- Email: [email protected]
Waiting to deliver a final diagnosis
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/survophthal
Afterimage
Waiting to deliver a final diagnosis Ali Mokhtarzadeh, MD*, Amanda Maltry, MD, Collin McClelland, MD Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA
article info
abstract
Article history:
We report a patient with left eye positional transient visual obscurations, diplopia, a left
Received 12 August 2016
afferent pupillary defect, and optic disk edemadall occurring during pregnancy. Non-
Received in revised form 17 January
contrast magnetic resonance imaging revealed an orbital mass encasing the optic nerve
2017
and extending through the superior orbital fissure into the cavernous sinus. An magnetic
Accepted 17 January 2017
resonance imaging with gadolinium after parturition and strongly positive somatostatin
Available online xxx
receptor scintigraphy suggested the diagnosis of meningioma. Biopsy confirmed the
Andrew Harrison and Michael Lee,
diagnosis, and she was treated with CyberKnife stereotactic radiosurgery. In cases of
Editors
atypical presentation, somatostatin receptor scintigraphy can help distinguish optic nerve sheath meningioma from alternative orbital masses.
Keywords:
ª 2017 Elsevier Inc. All rights reserved.
optic nerve sheath meningioma somatostatin receptor scintigraphy orbital tumor orbit meningioma
1.
Case presentation
A 33-year-old woman in week 26 of an uncomplicated pregnancy presented with diplopia in up-gaze and intermittent, 30second episodes of painless “graying out” of vision in the left eye exacerbated by position changes. Acuity was 20/20 OD and 20/25 OS with intact color vision in both eyes and a left relative afferent pupillary defect. There was a moderate elevation deficit of the left eye. The remainder of the cranial nerve examination and anterior slit lamp examination was normal. External examination showed 4 mm of relative left proptosis. Dilated fundus examination was normal in the right eye and showed severe optic disk edema in the left eye. Humphrey Vision Analyzer 24-2 visual fields were full in the right eye and showed an inferior altitudinal defect in the left eye.
Magnetic resonance imaging (MRI) brain without gadolinium revealed a 20 17 14 mm intraconal left orbital mass that was T1- and T2-isointense, encased the optic nerve, and extended through the superior orbital fissure into the left cavernous sinus (Fig. 1). Despite a trial of oral prednisone 60 mg daily, her left eye’s visual field worsened. The differential diagnosis at this point included steroidrefractory inflammation, infiltrative malignancy including lymphoma, and optic nerve sheath meningioma. The patient and care team opted for repeat imaging following Caesarean section at 37-week gestation. Postpartum MRI brain and orbits with gadolinium revealed no change in size of the homogeneously enhancing mass (Fig. 2). Orbital CT failed to reveal bone destruction, bone remodeling, or calcification.
* Corresponding author: Ali Mokhtarzadeh, MD, Department of Ophthalmology and Visual Neurosciences, University of Minnesota, MMC 493, Harvard Street, Minneapolis, MN 55455, USA. E-mail address: [email protected] (A. Mokhtarzadeh). 0039-6257/$ e see front matter ª 2017 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.survophthal.2017.01.006
2
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
Histopathology showed nests of bland meningothelial cells infiltrating skeletal muscle indicative of a WHO grade-1 meningioma (Fig. 5A). These cells were positive on immunohistochemical staining for progesterone receptors (Fig. 5B) and negative for estrogen receptors. The mass was treated with CyberKnife stereotactic radiosurgery, administering 2500 cGy over 5 equal fractions. Four months after treatment, her visual acuity improved to 20/20, the visual field defect improved significantly, and her motility remained stable.
Fig. 1 e Axial T2 fat-saturated magnetic resonance imaging (MRI) without gadolinium revealed a left intraconal mass (arrow) encasing the optic nerve (*) and extending through the supraorbital fissure (arrow head ) toward the left cavernous sinus.
Somatostatin receptor scintigraphy revealed focal radiotracer uptake of the mass indicating somatostatin receptor positivity and strongly suggesting the diagnosis of meningioma (Fig. 3). It is unusual for optic nerve sheath meningioma to track through the superior orbital fissure into the cavernous sinus, thus biopsy was recommended. Navigation-guided endoscopic sinus approach to the orbital apex allowed an incisional biopsy of the gelatinous mass lateral to the medial rectus muscle (Fig. 4).
