Unilateral malignant optic glioma following glioblastoma multiforme in the young: a case report and literature review
© The Author(s). 2017
Received: 5 December 2016
Accepted: 3 March 2017
Published: 11 March 2017
Malignant optic gliomas are rare, but they rapidly become lethal visual pathway tumors. We present the clinical course, treatment, and prognosis of a case of unilateral malignant optic glioma in a young man with a history of brain glioblastoma multiforme (GBM).
A 21-year-old man, who had GBM 7 years ago complained of a transient shadow in his vision and presented with normal visual acuity but optic disc edema and an enlarged blind spot in the right eye (oculus dexter, OD). Magnetic resonance imaging (MRI) showed a right intraorbital optic nerve tumor without a brain lesion. Chiasm involvement and severe vision deterioration occurred 3 months later. A biopsy of the right optic nerve revealed glioblastoma. Concurrent chemoradiotherapy (CCRT) prevented involvement of the fellow eye 1 year after symptom onset.
This report demonstrated that a regular ocular exam should be recommended for several years after GBM. In young healthy patients who are able to undergo chemotherapy and radiotherapy, visual function in the fellow eye can be preserved.
KeywordsOptic disc edema Malignant optic glioma Glioblastoma multiforme Young
Glioblastoma multiforme (GBM) is the most frequent and lethal type of malignant primary brain tumor in humans, and it has the poorest prognosis with a median survival of generally < 1 year from the time of diagnosis [1–3]. GBM is rarely observed in children, which comprising only 5–10% of childhood intracranial neoplasms .
Malignant optic glioma is rare and was first defined by Hoyt et al. in 1973 . It is a high grade astrocytoma, presenting as an anaplastic astrocytoma (AA) (WHO grade III) or glioblastoma (WHO grade IV) . Patients frequently have bilateral visual loss within a few weeks and die within 1–2 years [7, 8]. Here, we report a young man presenting with unilateral malignant optic glioma after having GBM for >5 years.
Relevant Past Medical History and Interventions
The 21-year old man was diagnosed with GBM at 13 years of age.
After tumor extirpation, he regularly underwent temozolomide chemotherapy and radiotherapy (66.6 Gy) from February 2007 to April 2007 and no brain GBM recurrence was shown at brain MRI during this round of treatment. However, progressing in cystic size was noted during regular clinics visit after previous treatment, radical resection of tumor was done in August 2007. Then he maintained with Temozolomide treatment for 8 more years (111 cycles).
Summaries from Initial and Follow-up Visits
Diagnostic Testing (including dates)
Chief complaint of an intermittent shadow in the vision when changing positions for several years.
Initial diagnosis: optic nerve tumor, likely secondary to GBM.
BCVA:20/20 in both eyes.
RAPD(+) and optic disc swelling (OD).
VF: enlarged blind spot (OD).
Orbital MRI: enhanced fusiform optic nerve tumor before the optic chiasma on a T1- weighted contrast image.
Aggressive chemotherapy (temozolomide 380 mg).
Clinical course deteriorated 3 months after symptom onset.
VF: severely constricted with only central area preservation(OD).
Orbital MRI: progressive changes in the right optic nerve lesion involving the chiasm on a T1- weighted image.
Clinical course severely deteriorated 4 months after symptom onset.
Histopathology proved malignant optic glioblastoma.
Histopathology from a right optic nerve biopsy: glioblastoma.
One month after the biopsy: CCRT with temozolomide (140 mg/day) and irradiation of the involved field (60 Gy, 30 times).
Afterwards, a new regimen of chemotherapy was administered (Bevacizumab).
Optic nerve tumor progressed 11 months after symptom onset.
Brain MRI: a right optic nerve tumor extending to the optic chiasm with progression in size.
Underwent right optic nerve tumor resection.
The ability to have CCRT can preserve visual function in the fellow eye.
