Abstract
Primary tumors of the optic nerve include anterior visual pathway gliomas and optic nerve sheath meningiomas. Both are relatively rare lesions that result in significant visual morbidity. Together they account for less than 4 % of all orbital tumors. There has been controversy about the natural history and appropriate management of these lesions, resulting from small sample sizes and short follow-up periods. Other tumors, such as gangliogliomas and primary lymphomas, have also been described but are extremely rare.
9.1 Introduction
Primary tumors of the optic nerve include anterior visual pathway gliomas and optic nerve sheath meningiomas. Both are relatively rare lesions that result in significant visual morbidity. Together they account for less than 4 % of all orbital tumors. There has been controversy about the natural history and appropriate management of these lesions, resulting from small sample sizes and short follow-up periods. Other tumors, such as gangliogliomas and primary lymphomas, have also been described but are extremely rare (Table 9.1).
9.2 Anterior Visual Pathway Glioma
Optic pathway gliomas (OPG) are uncommon benign lesions classified as pilocytic astrocytomas. They represent 1.5–4 % of all orbital tumors and 50–55 % of all primary optic nerve tumors [1, 2].
9.2.1 Clinical Features
9.2.1.1 Age Distribution
Gliomas have been described in patients from birth to 79 years of age. However, 71 % of cases occur in children in the first decade of life and 90 % within the first two decades. The overall mean age at presentation is 8.8 years for all optic gliomas.
9.2.1.2 Sex Distribution
The sexual distribution for all optic pathway gliomas shows approximately equal numbers of males and females. For gliomas confined to the optic nerve, 65 % occur in females, compared to 35 % for males. For tumors involving the optic chiasm, there is no sex predilection.
9.2.1.3 Location
About 20–25 % of optic gliomas are confined to the optic nerve alone, but in three-quarters of cases the chiasm is involved [3, 4]. Of the tumors that involve the chiasm, 40 % eventually extend into the adjacent hypothalamus or third ventricle. Several studies have shown that the orbital optic nerve is involved more frequently in patients with NF1 (21.5 %) than in those without NF1 (5.5 %) [5].
9.2.1.4 Association with Neurofibromatosis Type 1
Optic pathway gliomas may be sporadical or syndromic, the latter mostly associated with neurofibromatosis type 1. The reported incidence of neurofibromatosis type 1 (NF1) among patients with optic gliomas varies from 10 to 70 %, with an overall incidence of 29 % [1]. Although some reports have shown no difference in the course and prognosis of optic pathway gliomas with and without NF1, others have suggested a more indolent course and a better prognosis in patients with OPG and NF1 [6–8].
When associated with NF1 the glioma may present at a somewhat later age and show progression for a long time, justifying regular ophthalmological monitoring of this population over a long period [9, 10].
9.2.1.5 Signs and Symptoms
The specific findings of optic pathway gliomas depend principally on the location of the tumor. Regardless of location, 85 % of patients lose some vision, with about 25 % retaining good vision between 20/20 and 20/40. About 60 % of patients show vision of 20/300 or worse.
Proptosis is a presenting sign in 95 % of patients with an optic nerve glioma (Fig. 9.1). With gliomas of the optic chiasm, proptosis is much less common, seen in fewer than 20 % of patients, and all with concomitant intraorbital involvement. Limitation of ocular motility is seen infrequently with optic gliomas. It is reported in 30 % of intraorbital lesions and 20 % of gliomas involving the chiasm. Pain and headache are present in up to 30 % of patients with chiasmal tumors. Other rare symptoms seen with CNS invasion include nystagmus, spasmus nutans, seizures, hypothalamic signs, and hydrocephalus. On funduscopic examination 60 % of patients demonstrate some degree of optic atrophy. Disc edema, primarily associated with intraorbital gliomas, is seen in half of such cases. Disc edema occurs in only 20 % of patients with chiasmal tumors, and in these cases the tumor is usually contiguous with the intraorbital optic nerve (Box 9.1).