Fig. 2 e Coronal T1 fat-saturated postcontrast magnetic resonance imaging (MRI) through the midorbits demonstrating homogeneous enhancement of the mass (arrow head ) encasing the left optic nerve (arrow).
2.
Discussion
Optic nerve sheath meningiomas (ONSMs) constitute 1e2% of all meningiomas, yet are the second most common intrinsic optic nerve neoplasm.6 Like all meningiomas, ONSMs have a female predominance (F:M ¼ 3:2).6 Most ONSMs present among the middle-aged, although they may rarely present in childhood (4e7%).1,6,19 ONSM may be classified as primary, arising from the intraorbital or intracanalicular optic nerve, or secondary, arising from an intracranial location with extension to the optic nerve sheath.17 Among ONSMs exhibiting both an intracranial and intraorbital segment such as our case, it may be challenging to discern the tumor’s origin. Fortunately, this distinction of primary versus secondary ONSM carries little clinical significance in regards to management. CT of the orbits can aid the diagnosis by showing thickening of the optic nerve/sheath complex, calcifications within the tumor, and hyperostotic bone remodeling. Intravenous contrast may help distinguish a hyperdense sheath and hypodense optic nerve termed the “tram-track sign.” Readily available higher resolution MRI has greatly facilitated early detection of ONSMs. The “tram-track sign” is accentuated on T1-weighted postgadolinium sequences with avid enhancement of the thickened optic nerve sheath as compared to the optic nerve. The presence of tumor extension within the cavernous sinus, sellar region, and/or adjacent skull base as in our case is unusual in ONSM, but has been reported among 4/ 30 patients in 1 large series.3 The extent of intracranial involvement is best assessed with MRI. In ONSMs with intracranial extension, the presence of an enhancing, thickened dural tail strongly indicates the diagnosis of meningioma with a specificity of 94% in 1 study.18 ONSM is now often diagnosed earlier than in the pre-MRI era.13 In unusual cases such as ours, intracranial tumor and rapid symptom progression may lead to diagnostic challenges. Rapidly growing intracranial meningiomas indicate a more aggressive tumor grade, seen in 1.5e30% of tumors (WHO grade II or III) and are more common in men.5 Among ONSMs specifically, an aggressive growth pattern is more common in children.7 Rapid vision loss or acute realization of chronic vision loss in atypical ONSM presentations may lead to an initial misdiagnosis of optic neuritis.2,21 Sex-hormone receptor expression is common in intracranial meningiomas. In a study of 510 meningiomas, progesterone receptor, estrogen receptor, and androgen receptor positivity occurred in 88%, 40%, and 39% of tumors, respectively.11 Clinically, meningiomas may exhibit
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
3
Fig. 3 e Somatostatin receptor scintigraphy with intense focal radiotracer uptake in the left orbit.
increased growth during pregnancy.16,22 Tumor growth may result from physiologically increased circulating progesterone.22 Similarly, spontaneous radiographic shrinkage of a sphenoid wing meningioma following childbirth has been reported.10 Our patient’s rapidly progressive visual field loss may have been pregnancy related. Radiologically consistent tumor appearance, rapid symptom progression, and tumor growth during pregnancy would normally lead to the presumptive diagnosis of meningioma; however, our patient’s MRI showed intracranial extension that clouded the diagnosis. Meningiomas are known to express somatostatin receptors, along with lymphocytic inflammation and other neoplasms including neuroendocrine tumors and nonHodgkin lymphoma. Using radio-labeled octreotide to bind to somatostatin receptors, somatostatin receptor scintigraphy combined with single photon emission CT has been reported in 1 study of 61 patients as both a sensitive (100%) and specific (97.2%) means to distinguish ONSM from alternative orbital
Fig. 4 e Intraoperative view of the gelatinous tumor from an endoscopic transnasal approach.
masses including vascular tumors, inflammation, and nonHodgkin lymphoma.20 Our patient demonstrated high octreotide uptake characteristic of ONSM. Given the atypical extension through the superior orbital fissure, the diagnosis was confirmed by biopsy. Incisional biopsy of the optic nerve is rarely necessary to diagnose ONSM and is generally avoided. Neoplastic cells constituting the ONSM invade the subdural and subarachnoid space deep to the sheath and adhere to the adjacent optic nerve, forming a layer through which the extradural vascular supply of the optic nerve must travel. Incisional biopsy or attempted excision therefore caries a high risk of violating the optic nerve blood supply, causing catastrophic vision loss. As in this case, concern for an alternative, aggressive neoplasm may warrant the risks of biopsy.
