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Original Article

Current Trends in the Surgical Management of Intramedullary Tumors: A Multicenter Study of 1,033 Patients by the Neurospinal Society of Japan

Neurospine 2022;19(2):441-452.
Published online: June 30, 2022

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5Department of Neurosurgery, Dokkyo Medical University, Tochigi, Japan

6Department of Neurosurgery, Shinshu University, Shinshu, Japan

7Department of Neurosurgery, Shin-Aikai Spine Center, Katano Hospital, Osaka, Japan

8Department of Neurosurgery, Osaka Medical and Pharmaceutical University, Osaka, Japan

9Department of Neurosurgery, Sapporo Azabu Neurosurgical Hospital, Sapporo, Japan

Corresponding Author Toshiki Endo Division of Neurosurgery, Tohoku Medical and Pharmaceutical University, Fukumuro, Miyagino, Sendai 983-8536, Japan Email: endo@nsg.med.tohoku.ac.jp
• Received: February 15, 2022   • Revised: April 27, 2022   • Accepted: April 28, 2022

Copyright © 2022 by the Korean Spinal Neurosurgery Society

This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Current Trends in the Surgical Management of Intramedullary Tumors: A Multicenter Study of 1,033 Patients by the Neurospinal Society of Japan
Neurospine. 2022;19(2):441-452.   Published online June 30, 2022
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Current Trends in the Surgical Management of Intramedullary Tumors: A Multicenter Study of 1,033 Patients by the Neurospinal Society of Japan
Neurospine. 2022;19(2):441-452.   Published online June 30, 2022
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Current Trends in the Surgical Management of Intramedullary Tumors: A Multicenter Study of 1,033 Patients by the Neurospinal Society of Japan
Image Image Image
Fig. 1. Overall survival is demonstrated using Kaplan-Meier curves of those who underwent surgical interventions for intramedullary tumors.
Fig. 2. Overall survival is demonstrated using Kaplan-Meier curves for tumors with different histological diagnoses. Astrocytomas had worse survival than the other histological types.
Fig. 3. Time course of neurological function expressed using the modified McCormick scale. The proportions of patients with McCormick grades I and II decreased immediately postoperatively (at discharge). However, the proportions increased at 6 months postoperatively. The surgical results were better than the preoperative status. The improved functional status was maintained thereafter.
Current Trends in the Surgical Management of Intramedullary Tumors: A Multicenter Study of 1,033 Patients by the Neurospinal Society of Japan
Variable Value
Age on admission (yr) 48.4 (0–88)
Sex
 Men 560 (54.2)
 Women 473 (45.8)
Cases with hereditary disease
 Neurofibromatosis, type 1 6 (0.6)
 Neurofibromatosis, type 2 16 (1.6)
 von Hippel-Lindau disease 74 (7.1)
Mean duration of the symptoms (mo) 22.8
Clinical presentations
 Head, neck or back pain 426 (41.2)
 Limb pain 424 (40.9)
 Limb paresthesia 882 (85.1)
 Limb weakness 686 (66.2)
 Gait disturbance 593 (57.2)
 Bladder/bowel disturbance 342 (33.0)
Characteristic Value
Tumor levels
 Cervical 465 (44.9)
 Cervical and thoracic 127 (12.4)
 Thoracic 333 (32.2)
 Thoracic and lumbar 108 (10.5)
Tumor characteristics
 Cystic 85 (8.2)
 Solid 518 (50.1)
 Mixed 265 (25.6)
 Hemorrhagic 156 (15.1)
 Unclassified 9 (0.9)
Tumor length 39.1 (3–500)
Intracranial tumors, concomitant 130 cases
Type No. of cases (%)
Ependymoma 361 (35.0)
Hemangioblastoma 196 (18.9)
Astrocytoma 168 (16.2)
Cavernous malformations 160 (15.4)
Subependymoma 22 (2.1)
Metastasis 21 (2.0)
Capillary hemangioma 16 (1.5)
Lymphoma 13 (1.3)
Schwannoma 12 (1.2)
Embryonal tumors (medulloblastoma, PNET, ATRT) 8 (0.8)
Solitary fibrous tumor 5 (0.5)
Germ Cell tumors (germinoma, mature teratoma, yolk sac tumor) 5 (0.5)
Gangliocytoma, Ganglioglioma 5 (0.5)
Neurenteric cyst 4 (0.4)
Rosette-forming glioneuronal tumor 3 (0.3)
Malignant peripheral nerve sheath tumor 2 (0.2)
Neurofibroma 1 (0.1)
Pineoblastoma 1 (0.1)
Paraganglioma 1 (0.1)
Glioependymal cyst 1 (0.1)
Meningioma 1 (0.1)
Granuloma 2 (0.2)
Sclerosing epithelioid fibrosarcoma 1 (0.1)
Dermoid cyst 1 (0.1)
Central neurocytoma 1 (0.1)
Undiagnosed 22 (2.1)
Characteristic Value
Surgical approaches
 Posterior 1,023 (99.0)
 Anterior 10 (1.0)
Surgeons’ experience
 > 10 years 976 (94.5)