Box 9.1: Optic Pathway Glioma
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Early visual loss 88 %
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Optic disc swelling 35 %
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Optic disc atrophy 59 %
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Proptosis, orbital tumors 94 %; chiasmal tumors 22 %
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Nystagmus 24 %
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Hypothalamic signs 26 %
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Increased intracranial pressure 27 %
9.2.2 Diagnostic Evaluation
Imaging studies reveal enlargement of the optic canal in up to 80 % of patients with a glioma involving the optic nerves. Enlargement and J-shaped excavation of the sella turcica may be associated with chiasmal gliomas but are reported in only 25 % of patients.
9.2.2.1 Computed Tomography
Computed tomography (CT) imaging typically demonstrates enlargement of the optic nerve and/or chiasm. Contrast enhancement ranges from imperceptible to moderate but generally is less than with sheath meningiomas. Typical optic gliomas show a well-outlined fusiform swelling of the optic nerve (Fig. 9.2), but occasionally they may be more rounded or even tubular. Calcification occurs only occasionally.
9.2.2.2 Magnetic Resonance Imaging
Magnetic resonance imaging (MRI) has proved superior to CT for evaluation of chiasmal, hypothalamic, and optic tract lesions. Gliomas demonstrate normal to slightly prolonged T1 relaxation times, which image isointense to slightly hypointense compared to normal optic nerve. The T2 relaxation time is prolonged, giving a hyperintense image on T2-weighted sequences.
9.2.3 Histopathology
Although optic gliomas were formerly considered congenital nonneoplastic hamartomas with self-limiting growth, their histologic features, rates of growth, rare malignant potential, and a clear tendency to invade the leptomeninges show that these tumors are true neoplasms that have the ability to invade locally.
Optic gliomas arise from supporting astrocytes of the optic nerve and along the visual pathway. Most are classified as benign pilocytic astrocytomas in which proliferating neoplastic astrocytic cells predominate. Proliferating astrocytes may extend through the pia mater into the arachnoid and subarachnoid space, where they provoke an exuberant reactive proliferation of fibrovascular tissue and meningothelial cells. This so-called arachnoidal hyperplasia may extend beyond the limits of the tumor itself, simulating tumor extension. Enlargement of optic gliomas may occur as a result of proliferation of neoplastic cells, reactive arachnoidal proliferation, or an accumulation of extracellular, PAS-positive mucosubstance secreted by the astrocytes.
9.2.4 Treatment Options
Anterior visual pathway gliomas are neoplasms with the potential to spread into contiguous areas of the optic nerve, chiasm, and adjacent brain. They appear at an early age, grow slowly for a few years, and vision generally stabilizes in most cases. However, indolent growth can be seen in up to 40 % of cases. Although the best treatment options are still evolving, an algorithm for the management of patients with optic pathway gliomas is suggested (Fig. 9.3). As with most medical decisions, treatment must be individualized based on patient symptoms, findings, and clinical course [11].
9.2.4.1 Observation
Long-term survival shows a good prognosis even in patients followed conservatively without intervention [12–15]. After an initial period of deterioration, vision tends to stabilize in nearly 80 % of cases. There is little difference in visual outcome or survival in patients undergoing treatment versus observation alone [1]. However, significant progression of tumor can occur in some patients despite clinically stable visual acuity for many years.
9.2.4.2 Surgery
Until recently, surgery was considered the treatment of choice for optic nerve gliomas. Today most authorities limit the indications for surgery to resectable tumors involving the orbital or intracranial optic nerves or for direct inspection or biopsy of the chiasm. Once vision is lost, surgery can be beneficial for severe proptosis or orbital pain. Where vision is present, surgical intervention carries a very significant risk of visual morbidity and mortality.