3.
Conclusion
Minimally symptomatic ONSMs may be observed with serial ophthalmic examinations and neuroimaging.17 As modern radiation therapy techniques evolve, studies have shown excellent short-term and long-term outcomes of ONSM to fractionated radiotherapy.3,8,9,12 Andrews and colleagues found their rates of vision improvement (42%) and stability (50%) after fractionated stereotactic radiotherapy for ONSM (n ¼ 33 eyes) to be markedly higher than the rate of improvement (3%) and stability (27%) in their treatment control group (n ¼ 31 eyes) consisting of surgery or observation only.3 Considering the cumulative data supporting its safety and efficacy, the current treatment of choice for most visually significant ONSM is fractionated radiation.4 Evidence supports similar efficacy and safety between various types of fractionated radiation techniques for ONSM using fractions of 1.8e2.0 Gy (e.g., fractionated stereotactic radiotherapy, intensitymodulated radiotherapy, and conventional conformal radiotherapy).4 Single fraction, frame-based stereotactic surgery (e.g., Gamma Knife) for ONSM carries a higher risk to the optic nerve and demonstrates worse visual outcomes compared to published outcomes with fractionated radiation.4,14 As used in
4
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
Fig. 5 e A: H&E stained (203) orbital mass biopsy demonstrating nests of bland meningothelial cells (arrows) infiltrating extraocular skeletal muscle consistent with a WHO grade-I meningioma. B: Immunohistochemical staining for progesterone receptors (203) is strongly positive.
our patient, CyberKnife stereotactic radiation with larger radiation fractions (5 Gy) appears to be effective and safe without late toxicity.15
4.
Disclosures
The authors have no financial disclosures.
references
1. Alper MG. Management of primary optic nerve meningiomas. Current statusetherapy in controversy. J Clin Neuroophthalmol. 1981;1(2):101e17 2. Alroughani R, Behbehani R. Optic nerve sheath meningioma masquerading as optic neuritis. Case Rep Neurol Med. 2016;2016:5419432 3. Andrews DW, Faroozan R, Yang BP, et al. Fractionated stereotactic radiotherapy for the treatment of optic nerve sheath meningiomas: preliminary observations of 33 optic nerves in 30 patients with historical comparison to observation with or without prior surgery. Neurosurgery. 2002;51(4):890e902, discussion 903e894. 4. Bloch O, Sun M, Kaur G, Barani IJ, Parsa AT. Fractionated radiotherapy for optic nerve sheath meningiomas. J Clin Neurosci. 2012;19(9):1210e5 5. Buttrick S, Shah AH, Komotar RJ, Ivan ME. Management of atypical and anaplastic meningiomas. Neurosurg Clin N Am. 2016;27(2):239e47 6. Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37(3):167e83 7. Harold Lee HB, Garrity JA, Cameron JD, Strianese D, Bonavolonta G, Patrinely JR. Primary optic nerve sheath meningioma in children. Surv Ophthalmol. 2008;53(6):543e58 8. Ito M, Ishizawa A, Miyaoka M, Sato K, Ishii S. Intraorbital meningiomas. Surgical management and role of radiation therapy. Surg Neurol. 1988;29(6):448e53 9. Kennerdell JS, Maroon JC, Malton M, Warren FA. The management of optic nerve sheath meningiomas. Am J Ophthalmol. 1988;106(4):450e7
