Primary spinal cord oligodendroglioma: a case report and review of the literature
© Tunthanathip and Oearsakul. 2015
Received: 16 September 2015
Accepted: 26 October 2015
Published: 18 January 2016
Primary spinal cord oligodendroglioma is extremely rare. In an extensive review of this disease, 53 cases were reported. Furthermore, the authors summarize the characteristics of the primary spinal cord oligodendroglioma; chronological presentation , neurological imaging, treatment and the outcome obtained in the present case as well as review the literature.
A 46-year-old male who had progressive neck pain for a year. Magnetic resonance imaging showed an intramedullary mass from level C2 to T4. A radical resection was performed. Histology revealed oligodendroglioma. Thereafter, the patient was treated with adjuvant radiotherapy. A year later, tumor developed recurrence. The patinet died in 3 years and 6 months.
The available data of this disease was limited. Base on 11 published papers and the present case, surgical resection is the treatment of choice although recurrence of the tumor tends to occur after partial resection with or without radiotherapy. From the literature, the management of the recurrent disease is still surgery. Moreover, Temozolomide may be an advantage in recurrent situations.
KeywordsOligodendroglioma Spinal cord tumor Intramedullary tumor Treatment
Oligodendroglioma originates from oligodendrocyte, which is found in either the brain or spinal cord . This tumor is commonly found in the cerebral hemisphere while both primary and drop metastasis spinal oligodendroglioma are an unusual presentation of a disease [2, 3]. The lack of clinical information means there is no standard treatment in current practice. The authors report a case and review modality of treatment and prognosis from the literature.
A 46-year-old male was presented with progressive neck pain for a year. The symptoms began without any antecedent event. The symptoms were worse for 5 months. One month before admission, the patient developed left arm weakness and numbness in both arms. However, he had no urinary incontinence. Physical examination demonstrated grade 4/5 muscle weakness in the left arm. Other extremities revealed grade 5/5 of motor strength. Pinpricked sensation was suspended deficits at C5-T1 levels. Biceps, triceps and brachioradialis, knee and Achilles reflexes were 2+. Further, Hoffman reflexes were absent.
Oligodendrocytes are the myelination cells of the brain and spinal cord. In the spinal cord, most oligodendrocyte derived from oligodendrocyte precursor cells at the ventral ventricular zone, which migrate through the spinal cord and differentiate into oligodendrocytes . Oligodendrogliomas are one of the primary brain tumors usually occurring in the cerebral hemispheres while these tumors of the spinal cord are rare. To the authors’ knowledge, only 53 cases have been reported in literature [4–19].
Review of the literature in the treatment and outcome of primary spinal cord oligodendroglioma
Fortuna et al. 
Guidetti et al. 
3 years, death
Pagni et al. 
Kyphoscoliosis, painful sensation involved at neck and shoulder and tetraparesis
IM at C5-T2
2 years, improvement
Wang et al. 
PR and RT
Nam et al. 
Scoliosis, and monoparesis of right lower limb
IM with syringomyelia along T4-conus medullaris
PR and RT
50 months, no progression of tumor
Ushida et al. 
Scoliosis, hypesthesia of legs, Hyperreflexia of legs
IM with syringomyelia at C7-T12
-10 months, recurrence of tumor
-2nd PR and RT
Gilmer-Hill et al. 
Irritability, Hypotonia, ataxic gait
Primary intramedullary oligodendroglioma at C6-T1 with gliomatosis
-1st cerebellar, occipital Bx (Spondgliform cerebellar degeneration)
-2nd temporal Bx (Oligodendrogliomatosis)
-3rd spinal Bx (Oligodendroglioma) and CMT
-10 months after CMT, recurrence at temporal lobe
-4th PR and RT
-4 months after RT, recurrence at occipital lobe
-5th PR and CMT (AO)
-7 months after surgery, stable disease
Fountas et al. 
Neurogenic bladder, left ankle weakness and sensory deficit of S1 nerve root on the left.
IDEM with extension at T11-L2
28 months, no recurrence of tumor
Gürkanlar et al. 
Bilateral leg pain
IDEM at L1-L2 with parital invasion of conus
Ramirez et al. 
Paresthesia, LBP and left Brown-Sequard syndrome
IM with hematomyelia at C5-C7
PR and RT
2 years, brain metastasis
Wang et al. 
Low back pain, lower limb weakness, hypalgesia below level T5
IM with syringomyelia at T8-T10
1st PR and RT
-8 months, tumor recurrence
-2nd PR and TMZ
-1 year after TMZ, no recurrence of tumor
Neck pain, left arm weakness, suspended sensory deficit at C5-T1 levels
IM with syringomyelia at C3-T4
PR and RT
-1 year, tumor recurrence
In literature, Ushida et al. reviewed the characteristics of patients with this tumor. The mean age was 28.4 years, no significant difference in sex . Spinal pain that included back pain, buttock pain and sciatica was the most common first symptom for 69.3 % whereas the most common symptom on physical examination was a sensorimotor deficit . According to the location of spinal cord, these tumors have been found frequently in the thoracic (30 %), cervical (25 %) and lumbar (5 %) area respectively . Furthermore, this disease frequently presented with an intramedullary lesion in the spinal canal. Intradural extramedullary was an unusual presentation [16, 17]. Common spinal features on MRI’s were isointense in T1-weighted images and hyperintense in T2-weighted images and heterogenous enhancement in Gadolinium-enhanced sagittal T1-weighted images [7, 12, 16–19]. Uncommon findings might be found as an adjacent cystic component [11–13], microhemorrhage [4, 16, 19]. Diagnosis of this disease are difficult to distinguish from other gliomas by MRI because these findings can find in spinal astrocytoma and ependymoma . However, microcalcification is important finding, which mentioned oligodendroglioma similar to intracranial oligodendroglioma [11, 16]. Regarding to histology, Most of the tumors are oligodendroglioma WHO grade 2. Wang et al. reviewed 8 cases, which were anaplastic oligodendroglioma in the literature and added a case .
From Table 1, surgical resection is the treatment of choice. Unfortunately, gross total resection is so difficult because almost all tumors are the intramedullary type. In the literature, gross total resection was achieved in only 16.6 % (2/12) of the cases. Moreover, tumor recurrence was observed almost 42 % (5/12) within 8 months to 2 years. Second operation was mentioned in recurrent situation with postoperative adjuvant therapy. The benefit of radiation therapy is still controversial. Nam et al. reported outcome of anaplastic oligodendroglioma, which was treated with partial tumor resection and postoperative radiation. The patient has no progression of residual tumor after 50 months follow up . However, Ramirez et al. and Wang et al. were reported the patients whom are treated with partial tumor resection and postoperative radiation too [18, 19]. Unfortunately, both patients have progressive disease.
The adjuvant chemotherapy has been mentioned to treat recurrent scenario [13, 19]. Temozolomide (TMZ) that is a novel alkylating and methylating agent has been reported the benefits in oligodendroglioma . Adjuvant TMZ treatment has recently reported in relapse conditions. The patient who was operated in the second time received postoperative TMZ to treat the residual tumor. Complete resolution of the residual tumor within six months of TMZ chemotherapy was observed on MRI of the spine .
Primary spinal cord oligodendrogliomas are extremely rare tumors. In spite of limited data of treatment, surgical resection is the treatment of choice although recurrence of the tumor tends to occur after partial resection with or without radiotherapy. From the literature, the management of the recurrent disease is still surgery. Moreover, TMZ may be an advantage in recurrent situations.
Written, informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Magnetic resonance imaging
The immunohistochemical figures referred to in this study are the work of Dr. Kanet Kanjanapadit.