9.2.4.3 Radiotherapy
The role of radiotherapy has been a subject of debate for decades. Some studies have failed to show any benefit of radiotherapy on long-term survival, visual acuity, or both [1]. However, other studies showed improvement or stability in visual acuity after treatment and better progression-free survival interval [16, 17]. Overall, the data suggest a possible benefit. Any potential benefit must also be tempered by the adverse effects of radiotherapy on the central nervous system in younger children. Several cases of malignant transformation have been reported following radiotherapy.
9.2.4.4 Chemotherapy
Several reports have suggested a role for chemotherapy in the management of optic pathway gliomas. Most studies show stabilization of vision and/or tumor regression in 50–65 % of treated patients [18–21]. Although this is not much better than for tumors observed conservatively, chemotherapy may be useful for young children with progressive lesions in order to delay the potential complications of radiotherapy.
9.2.5 Prognosis
A review of the literature shows that for all patients with optic pathway gliomas in all locations and with all forms of treatment, including observation, tumor recurrence or progression occurs in 38 % of cases [1]. The overall tumor-related mortality is 36 % with a mean follow-up of 11 years. However, with longer follow-up intervals of 25–30 years, the prognosis for survival may be considerably worse. The outlook for vision is actually rather good. About 55 % of patients retain stable vision or show some improvement. Only 45 % show progressive loss of vision. One case has been reported of spontaneous malignant transformation [22]. Spontaneous regression has been reported [23].
9.2.5.1 Glioma Confined to the Optic Nerve
For gliomas initially confined to the optic nerve and treated conservatively or incompletely excised, recurrence or progression is seen in 17 %. The mortality rate is 12 %, typically from intracranial extension. The prognosis for vision, however, is good, with over 90 % remaining stable over many years. With optic nerve tumors treated by complete surgical excision or partial excision plus radiotherapy, the mortality rate drops to zero. The same is true for tumors that progress but remain confined to the optic nerve. Obviously, the prognosis for vision in such cases is poor after surgery.
9.2.5.2 Gliomas with Extension to the Chiasm
Gliomas that extend to the chiasm but that do not invade the adjacent hypothalamus or third ventricle show results similar to those for untreated optic nerve tumors. Chiasmal gliomas left untreated or that are partially excised show a mortality rate of 17 % over a mean follow-up period of 10 years. As with optic nerve tumors, death results from extension into the hypothalamus or third ventricle. Recurrence or progression of tumor after partial excision is seen in 64 % of cases. Visual prognosis in this group is good, with 80 % remaining stable. In patients with chiasmal tumors radiotherapy may delay progression to some extent. Mortality in this group is 22 %, and recurrence or progression of tumor is seen in 43 % of cases. Prognosis for vision is similar to that of untreated patients, with 68 % remaining stable or demonstrating a slight improvement.
9.2.5.3 Gliomas with Extension to the Chiasm and Hypothalamus
In patients with chiasmal tumors plus hypothalamic or third ventricle involvement at the time of presentation, the prognosis for life is markedly reduced. The mortality rate is 50 % or more over 15 years. For patients who received radiotherapy, recurrence or progression is noted in 52 %, but the mortality rate is somewhat better, at 43 %.
9.3 Malignant Optic Nerve Glioma
In 1973 Hoyt et al. [24] described five cases of optic pathway glioma in adults which had an aggressive behavior and a uniformly fatal outcome. To date less than 50 additional cases have been reported in the literature, all confirming the original concept of the disease.
9.3.1 Clinical Features
9.3.1.1 Age Distribution
Malignant optic pathway gliomas have been seen in patients from 6 to 79 years old but occur most commonly in middle age. The mean age at presentation is 48 years.
9.3.1.2 Sex Distribution
This disease has a distinct sexual predilection, with 65 % occurring in males and 35 % in females.
9.3.1.3 Location
In all described cases, the optic chiasm is the major site of origin. In all cases except one [25]. the disease is bilateral with both optic nerves also involved. In only 23 % of cases does the tumor extend to the intraorbital portion of the nerve. In nearly half of patients, the tumor extends posterior to the optic tracts, hypothalamus, or temporal lobe [1].