10. Kerschbaumer J, Freyschlag CF, Stockhammer G, et al. Hormone-dependent shrinkage of a sphenoid wing meningioma after pregnancy: case report. J Neurosurg. 2016;124(1):137e40 11. Korhonen K, Salminen T, Raitanen J, Auvinen A, Isola J, Haapasalo H. Female predominance in meningiomas can not be explained by differences in progesterone, estrogen, or androgen receptor expression. J Neurooncol. 2006;80(1):1e7 12. Kupersmith MJ, Warren FA, Newall J, Ransohoff J. Irradiation of meningiomas of the intracranial anterior visual pathway. Ann Neurol. 1987;21(2):131e7 13. Lesser RL, Knisely JP, Wang SL, Yu JB, Kupersmith MJ. Longterm response to fractionated radiotherapy of presumed optic nerve sheath meningioma. Br J Ophthalmol. 2010;94(5):559e63 14. Liu D, Xu D, Zhang Z, et al. Long-term results of Gamma Knife surgery for optic nerve sheath meningioma. J Neurosurg. 2010;113(Suppl):28e33 15. Marchetti M, Bianchi S, Milanesi I, et al. Multisession radiosurgery for optic nerve sheath meningiomasean effective option: preliminary results of a single-center experience. Neurosurgery. 2011;69(5):1116e22, discussion 1122e1113. 16. Marosi C, Hassler M, Roessler K, et al. Meningioma. Crit Rev Oncol Hematol. 2008;67(2):153e71 17. Miller NR. New concepts in the diagnosis and management of optic nerve sheath meningioma. J Neuroophthalmol. 2006;26(3):200e8 18. Rokni-Yazdi H, Sotoudeh H. Prevalence of “dural tail sign” in patients with different intracranial pathologies. Eur J Radiol. 2006;60(1):42e5 19. Saeed P, Rootman J, Nugent RA, White VA, Mackenzie IR, Koornneef L. Optic nerve sheath meningiomas. Ophthalmology. 2003;110(10):2019e30 20. Saeed P, Tanck MW, Freling N, Baldeschi L, Mourits MP, Bennink RJ. Somatostatin receptor scintigraphy for optic nerve sheath meningiomas. Ophthalmology. 2009;116(8):1581e6 21. Sawaya RA, Sidani C, Farah N, Hourani-Risk R. Presumed bilateral optic nerve sheath meningiomas presenting as optic neuritis. J Neuroophthalmol. 2008;28(1):55e7 22. Wahab M, Al-Azzawi F. Meningioma and hormonal influences. Climacteric. 2003;6(4):285e92
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/survophthal
Afterimage
Waiting to deliver a final diagnosis Ali Mokhtarzadeh, MD*, Amanda Maltry, MD, Collin McClelland, MD Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA
article info
abstract
Article history:
We report a patient with left eye positional transient visual obscurations, diplopia, a left
Received 12 August 2016
afferent pupillary defect, and optic disk edemadall occurring during pregnancy. Non-
Received in revised form 17 January
contrast magnetic resonance imaging revealed an orbital mass encasing the optic nerve
2017
and extending through the superior orbital fissure into the cavernous sinus. An magnetic
Accepted 17 January 2017
resonance imaging with gadolinium after parturition and strongly positive somatostatin
Available online xxx
receptor scintigraphy suggested the diagnosis of meningioma. Biopsy confirmed the
Andrew Harrison and Michael Lee,
diagnosis, and she was treated with CyberKnife stereotactic radiosurgery. In cases of
Editors
atypical presentation, somatostatin receptor scintigraphy can help distinguish optic nerve sheath meningioma from alternative orbital masses.
Keywords:
ª 2017 Elsevier Inc. All rights reserved.
optic nerve sheath meningioma somatostatin receptor scintigraphy orbital tumor orbit meningioma
1.
Case presentation
A 33-year-old woman in week 26 of an uncomplicated pregnancy presented with diplopia in up-gaze and intermittent, 30second episodes of painless “graying out” of vision in the left eye exacerbated by position changes. Acuity was 20/20 OD and 20/25 OS with intact color vision in both eyes and a left relative afferent pupillary defect. There was a moderate elevation deficit of the left eye. The remainder of the cranial nerve examination and anterior slit lamp examination was normal. External examination showed 4 mm of relative left proptosis. Dilated fundus examination was normal in the right eye and showed severe optic disk edema in the left eye. Humphrey Vision Analyzer 24-2 visual fields were full in the right eye and showed an inferior altitudinal defect in the left eye.
