord ependymoma was reported by Cushing in 1927;[2] until now, 6 cases of holocord ependymoma has been reported in literature.[3456] To the author's best knowledge, this is the 7th case of holocord ependymoma. In this paper, we present our case and discuss diagnosis, treatment, and prognosis.

Case Report

The 15-year-old male patient presented with pain in nape of neck for 3 years and gradually progressive quadriparesis for last 9 months with early bladder and bowel involvement. On examination, tone was decreased in both upper limbs and increased in both lower limbs with grade 4/5 power in all four limbs. Deep tendon reflexes were absent in both upper limbs and exaggerated in both lower limbs, there was no obvious sensory loss, and posterior column were impaired in both lower limbs. Magnetic resonance imaging (MRI) shows intramedullary mass extending from cervicomedullary junction to conus [Figure 1]. Mass was hypointense and hyperintense on T1-weighted images and T2-weighted images, respectively, with heterogeneous contrast-enhancement on contrast MRI [Figure 2]. There was a polar cyst above the mass lesion. The patient was planned for the decompression at maximal dilated segment but during the surgery, it was realized that tumor was having a good plane of cleavage and was easily suckable. The operating team was well-equipped with pneumatic drill for further laminectomy/laminoplasty. The team of neuroanesthesia was ready to handle the further extend of surgery by 2–3 h. Patient undergone excision of the tumor in a single stage, laminoplasty at C2–D3, and D7–L1, D4–D7 laminectomy was done. Near total excision of the tumor was done; small portion of tumor at superior pole near the obex was left behind. In postoperative period, patient had deterioration in power in both lower limbs with power in the right side 0–1/5 and on left side 3/5. On follow-up at 6 months, the patient had significant improvement in power in both lower limbs, and now patient is able to ambulate independently.

Figure 1

T2-weighted images sagittal sequence showing heterogeneously hyperintense intramedullary tumor extending from cervicomedullary junction to conus region

Figure 2

(a and b) T1-weighted images contrast sequences showing heterogeneously enhancing intramedullary tumor

Discussion

Ependymoma is the most common intramedullary tumor in adults followed by astrocytoma, which is more common in children and young adults. Holocord ependymoma is extremely rare - only six cases of holocord ependymoma have been reported in literature. To the best of our knowledge, this is the seventh case of holocord ependymoma reported in literature and only second case operated in a single stage.

Ependymoma consists of 4–6% of primary central nervous system tumors and 1/3 of these tumors arise in the spinal cord.[7] In the spinal cord the most common location for ependymoma is lower spine and conus region followed by cervical spine. Ependymomas are nonencapsulated glial origin tumors which are mostly benign. These tumors are usually noninfiltrative and slow growing and usually present with prolonged symptoms before diagnosis.

Ependymomas are usually associated with cysts which maybe tumoral or nontumoral. Tumoral cyst found inside the tumor requires excision, but nontumoral cyst found at rostral and caudal ends of tumor do not require excision as they decrease in size when tumor is completely removed.[8] In the present case of holocord ependymoma tumor was associated with small polar cyst at rostral and caudal end of solid component.

MRI is the best investigation for the evaluation of intramedullary spinal cord tumors. Ependymoma usually appear hypointense to isointense on T1 weighted images and hyperintense on T2-weighted images. Heterogeneous signal may appear due to cystic or hemorrhagic changes. Ependymoma usually shows homogenous contrast enhancement. Tumoral cyst can be identified from nontumoral cyst as its wall shows enhancement on contrast study.

Ependymoma usually has a good plane of cleavage from surrounding neural tissue so gross total removal can be achieved without any additional neurological deficit to patient. In patients with ependymoma, outcome depends upon the extent of tumor removal and functional status of patients at the time of surgery. So for better results early surgery is advocated because postoperative functional status depends on preoperative functional status.

Peker et al.[9] in their series correlated various factors such as tumor length, tumor width, cyst length, and the presence of cord edema, with neurological status. They found that patients with wider tumor diameter with tumor cord ratio more than 0.8 were associated with poor preoperative neurological status and poor postoperative outcome. Tumor length did not correlate with poor outcome but was associated with postoperative dysesthesia. Aghakhani et al.[10] described 82 consecutive patients with intramedullary ependymoma and focused on 10 patients who were without neurological deficit. Gross total excision was done in 9 patients and in 1 patient subtotal excision was done. Seventy percentage patients worsened in immediate postoperative period however on follow-up 90% patients returned to preoperative neurological status. Surgery was able to resolve their preoperative complaints such as back pain in only 30% cases, in 60% cases it stabilized, and worsened in 10% cases. Hence, surgery should be carefully considered in patients with spinal cord ependymoma with no objective neurological deficit. Management of holocord spinal tumors remains a debated topic as optimal treatment has not been described in literature. Until now, the same principle as used in intramedullary tumor is used for the management of holocord tumors. Early surgery is key to prevent further neurological deterioration and possibly improving neurological function.

The choice of laminoplasty versus laminectomy depends upon availability of instruments and surgeon choice. The advantage of laminoplasty over laminectomy has been proven in literature.[11] In this patient, we have done the laminoplasty at D7–L1, and once the tumor was found to be easily suckable and having good plane of cleavage the exposure was extended up to D4 by laminectomy and further extension was done by C2–D3 laminoplasty. At the end of 6 months follow-up, the patient did not have any deformity.

Tobias et al.[12] described 13 cases of long segment intramedullary tumors which were excised either in single stage or staged manner (in 2 patients). Modified McCormick score (MMS) had worsened in only two (15%) patients, improved in three (23%) patients, and remained stable in seven (54%) patients compared to preoperative MMS and one patient of anaplastic oligodendroglioma died.

Some author advised holocord tumors should be excised in staged manner and for staging two variables should be considered size of tumor and age of patient. If in an adult patient the tumor is extending more than 15 vertebral levels, surgery can be staged.

We describe a pediatric patient with holocord ependymoma which was excised in a single stage. Patient in the preoperative period presented to us in modified McCormick grade 2, in immediate preoperative period patients neurological status worsened to modified McCormick grade 4. At 6 months follow-up, the patient shows improvement now has grade 2 status.

Conclusion

Holocord ependymoma is a rare entity with only 6 cases reported in literature. Specific treatment protocol has not been described in literature for these long segment tumors. After reviewing the literature, we proposed following parameters to be assessed carefully to decide for single versus staged surgery. Postoperative outcome of these patients with long segment intramedullary tumor depends upon patient's preoperative modified McCormick grade, plane of cleavage of the tumor, and suckability from surrounding tumor tissue, equipment to facilitate fast surgery (surgeon friendly operating microscope, drill, ultrasonic aspirator, and fluid warmer), and good neuroanesthesia support.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest