An Official publication of The Asian Congress of Neurological Surgeons (AsianCNS)

Search Article
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Advertise Subscribe Contacts Login  Facebook Tweeter
  Users Online: 93 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

   Table of Contents      
Year : 2021  |  Volume : 16  |  Issue : 3  |  Page : 626-629

Cranial and spinal oligodendrogliomatosis: A case report and review of the literature

1 Department of Neurosurgery, Ankara University School of Medicine, Ibn-i Sina Hospital, Ankara, Turkey
2 Department of Neurosurgery, Faculty of Medicine, Yuksek Ihtisas University, Ankara, Turkey
3 Department of Pathology, Ankara University School of Medicine, Ankara, Turkey

Date of Submission28-Nov-2020
Date of Decision22-Jan-2021
Date of Acceptance15-Apr-2021
Date of Web Publication14-Sep-2021

Correspondence Address:
Dr. Koral Erdogan
Ibn-i Sina Hastanesi, Hacettepe Mah., Talatpasa Blv. No: 82, Altindag, Ankara 06230
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajns.AJNS_514_20

Rights and Permissions

Secondary leptomeningeal gliomatosis is a condition known as a result of invasion of the subarachnoid space or the ventricular system of primary intraparenchymal glioma. In this article, we present a 7-year-old boy presented with neck and back deformity and deterioration of gait. Cranial and spinal magnetic resonance imaging revealed lesions in the supratentorial and infratentorial areas, in the brainstem downward the spinal cord. Disseminated oligodendrogliomatosis is extremely rare and our case we present is the 24th in the literature.

Keywords: 1p19q deletion, leptomeningeal dissemination, oligodendrogliomatosis

How to cite this article:
Erdogan K, Mammadkhanli O, Dogan I, Ersoz CC, Okcu AH, Unlu MA. Cranial and spinal oligodendrogliomatosis: A case report and review of the literature. Asian J Neurosurg 2021;16:626-9

How to cite this URL:
Erdogan K, Mammadkhanli O, Dogan I, Ersoz CC, Okcu AH, Unlu MA. Cranial and spinal oligodendrogliomatosis: A case report and review of the literature. Asian J Neurosurg [serial online] 2021 [cited 2021 Dec 2];16:626-9. Available from:

  Introduction Top

Primary oligodendrogliomatosis is a rare entity involving intracranial and spinal leptomeninges without parenchymal focus with a poor prognosis. Secondary oligodendrogliomatosis is the dissemination of a primary parenchymal oligodendroglioma in leptomeninges via subarachnoid space or ventricular system. Early diagnosis is important for early initiation of radiotherapy/chemotherapy that will improve the patient's quality of life and contribute to long-term surveillance. In this article, we report a 7-year-old boy presented with deformity in his back and neck and gait disturbance that magnetic resonance imaging (MRI) demonstrates multiple hyperintense small cystic lesions predominantly in the infratentorial region and an intramedullary expansile contrast-enhanced lesion extending from T5 to T7 level.

  Case Report Top

A 7-year-old boy was evaluated with a complaint of deformity in his back and neck which was noticed 2 months ago and worsening of walking. He was neurologically intact except spasticity in both lower extremities and hyperactivity in deep tendon reflexes. Routine laboratory values were normal. MRI revealed extra-axial multiple cystic lesions in both cerebellar hemispheres and vermis and cerebellar peduncles, in brainstem most prominently in the periaqueductal area extending to the interpeduncular cistern, and in the hippocampus and parahippocampal area, 4 mm diameter nodular lesion on the posterior wall of the fourth ventricle, communicating hydrocephalus. Cystic lesions in the posterior fossa were also observed extensively along the spinal cord at the cervical, thoracic, and lumbar levels [Figure 1]. The patient was radiologically hydrocephalic but had no symptoms due to increased intracranial pressure. In addition, in T2-weighted images heterogeneous and hyperintense and contrast enhanced in T1-weighted MRI images, an intramedullary expansile lesion was observed extending from lower T5 to lower T7 level [Figure 2]. Baclofen was administered orally 3 × 5 mg for spasticity in lower extremities with the recommendation of pediatric neurology clinic and started on prophylactic anticonvulsant therapy with levetiracetam. Informed consent was received from the family and the patient underwent T6–7 laminoplasty, biopsy from the dural lesion, and duraplasty. Intraoperative somatosensory-evoked potential changes with amplitude reduction did not allow us to go beyond biopsy and more tumor removal. There was no postoperative additional neurological deficit.
Figure 1: (a and c) T2 fluid-attenuated inversion recovery axial image shows multiple hyperintense cystic lesions in the cerebellum and cervical spine. (b) T1-weighted postcontrast image shows a nodular lesion on the posterior wall of fourth ventricle