9.3.1.4 Signs and Symptoms
All patients present with rapidly progressive loss of vision, first in one and then in the second eye. In 63 % of cases, both eyes may be affected at presentation. Initially the condition may be misdiagnosed as optic neuritis. Bilateral blindness typically results within a matter of months. Optic disc edema is seen in most patients, and, if they survive long enough, optic atrophy results. Orbital signs are uncommon, as most tumors are confined to the intracranial compartment. Proptosis and ophthalmoplegia are seen in only 20–25 % of cases (Box 9.2).
Box 9.2: Malignant Optic Glioma
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Very rapid loss of vision to blindness over weeks to months
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Optic disc swelling 43 %
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Optic disc atrophy 31 %
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Proptosis 23 %
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Ophthalmoplegia 19 %
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Other neurologic signs 35 %
9.3.2 Diagnostic Evaluation
Enlargement and enhancement of the optic chiasm on CT or MRI is the most common finding, seen in 80 % of cases. One or both optic nerves may also be involved along their intracranial portions. The final diagnosis is usually made only after tissue biopsy.
9.3.3 Histopathology
Histopathology shows malignant astrocytes with pleomorphic nuclei and areas of vascular proliferation and necrosis. Subpial extension along the optic pathways is seen. There is invasion into the optic chiasm and nerve and into adjacent areas of the brain.
9.3.4 Treatment
To date no treatment has proved effective in slowing the progression of this disease. Neither surgery nor radiotherapy up to 60 Gy has significantly altered the prognosis [26].
9.3.5 Prognosis
Prognosis for vision is dismal, with all patients progressing to profound visual loss within months of initial presentation. The prognosis for life is equally dismal, with a nearly 100 % mortality rate. The mean survival rate is typically less than 1 year.
9.4 Optic Nerve Sheath Meningioma
Meningiomas are the second most common brain neoplasms after gliomas. They represent 15–20 % of all intracranial tumors in adults and 2 % of intracranial tumors in children [27]. Although most orbital meningiomas are extensions from intracranial sites, primary orbital meningiomas have been documented and account for 1.3 % of all meningiomas.
9.4.1 Clinical Features
9.4.1.1 Age Distribution
Despite several reports of orbital meningiomas occurring with high frequency in young individuals, most series confirm that this is a disease primarily of middle age. On imaging studies, meningiomas may be confused with arachnoid proliferation associated with optic gliomas in young patients. The mean age for presentation of optic sheath meningioma is 41 years, and only 4 % of patients are under 20 [27, 28].
9.4.1.2 Sex Distribution
It has long been recognized that meningiomas occur more frequently in females. When large series are examined the ratio has tended to equalize, but there does appear to be a slight female preponderance of approximately 60 %.
9.4.1.3 Laterality
A slight predilection for the right optic nerve has been reported in several studies. Others have not confirmed these findings. However, when larger series are examined 52 % of sheath meningiomas occurred in the right optic nerve, 42 % in the left, and 6 % were bilateral [27]. Interestingly, among bilateral cases 60 % are canalicular meningiomas, compared to all sheath meningiomas together, where canalicular tumors account for only 8 %.
9.4.1.4 Sites of Origin
For optic sheath meningiomas 94 % are unilateral and 6 % bilateral. In about 8 % of cases the meningioma is confined to the optic canal. Among these canalicular tumors there is a significant propensity toward bilaterality, 65 % being unilateral and 35 % bilateral. About 4 % of optic sheath meningiomas show focal tumor invasion of the optic disc, sclera, choroid, and retina. Tumor may enter the globe along penetrating vascular channels. Dutton [27] noted that 18 of 475 cases of primary orbital meningioma arose ectopic to the optic nerve sheath. The exact etiology of such lesions remains uncertain, and it is possible that in some cases they represent other lesions mistaken for meningiomas.