 < 10 years 57 (5.5)
Main operator
 Board-certified spine surgeons 912 (88.3)
Operation time (min) 399.9 ± 173.2
Blood loss (mL) 186.0 ± 247.0
Degrees of removal
 Total 672 (65.1)
 Subtotal 134 (13.0)
 Partial 146 (14.1)
 Biopsy 81 (7.8)
Total removals
 Ependymoma 270/361 (74.8)
 Hemangioblastoma 180/196 (91.8)
 Cavernous malformations 142/160 (88.8)
 Astrocytoma 18/168 (10.7)
 Others 54/126 (42.9)
Postoperative radiation
 Local 117 (11.3)
 Whole spine 13 (1.3)
Chemotherapy
 Temozolomide only 57 (5.5)
 Temozolomide and bevacizumab 24 (2.3)
Characteristic No. (%)
Symptoms at discharge
 Improved 286 (27.7)
 Unchanged 333 (32.2)
 Worsened 414 (40.1)
Modified McCormick Scales at discharge (comparison with the preoperative status)
 Improved 153 (14.8)
 Unchanged 646 (62.5)
 Worsened 234 (22.7)
Modified McCormick Scales 6 months after the operations (comparison with the preoperative status)
 Improved 251 (27.6)
 Unchanged 500 (54.9)
 Worsened 160 (17.6)
Complications
 CSF leak 28 (2.7)
 Postoperative hematoma 8 (0.8)
 Infection 9 (0.9)
 DVT, pulmonary embolism 2 (0.2)
Relapse of the tumors
 Local recurrence 42 (4.1)
 CSF dissemination 10 (1.0)
 Local recurrence and CSF disseminations at the same time 27 (2.6)
Variable Multivariable analysis
OR 95% CI p-value
Age 0.99 0.97–0.99 0.046
Male sex 1.29 0.75–2.22 0.36
Surgery
 Biopsy Reference
 Partial removal 0.64 0.32–1.28 0.21
 Subtotal removal 0.56 0.24–1.31 0.18
 Total removal 0.12 0.04–0.33 < 0.001
Modified McCormick Scales (III, IV, V) 4.82 2.62–8.88 < 0.001
Histopathology
 Ependymoma Reference
 Astrocytoma 6.20 2.53–15.20 < 0.001
 Cavernous malformations 0.30 0.04–2.44 0.26
 Hemangioblastoma 1.59 0.44–5.72 0.48
 Others 3.31 1.29–8.49 0.01
Locations 0.89 0.72–1.11 0.30
Hosmer and Lemeshow goodness of fit test 0.146
Variables Multivariable analysis
OR 95% CI p-value
Age 0.98 0.97–0.99 0.046
Male sex 1.02 0.73–1.44 0.90
Surgery
 Biopsy Reference
 Partial removal 1.04 0.47–2.31 0.92
 Subtotal removal 1.78 0.76–4.19 0.19
 Total removal 3.66 1.62–8.23 0.002
Modified McCormick Scales (I, II) 14.26 10.03–20.29 < 0.001
Histopathology
 Ependymoma Reference
 Astrocytoma 0.41 0.22–0.77 0.006
 Cavernous malformations 1.20 0.72–1.98 0.49
 Hemangioblastoma 0.60 0.35–1.05 0.08
 Others 0.60 0.35–1.05 0.08
Locations 0.91 0.79–1.05 0.21
Hosmer and Lemeshow goodness of fit test 0.469
Study Intramedullary tumor cases Complete resection Postoperative deterioration Prognostic factors
Cooper and Epstein, [12] 1985 29 Cases 72% 28% Satisfactory neurological status before surgery
-14 Ependymomas Histological type
-11 Astrocytomas Complete removal
Cristante and Herrmann, [17] 1994 69 Cases 55.1% 29.4%–31.5% Preoperative neurologic deficit
-34 Ependymomas
-28 Astrocytomas
Constantini et al., [10] 2000 164 Cases 76.8% 23.% Histological type
-19 Ependymomas Preoperative functions
-76 Astrocytomas Patients with shunts
Sandalcioglu et al., [13] 2005 78 Cases 83.3% 34.6% Preoperative neurological condition
-32 Ependymomas Histological differentiation
-15 Astrocytomas
Raco et al., [11] 2005 202 Cases 57.8% 38.2% Functional status at surgery
-86 Astrocytomas Histological type
-68 Ependymomas Extent of surgical removal
Woodworth et al., [14] 2007 78 Cases Not addressed 19.2% Serum glucose levels
-27 Ependymomas Preoperative ambulatory status
-23 Astrocytomas Preoperative radiation therapy
Matsuyama et al., [15] 2009 106 Cases 56.0% 31.5% Good preoperative neurological status
-46 Ependymomas Total resections
-12 Astrocytomas
-16 Hemangioblastomas
-17 Cavernous malformations
Klekamp, [19] 2013 250 Cases 61.2% 19.5% Surgical experience
-99 Ependymomas Preoperative status
-76 Astrocytomas Thoracic tumors
-28 Hemangioblastomas Tumor hemorrhage
-13 Cavernous malformations Recurrent tumors
Kumar and Banerjee, [9] 2014 43 Cases 69.8% 23.3% Preoperative neurological grade
-21 Ependymomas Gross tumor resections
-12 Astrocytomas High histological grades
-5 Hemangioblastomas
Boström et al., [16] 2014 70 Cases 64.3% 14.3% Degree of resection
-39 Ependymomas Preoperative status
-11 Astrocytomas
-5 Hemangioblastomas
-3 Cavernous Malformations
Hongo et al., [18] 2019 49 Cases 49.0% Not addressed Histological type
-32 Ependymomas Gross total resection for ependymoma
-17 Astrocytomas
Table 1. Summary of patient demographics among 1,033 cases of the intramedullary spinal cord tumors