Click here to view
Figure 2: (a) Leptomeningeal contrast enhancement at the conus. (b) Sagittal contrast-enhanced T1-weighted image shows intramedullary lesion at T5–7 level. (c) T2 fluid-attenuated inversion recovery axial image shows hydrocephalus

Click here to view

Pathological result of the patient was reported as a diffuse leptomeningeal glioneuronal tumor [Figure 3]. The tumor cells were characterized by round-to-oval nuclei with finely granular dispersed chromatin, inconspicuous nucleoli with clear oligodendrocyte-like features with perinuclear haloes. No necrosis or endothelial vascular proliferation was observed in the tumor. Focal myxoid change and 1 mitosis per 10 HPF were detected. The neoplastic cells were diffusely immunopositive for S100 and SYNP and immunonegative for GFAP. No ATX loss was detected and IDH1 and p53 were negative. Ki67 proliferation index was 5%–7%. Fluorescence in situ hybridization analysis revealed deletion of 1p, whereas 19q was intact. The patient was referred to the pediatric oncology clinic for chemotherapy and radiation therapy. However, the parents preferred to take these treatments in other institutes.
Figure 3: (a) An H and E section showed oligodendroglioma-like cytologic features with branching capillary networks. (b-d) The neoplastic cells were negative with GFAP (b); diffusely immunoreactive for synaptophysin (c); there was also strong and diffuse S100 immunopositivity (d). (e) Low proliferation activity detected by Ki67 immunostaining. (f and g) Fluorescence in situ hybridization analysis (f) 1p deletion of tumor cell nuclei; (g) 19q was intact

Click here to view

  Discussion Top

Primary diffuse leptomeningeal gliomatosis is pathologically available in two types: primary leptomeningeal astrocytoma and primary leptomeningeal oligodendroglioma. In the literature, primary oligodendrogliomatosis cases have been reported which Michotte et al. have compiled in their own study.[1] Secondary oligodendrogliomatosis is the dissemination of a primary glial tumor by subarachnoid space or ventricular system. In addition to leptomeningeal lesions, intraparenchymal lesions can be seen. In this article, we present a case with cranial and fourth ventricular lesions involved the spinal cord. There are very few reported studies of secondary oligodendrogliomatosis in the literature. There were a total of 24 cases with a current patient, of which 10 were adults and 14 were children [Table 1]. Sudden death was seen in at least three of these cases.[4],[13],[18] Only three patients have been reported that lived 5 years or more.[10],[11],[14] Only two patients had 1p 19q deletion, 8 years and more of surveillance was reported in these cases, 1p deletion was observed in our case, but 19q deletion was not seen.[1],[14]
Table 1: Review of the available literature

Click here to view

Oligodendrogliomatosis may be difficult to diagnose because it presents with progressive pial enhancement or various symptoms such as hydrocephalus and nonspecific imaging findings. Most patients with oligodendrogliomatosis present with neurological symptoms ranging from headache, nausea, and vomiting to sensory and motor disorders.[10],[13],[16],[17]

Since it is a rare disease, optimal treatment is controversial. To confirm the pathological diagnosis is the main purpose. Only partial resection was documented in all reported cases. In selected cases, neurosurgical intervention may be necessary for related findings such as hydrocephalus. Chemotherapy and craniospinal irradiation revealed good results. Bourne et al. reported stable disease after chemotherapy with cisplatin, vincristine, cyclophosphamide, and etoposide.[15] Franceschi et al. recently announced the treatment with temozolomide.[20] In general, the prognosis of this disease is poor. However, a higher quality of life has been reported following palliative treatment.