Magnetic resonance imaging (MRI) brain without gadolinium revealed a 20 17 14 mm intraconal left orbital mass that was T1- and T2-isointense, encased the optic nerve, and extended through the superior orbital fissure into the left cavernous sinus (Fig. 1). Despite a trial of oral prednisone 60 mg daily, her left eye’s visual field worsened. The differential diagnosis at this point included steroidrefractory inflammation, infiltrative malignancy including lymphoma, and optic nerve sheath meningioma. The patient and care team opted for repeat imaging following Caesarean section at 37-week gestation. Postpartum MRI brain and orbits with gadolinium revealed no change in size of the homogeneously enhancing mass (Fig. 2). Orbital CT failed to reveal bone destruction, bone remodeling, or calcification.
* Corresponding author: Ali Mokhtarzadeh, MD, Department of Ophthalmology and Visual Neurosciences, University of Minnesota, MMC 493, Harvard Street, Minneapolis, MN 55455, USA. E-mail address: [email protected] (A. Mokhtarzadeh). 0039-6257/$ e see front matter ª 2017 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.survophthal.2017.01.006
2
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
Histopathology showed nests of bland meningothelial cells infiltrating skeletal muscle indicative of a WHO grade-1 meningioma (Fig. 5A). These cells were positive on immunohistochemical staining for progesterone receptors (Fig. 5B) and negative for estrogen receptors. The mass was treated with CyberKnife stereotactic radiosurgery, administering 2500 cGy over 5 equal fractions. Four months after treatment, her visual acuity improved to 20/20, the visual field defect improved significantly, and her motility remained stable.
Fig. 1 e Axial T2 fat-saturated magnetic resonance imaging (MRI) without gadolinium revealed a left intraconal mass (arrow) encasing the optic nerve (*) and extending through the supraorbital fissure (arrow head ) toward the left cavernous sinus.
Somatostatin receptor scintigraphy revealed focal radiotracer uptake of the mass indicating somatostatin receptor positivity and strongly suggesting the diagnosis of meningioma (Fig. 3). It is unusual for optic nerve sheath meningioma to track through the superior orbital fissure into the cavernous sinus, thus biopsy was recommended. Navigation-guided endoscopic sinus approach to the orbital apex allowed an incisional biopsy of the gelatinous mass lateral to the medial rectus muscle (Fig. 4).
Fig. 2 e Coronal T1 fat-saturated postcontrast magnetic resonance imaging (MRI) through the midorbits demonstrating homogeneous enhancement of the mass (arrow head ) encasing the left optic nerve (arrow).
2.
Discussion
Optic nerve sheath meningiomas (ONSMs) constitute 1e2% of all meningiomas, yet are the second most common intrinsic optic nerve neoplasm.6 Like all meningiomas, ONSMs have a female predominance (F:M ¼ 3:2).6 Most ONSMs present among the middle-aged, although they may rarely present in childhood (4e7%).1,6,19 ONSM may be classified as primary, arising from the intraorbital or intracanalicular optic nerve, or secondary, arising from an intracranial location with extension to the optic nerve sheath.17 Among ONSMs exhibiting both an intracranial and intraorbital segment such as our case, it may be challenging to discern the tumor’s origin. Fortunately, this distinction of primary versus secondary ONSM carries little clinical significance in regards to management. CT of the orbits can aid the diagnosis by showing thickening of the optic nerve/sheath complex, calcifications within the tumor, and hyperostotic bone remodeling. Intravenous contrast may help distinguish a hyperdense sheath and hypodense optic nerve termed the “tram-track sign.” Readily available higher resolution MRI has greatly facilitated early detection of ONSMs. The “tram-track sign” is accentuated on T1-weighted postgadolinium sequences with avid enhancement of the thickened optic nerve sheath as compared to the optic nerve. The presence of tumor extension within the cavernous sinus, sellar region, and/or adjacent skull base as in our case is unusual in ONSM, but has been reported among 4/ 30 patients in 1 large series.3 The extent of intracranial involvement is best assessed with MRI. In ONSMs with intracranial extension, the presence of an enhancing, thickened dural tail strongly indicates the diagnosis of meningioma with a specificity of 94% in 1 study.18 ONSM is now often diagnosed earlier than in the pre-MRI era.13 In unusual cases such as ours, intracranial tumor and rapid symptom progression may lead to diagnostic challenges. Rapidly growing intracranial meningiomas indicate a more aggressive tumor grade, seen in 1.5e30% of tumors (WHO grade II or III) and are more common in men.5 Among ONSMs specifically, an aggressive growth pattern is more common in children.7 Rapid vision loss or acute realization of chronic vision loss in atypical ONSM presentations may lead to an initial misdiagnosis of optic neuritis.2,21 Sex-hormone receptor expression is common in intracranial meningiomas. In a study of 510 meningiomas, progesterone receptor, estrogen receptor, and androgen receptor positivity occurred in 88%, 40%, and 39% of tumors, respectively.11 Clinically, meningiomas may exhibit
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
3
Fig. 3 e Somatostatin receptor scintigraphy with intense focal radiotracer uptake in the left orbit.