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
- Bradl M, Lassmann H. Oligodendrocytes: biology and pathology. Acta Neuropathol. 2010;119:37–53.View ArticlePubMedPubMed CentralGoogle Scholar
- Ng HK, Sun DT, Poon WS. Anaplastic oligodendroglioma with drop metastasis to the spinal cord. Clin Neurol Neurosurg. 2002;104:383–6.View ArticlePubMedGoogle Scholar
- Carlsen JG, Tietze A, Lassen YA, Rosendal F. Paraplegia due to drop metastases from anaplastic oligodendroglioma. Br J Neurosurg. 2012;26:d94–5.View ArticleGoogle Scholar
- Fortuna A, Celli P, Palma L. Oligodendrogliomas of the spinal cord. Acta Neurochir. 1980;52:305–29.View ArticlePubMedGoogle Scholar
- Guidetti B, Mercuri S, Vagnozzi R. Long-term results of the surgical treatment of 129 intramedullary spinal gliomas. J Neurosurg. 1981;54:323–30.View ArticlePubMedGoogle Scholar
- Alvis C, Cerisoli M, Giulioni M. Intramedullary spinal gliomas: long-term results of surgical treatments. Acta Neurochir (Wien). 1984;70:169–79.View ArticleGoogle Scholar
- Pagni CA, Canavero S, Gaidolfi E. Intramedullary “holocord” oligodendroglioma: case report. Acta Neurochir. 1991;113:96–9.View ArticlePubMedGoogle Scholar
- Wang KC, Chi JG, Cho BK. Oligodendroglioma in childhood. J Korean Med Sci. 1993;8:110–6.View ArticlePubMedPubMed CentralGoogle Scholar
- Cristante L, Herrmann HD. Surgical management of intramedullary spinal cord tumor: functional outcome and source of morbidity. Neurosurgery. 1994;35:69–76.View ArticlePubMedGoogle Scholar
- Constantini S, Houten J, Miller DC, Freed DF, Ozek MM, Rorke LB. Intramedullary spinal cord tumors in children under the age of 3 years. J Neurosurg. 1996;85:1036–43.View ArticlePubMedGoogle Scholar
- Nam DH, Cho BK, Kim YM, Chi JG, Wang KC. Intramedullary anaplastic oligodendroglioma in a child. Childs Nerv Syst. 1998;14:127–30.View ArticlePubMedGoogle Scholar
- Ushida T, Sonobe H, Mizobuchi H, Toda M, Tani T, Yamamoto H. Oligodendroglioma of the “widespread” type in the spinal cord. Childs Nerv Syst. 1998;14:751–5.View ArticlePubMedGoogle Scholar
- Gilmer-Hill HS, Ellis WG, Imbesi SG, Boggan JE. Spinal oligodendroglioma with gliomatosis in a child: case report. J Neurosurg. 2000;92:109–13.PubMedGoogle Scholar
- Miller DC. Surgical pathology of intramedullary spinal cord neoplasms. J Neurooncol. 2000;47:189–94.View ArticlePubMedGoogle Scholar
- Aman RA, Padmosantjojo MH, Atmadji LB, Soemitro D. Intramedullary oligodendroglioma: a case report. Gan To Kagaku Ryoho. 2000;27:571–3.PubMedGoogle Scholar
- Fountas KN, Karampelas I, Nikolakakos LG, Troup EC, Robinson JS. Primary spinal cord oligodendroglioma: case report and review of the literature. Childs Nerv Syst. 2005;21:171–5.View ArticlePubMedGoogle Scholar
- Gürkanlar D, Koçak H, Aciduman A, Yucel E, Ekinci O. Primary spinal cord oligodendroglioma. Case illustration. Neurocirugia. 2006;17:542–3.View ArticlePubMedGoogle Scholar
- Ramirez C, Delrieu O, Mineo JF, Paradot G, Allaoui M, Dubois F, et al. Intracranial dissemination of primary spinal cord anaplastic oligodendroglioma. Eur J Neurol. 2007;14:578–80.View ArticlePubMedGoogle Scholar
- Wang F, Qiao G, Lou X. Spinal cord anaplastic oligodendroglioma with 1p deletion: report of a relapsing case treated with temozolomide. J Neurooncol. 2011;104:387–94.View ArticlePubMedGoogle Scholar
- Koeller KK, Rosenblum RS, Morrison AL. Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. Radiographics. 2000;20:1721–49.View ArticlePubMedGoogle Scholar
- Gan HK, Rosenthal MA, Dowling A, Kalnins R, Algar E, Wong N, et al. A phase II trial of primary temozolomide in patients with grade III oligodendroglial brain tumors. Neuro Oncol. 2010;12:500–7.View ArticlePubMedPubMed CentralGoogle Scholar