Values are presented as mean (range) or number (%) unless otherwise indicated.

Table 2. Tumor characteristics of the 1,033 cases of the intramedullary spinal cord tumors

Values are presented as number (%) or mean (range) unless otherwise indicated.

Table 3. Histopathological types of the 1,033 intramedullary tumors in Japan, data arranged in decreasing order of frequency

PNET, primitive neuroectodermal tumor; ATRT, atypical teratoid/rhabdoid tumor.

Table 4. Surgical details and the results of the 1,033 intramedullary tumor cases

Values are presented as number (%) or mean±standard deviation.

Table 5. Postoperative course and the complications

CSF, cerebrospinal fluid; DVT, deep venous thrombosis.

Table 6. Multiple logistic regression modeling with clinical factors indicating mortality (n=978, 55 cases were lost of follow up)

OR, odds ratio; CI, confidence interval.

OR was calculated with Female as reference.

OR was calculated with modified McCormick Scales I and II as reference.

Table 7. Multiple logistic regression modeling with clinical factors indicating the better functional outcomes (n=896 with 6-month follow-up)

OR, odds ratio; CI, confidence interval.

OR was calculated with Female as reference.

OR was calculated with Modified McCormick scales III, IV, V as reference.

Table 8. Previous studies demonstrating surgical results for the intramedullary tumors