increased growth during pregnancy.16,22 Tumor growth may result from physiologically increased circulating progesterone.22 Similarly, spontaneous radiographic shrinkage of a sphenoid wing meningioma following childbirth has been reported.10 Our patient’s rapidly progressive visual field loss may have been pregnancy related. Radiologically consistent tumor appearance, rapid symptom progression, and tumor growth during pregnancy would normally lead to the presumptive diagnosis of meningioma; however, our patient’s MRI showed intracranial extension that clouded the diagnosis. Meningiomas are known to express somatostatin receptors, along with lymphocytic inflammation and other neoplasms including neuroendocrine tumors and nonHodgkin lymphoma. Using radio-labeled octreotide to bind to somatostatin receptors, somatostatin receptor scintigraphy combined with single photon emission CT has been reported in 1 study of 61 patients as both a sensitive (100%) and specific (97.2%) means to distinguish ONSM from alternative orbital
Fig. 4 e Intraoperative view of the gelatinous tumor from an endoscopic transnasal approach.
masses including vascular tumors, inflammation, and nonHodgkin lymphoma.20 Our patient demonstrated high octreotide uptake characteristic of ONSM. Given the atypical extension through the superior orbital fissure, the diagnosis was confirmed by biopsy. Incisional biopsy of the optic nerve is rarely necessary to diagnose ONSM and is generally avoided. Neoplastic cells constituting the ONSM invade the subdural and subarachnoid space deep to the sheath and adhere to the adjacent optic nerve, forming a layer through which the extradural vascular supply of the optic nerve must travel. Incisional biopsy or attempted excision therefore caries a high risk of violating the optic nerve blood supply, causing catastrophic vision loss. As in this case, concern for an alternative, aggressive neoplasm may warrant the risks of biopsy.
3.
Conclusion
Minimally symptomatic ONSMs may be observed with serial ophthalmic examinations and neuroimaging.17 As modern radiation therapy techniques evolve, studies have shown excellent short-term and long-term outcomes of ONSM to fractionated radiotherapy.3,8,9,12 Andrews and colleagues found their rates of vision improvement (42%) and stability (50%) after fractionated stereotactic radiotherapy for ONSM (n ¼ 33 eyes) to be markedly higher than the rate of improvement (3%) and stability (27%) in their treatment control group (n ¼ 31 eyes) consisting of surgery or observation only.3 Considering the cumulative data supporting its safety and efficacy, the current treatment of choice for most visually significant ONSM is fractionated radiation.4 Evidence supports similar efficacy and safety between various types of fractionated radiation techniques for ONSM using fractions of 1.8e2.0 Gy (e.g., fractionated stereotactic radiotherapy, intensitymodulated radiotherapy, and conventional conformal radiotherapy).4 Single fraction, frame-based stereotactic surgery (e.g., Gamma Knife) for ONSM carries a higher risk to the optic nerve and demonstrates worse visual outcomes compared to published outcomes with fractionated radiation.4,14 As used in
4
s u r v e y o f o p h t h a l m o l o g y x x x ( 2 0 1 7 ) 1 e4
Fig. 5 e A: H&E stained (203) orbital mass biopsy demonstrating nests of bland meningothelial cells (arrows) infiltrating extraocular skeletal muscle consistent with a WHO grade-I meningioma. B: Immunohistochemical staining for progesterone receptors (203) is strongly positive.
our patient, CyberKnife stereotactic radiation with larger radiation fractions (5 Gy) appears to be effective and safe without late toxicity.15
4.