9.4.1.5 Association with Neurofibromatosis Type 1
The incidence of NF1 in patients with sheath meningiomas is unclear because most studies in the past failed to mention the occurrence. Of the studies that specifically examined for NF, 9 % of patients were affected. This is considerably lower than the 29 % association with optic gliomas but still significantly higher than the 0.3–0.5 % incidence of NF in the general population.
9.4.1.6 Signs and Symptoms
The most frequent presenting symptom of optic sheath meningioma is loss of vision, seen in 97 % of cases. In about 45 % visual acuity is 20/20–20/40, and in only 25 % is it counting fingers or worse. Visual loss usually takes place over several years. In bilateral cases, visual loss in the two eyes may be separated in time by 2–30 years. Visual field defects are noted in 83 % of patients. Most commonly these include peripheral constriction; central, centrocecal, and paracentral scotomas; altitudinal defects; and increased size of the blind spot. Proptosis is found on initial examination in 65 % of patients (Fig. 9.4, Box 9.3). It is seen less frequently in patients with canalicular lesions, as they typically have significant visual loss while the tumor is still very small. Limitation of ocular motility is variable but may be seen in more than half of patients. Upgaze is commonly severely impaired, possibly because of stiffening of the optic nerve from the relatively firm tumor.
Chronic disc edema is an early finding in 48 % of patients. Optic atrophy, which may be subtle, is a somewhat later finding, noted in two-thirds of cases at presentation. Both edema and atrophy may be seen together, and overall 98 % of patients will show one or the other of these two findings. The association between optic sheath meningiomas and optociliary shunt vessels has long been considered a key finding suggestive of optic sheath meningiomas. However, chronic disc edema and congestion of the central retinal vein usually precede the first appearance of shunts by several years, and the shunts usually disappear as optic atrophy becomes complete. In fact, optociliary shunt vessels are relatively infrequent with sheath meningiomas, being seen in only 30 % of reported cases. Because shunts tend to appear some years after symptoms begin and involute as optic atrophy is complete, this probably does not indicate their true incidence.
Box 9.3: Optic Nerve Sheath Meningioma
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Slowly progressive visual loss 96 %
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Optic disc swelling 48 %
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Optic disc atrophy 66 %
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Proptosis 65 %
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Decreased ocular motility 47 %
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Optociliary shunt vessels 30 %
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Increased intracranial pressure 27 %
9.4.2 Diagnostic Evaluation
9.4.2.1 Computed Tomography
Plain orbital radiographs and tomography through the optic canals demonstrate enlargement of the optic foramen in less than 30 % of cases. CT scanning demonstrates enlargement of the optic nerve in 97 % of examinations. The most common pattern is diffuse tubular enlargement, but a globular or fusiform shape may be seen also. Tram-tracking, a radiographic sign in which the denser and thickened optic nerve sheath outlines a central lucency representing the residual optic nerve, is a characteristic of sheath meningioma (Fig. 9.5). Contrast studies generally show moderate to marked enhancement. Calcification, an important finding, may help differentiate meningiomas from optic gliomas. It is seen in 20–50 % of patients.
9.4.2.2 Magnetic Resonance Imaging
Magnetic resonance imaging shows a thickening of the nerve and sheath contrasted against orbital fat, and there is increased signal intensity compared to normal nerve on both the T1- and T2-weighted sequences.
9.4.3 Histopathology
Optic meningiomas arise from meningothelial cap cells of the arachnoid villi that lie along the intraorbital optic nerve. Two histologic patterns are seen. In the meningothelial or syncytial pattern, polygonal cells are arranged in sheets separated by vascular trabeculae. In the transitional pattern, spindle-shaped or oval cells are arranged in a concentric whorl formation. Psammoma bodies are seen more commonly in the transitional pattern and contain the calcifications noted on radiologic studies. Meningioma typically remains indolent over many years. As the tumor grows within the subarachnoid space, it commonly encircles the optic nerve. Compression results in obstruction to axoplasmic flow, disc edema, dilatation of optociliary shunt channels, and eventually demyelinization and optic atrophy. Tumor cells may also invade through the dura and into surrounding orbital tissues. Although they do invade along the intracranial optic nerve to the chiasm, meningiomas do not invade the brain.