  Conclusion Top

We reviewed the literature for oligodendrogliomatosis for treatment and management options in pediatric age that is a very rare disease that patients may present differently. We present a case who underwent surgery for a biopsy. After pathological diagnosis, chemotherapy and radiotherapy were started for the case that proven to prolong survival.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Michotte A, Chaskis C, Sadones J, Veld PI, Neyns B. Primary leptomeningeal anaplastic oligodendroglioma with a 1p36-19q13 deletion: Report of a unique case successfully treated with Temozolomide. J Neurol Sci 2009;287:267-70.  Back to cited text no. 1
Beck DJ, Russell DS. Oligodendrogliomatosis of the cerebrospinal pathway. Brain 1942;65:352-72.  Back to cited text no. 2
Koreın J, Feıgın I, Shapıro MF. Oligodendrogliomatosis with intracranial hypertension. Neurology 1957;7:589-94.  Back to cited text no. 3
Best PV. Intracranial oligodendrogliomatosis. J Neurol Neurosurg Psychiatry 1963;26:249-56.  Back to cited text no. 4
Pitt MA, Jones AW, Reeve RS, Cowie RA. Oligodendroglioma of the fourth ventricle with intracranial and spinal oligodendrogliomatosis: A case report. Br J Neurosurg 1992;6:371-4.  Back to cited text no. 5
Rogers LR, Purvis JB, Lederman RJ, Rosenbloom SA, Tomsak RL, Estes ML, et al. Alternating sequential intracarotid BCNU and cisplatin in recurrent malignant glioma. Cancer 1991;68:15-21.  Back to cited text no. 6
Chen R, Macdonald DR, Ramsay DA. Primary diffuse leptomeningeal oligodendroglioma. Case report. J Neurosurg 1995;83:724-8.  Back to cited text no. 7
Ng HK, Poon WS. Diffuse leptomeningeal gliomatosis with oligodendroglioma. Pathology 1999;31:59-63.  Back to cited text no. 8
Pradat PF, Hoang-Xuan K, Cornu P, Mokhtari K, Martin-Duverneuil N, Poisson M, et al. Treatment of meningeal gliomatosis. J Neurooncol 1999;44:163-8.  Back to cited text no. 9
Armao DM, Stone J, Castillo M, Mitchell KM, Bouldin TW, Suzuki K. Diffuse leptomeningeal oligodendrogliomatosis: Radiologic/pathologic correlation. AJNR Am J Neuroradiol 2000;21:1122-6.  Back to cited text no. 10
Gilmer-Hill HS, Ellis WG, Imbesi SG, Boggan JE. Spinal oligodendroglioma with gliomatosis in a child. Case report. J Neurosurg 2000;92:109-13.  Back to cited text no. 11
Stödberg T, Deniz Y, Esteitie N, Jacobsson B, Mousavi-Jazi M, Dahl H, et al. A case of diffuse leptomeningeal oligodendrogliomatosis associated with HHV-6 variant A. Neuropediatrics 2002;33:266-70.  Back to cited text no. 12
Ozkul A, Meteoglu I, Tataroglu C, Akyol A. Primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death. J Neurooncol 2007;81:75-9.  Back to cited text no. 13
Guppy KH, Akins PT, Moes GS, Prados MD. Spinal cord oligodendroglioma with 1p and 19q deletions presenting with cerebral oligodendrogliomatosis. J Neurosurg Spine 2009;10:557-63.  Back to cited text no. 14
Bourne TD, Mandell JW, Matsumoto JA, Jane JA Jr., Lopes MB. Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report. J Neurosurg 2006;105:465-9.  Back to cited text no. 15
Chellathurai A, Vaidya JS, Kathirvelu G, Alagappan P. Primary diffuse leptomeningeal oligodendrogliomatosis: A case report and literature review. Indian J Radiol Imaging 2016;26:337-41.  Back to cited text no. 16
[PUBMED]  [Full text]  
Mathews MS, Paré LS, Kuo JV, Kim RC. Primary leptomeningeal oligodendrogliomatosis. J Neurooncol 2009;94:275-8.  Back to cited text no. 17
Reynolds RM, Boswell E, Hulette CM, Cummings TJ, Haglund MM, Boswell E, et al. Sudden death from diffuse leptomeningeal oligodendrogliomatosis. J Neurosurg Spine 2011;15:625-9.  Back to cited text no. 18
Lee SL, Wong A, Stadler JA 3rd, McClendon J Jr, Smith TR, Wadhwani NR, et al. Cranial and spinal oligodendrogliomatosis: A case report and review of the literature. Childs Nerv Syst 2015;31:147-53.  Back to cited text no. 19
Franceschi E, Cavallo G, Scopece L, Esposti RD, Paioli G, Paioli A, et al. Temozolomide-induced partial response in a patient with primary diffuse leptomeningeal gliomatosis. J Neurooncol 2005;73:261-4.  Back to cited text no. 20


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)  

  In this article
  Case Report
   Article Figures
   Article Tables

 Article Access Statistics
    PDF Downloaded65    
    Comments [Add]    

Recommend this journal