BCVA and VF were within the normal range in the left eye and no recurrence or metastasis was noted in the latest follow-up brain MRI.
However, at 11 months after symptom onset, brain MRI showed a right optic nerve tumor extending to the optic chiasm with progression in size (Modified Dodge classification : 2b R/L)  (Fig. 2k, l). He underwent a right optic nerve tumor resection.
One year after symptom onset, BCVA and VF were within the normal range in the left eye (oculus sinister, OS) and no recurrence or metastasis was noted in the latest follow-up brain MRI.
Discussion and Conclusions
Currently, standard care to improve GBM patient survival begins with surgical removal followed by radiation and chemotherapy . Despite optimal treatment, most patients die of the disease, and the median overall survival time is 12–15 months [1, 12]. The main parameters associated with long-term survival are younger age (<40 years), good performance status, and the ability to undergo gross total resection, followed by radiotherapy and chemotherapy [2, 10, 13]. GBM can easily metastasize within the neuroaxis (i.e., the meninges or spinal cord) via the CSF occurring in approximately 20% of GBM patients . GBM metastases outside the central nervous system are rare , but may be found in the extraocular muscles, limbus, or orbit [16, 17]. Systemic chemotherapy is the only treatment that considerably prolongs overall survival times , which seem not to differ regardless of GBM developing extracranial metastasis.
The most common tumor of the optic nerve is optic nerve glioma, which represents approximately 66% of all primary optic nerve tumors. Approximately 90% of optic gliomas occur in children and most cases are relatively benign . Malignant optic gliomas are rare, and mostly present in middle aged men . From 1900 to 2015, only 22 cases of pathology proven optic nerve glioblastomas were recorded among 66 cases of primary malignant optic gliomas [7, 8]. At onset, 70% patients with malignant optic nerve gliomas present with symptoms of acute unilateral visual loss, spreading rapidly over the chiasm and leading to total blindness typically within 3 months .
Neuroradiologic findings are unspecific and typically described as contrast enhancement and thickening of the optic nerve, chiasm, or tract, with lesions having hypo- to iso-intensity on T1-weighted images . Biopsies continue to be necessary to confirm a diagnosis .
The current standard therapy consists of surgical resection or biopsy as feasible, followed by radiotherapy and/or temozolomide chemotherapy for high grade astrocytomas [7, 21]. The prognosis of a malignant optic glioma is fatal and most patients die within 1 year [7, 8].
Our case was challenging; diagnosis was difficult before biopsy because the optic nerve tumor appeared 7 years after brain GBM and visual function was initially normal. In addition, the complications of radiotherapy were a concern. The relationship between delayed radiotherapy and the progression and extension of the optic nerve tumor was unclear. Fortunately, BCVA and VF in the fellow eye remained unchanged despite the chiasm becoming involved 1 year after the symptom onset. Compared with previous reports, the progression of the optic malignant tumor was slower and the prognosis was better.
In summary, malignant optic tumors may appear >5 years after brain GBM. Regular ophthalmic examinations and brain MRIs are recommended for early diagnosis. Additional studies should evaluate the role and optimal timing of early adjuvant radiotherapy before visual function decreases. Young age, good health, and the ability to undergo CCRT can palliate the clinical course and preserve visual function in the fellow eye.
Best-corrected visual acuity
Magnetic resonance imaging
- oculus dexter:
OD, Right eye
- oculus sinister:
OS, Left eye
Relative afferent pupillary defect
Swedish Interactive Threshold Algorithm
The authors do not have any acknowledgements to state.
No financial support was received.
Availability of data and materials
Data sharing is not applicable to this article as no datasets were generated or analysed during the current study.
CYL was a major contributor in writing the manuscript. HMH examined and evaluated the patient, in addition to evaluating the manuscript. Both authors read and approved the final manuscript.
The authors declare that they have no competing interests.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Ethics approval and consent to participate
Ethical approval and consent were obtained (201600931B0D001) from our institute when the data were collected.
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