9.4.4 Treatment
The most appropriate therapy for optic sheath meningiomas has been a matter of some controversy. For sheath meningiomas that extend to the intracanalicular or intracranial portions of the optic nerve, the decision regarding treatment becomes less complex. The major rationale for treatment is the perceived risk of spread to the contralateral optic nerve. The actual risk of tumor spread from one optic nerve to the other remains unknown, but based on the high incidence of bilaterality with canalicular tumors and on documented unilateral tumors with progressive posterior extension, it may be very real. Because vision in such cases will eventually be lost, treatment to prevent possible extension to the contralateral side is justified. In most cases radiotherapy may slow or halt tumor progression. However, in cases of treatment failure, surgical excision should be considered. Newer treatment options have gained considerable support over the past decade, and these are changing the approach to management. A proposed treatment algorithm is shown in Fig. 9.6.
9.4.4.1 Observation
For meningiomas confined to the intraorbital optic nerve, when vision remains and symptoms and radiographic findings are stable, observation without treatment is appropriate [28]. The only possible exceptions that justify surgery are small anterior tumors and cases in young children, where biopsy for diagnosis may be indicated. Progressive visual loss is expected in most cases, but some patients may remain stable for many years. The prognosis for life is excellent, and there have been no tumor-related deaths reported for this disease.
9.4.4.2 Surgery
Some have considered surgical excision necessary to prevent intracranial extension. This may be possible for some small anterior orbital lesions [29] or even with some posterior tumors [30], but in general the morbidity is high and loss of vision is a very common sequela due to disruption of vascular supply to the optic nerve. Once blindness results, surgical extirpation may be necessary for relief of disfiguring proptosis, orbital pain, or intraocular complications. Incomplete excision has been associated with diffuse orbital invasion and intracranial spread to the chiasm. The risk of such spread outweighs the potential benefit of attempted resection. Attempts to decompress the optic nerve by opening the dural sheath have proved disappointing and have resulted in massive orbital invasion requiring exenteration following surgical decompression. For lesions in the optic canal, canal decompression with dural sheath release may preserve sight in some cases [31].
9.4.4.3 Radiotherapy
For lesions that show progression by worsening symptoms or radiographic findings, radiotherapy would be an appropriate option. In the past radiotherapy was considered ineffective; however, more recent reports using newer techniques suggest that in appropriate doses radiotherapy can be effective [32]. Fractionated stereotactic radiotherapy may offer a promising refinement with fewer complications [33]. The optimum total dose appears to be in the range of 50–55 Gy. Stability or improvement of vision has been reported in 50–95 % of cases. Similar results are being reported with the use of three-dimensional conformal fractionated radiation [34]. Complications of radiotherapy are reported in up to 15 % of cases and include new visual field defects, central retinal artery occlusion, and encephalopathy. More recently, staged fractionated robotic CyberKnife radiosurgery with 20 Gy has given local control with visual improvement in 80 % of patients [35].
9.4.5 Prognosis
Patients with optic sheath meningiomas have an excellent prognosis for life. There are few, if any, documented cases of tumor-related death. The prognosis for vision, however, is poor. Without treatment, in most patients visual loss progresses slowly but inexorably to blindness in the affected eye. However, in some cases a spontaneous improvement in vision or visual field has been reported. Surgery offers little additional benefit and in most cases accelerates the process of visual loss. Rarely, a small anteriorly situated tumor may be excised with preservation or improvement of vision. Radiotherapy may stabilize or improve visual symptoms in some cases.
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Dutton, J.J. (2014). Optic Nerve Tumors. In: Perry, J., Singh, A. (eds) Clinical Ophthalmic Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-40492-4_9
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