Disclosures
The authors have no financial disclosures.
references
1. Alper MG. Management of primary optic nerve meningiomas. Current statusetherapy in controversy. J Clin Neuroophthalmol. 1981;1(2):101e17 2. Alroughani R, Behbehani R. Optic nerve sheath meningioma masquerading as optic neuritis. Case Rep Neurol Med. 2016;2016:5419432 3. Andrews DW, Faroozan R, Yang BP, et al. Fractionated stereotactic radiotherapy for the treatment of optic nerve sheath meningiomas: preliminary observations of 33 optic nerves in 30 patients with historical comparison to observation with or without prior surgery. Neurosurgery. 2002;51(4):890e902, discussion 903e894. 4. Bloch O, Sun M, Kaur G, Barani IJ, Parsa AT. Fractionated radiotherapy for optic nerve sheath meningiomas. J Clin Neurosci. 2012;19(9):1210e5 5. Buttrick S, Shah AH, Komotar RJ, Ivan ME. Management of atypical and anaplastic meningiomas. Neurosurg Clin N Am. 2016;27(2):239e47 6. Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37(3):167e83 7. Harold Lee HB, Garrity JA, Cameron JD, Strianese D, Bonavolonta G, Patrinely JR. Primary optic nerve sheath meningioma in children. Surv Ophthalmol. 2008;53(6):543e58 8. Ito M, Ishizawa A, Miyaoka M, Sato K, Ishii S. Intraorbital meningiomas. Surgical management and role of radiation therapy. Surg Neurol. 1988;29(6):448e53 9. Kennerdell JS, Maroon JC, Malton M, Warren FA. The management of optic nerve sheath meningiomas. Am J Ophthalmol. 1988;106(4):450e7
10. Kerschbaumer J, Freyschlag CF, Stockhammer G, et al. Hormone-dependent shrinkage of a sphenoid wing meningioma after pregnancy: case report. J Neurosurg. 2016;124(1):137e40 11. Korhonen K, Salminen T, Raitanen J, Auvinen A, Isola J, Haapasalo H. Female predominance in meningiomas can not be explained by differences in progesterone, estrogen, or androgen receptor expression. J Neurooncol. 2006;80(1):1e7 12. Kupersmith MJ, Warren FA, Newall J, Ransohoff J. Irradiation of meningiomas of the intracranial anterior visual pathway. Ann Neurol. 1987;21(2):131e7 13. Lesser RL, Knisely JP, Wang SL, Yu JB, Kupersmith MJ. Longterm response to fractionated radiotherapy of presumed optic nerve sheath meningioma. Br J Ophthalmol. 2010;94(5):559e63 14. Liu D, Xu D, Zhang Z, et al. Long-term results of Gamma Knife surgery for optic nerve sheath meningioma. J Neurosurg. 2010;113(Suppl):28e33 15. Marchetti M, Bianchi S, Milanesi I, et al. Multisession radiosurgery for optic nerve sheath meningiomasean effective option: preliminary results of a single-center experience. Neurosurgery. 2011;69(5):1116e22, discussion 1122e1113. 16. Marosi C, Hassler M, Roessler K, et al. Meningioma. Crit Rev Oncol Hematol. 2008;67(2):153e71 17. Miller NR. New concepts in the diagnosis and management of optic nerve sheath meningioma. J Neuroophthalmol. 2006;26(3):200e8 18. Rokni-Yazdi H, Sotoudeh H. Prevalence of “dural tail sign” in patients with different intracranial pathologies. Eur J Radiol. 2006;60(1):42e5 19. Saeed P, Rootman J, Nugent RA, White VA, Mackenzie IR, Koornneef L. Optic nerve sheath meningiomas. Ophthalmology. 2003;110(10):2019e30 20. Saeed P, Tanck MW, Freling N, Baldeschi L, Mourits MP, Bennink RJ. Somatostatin receptor scintigraphy for optic nerve sheath meningiomas. Ophthalmology. 2009;116(8):1581e6 21. Sawaya RA, Sidani C, Farah N, Hourani-Risk R. Presumed bilateral optic nerve sheath meningiomas presenting as optic neuritis. J Neuroophthalmol. 2008;28(1):55e7 22. Wahab M, Al-Azzawi F. Meningioma and hormonal influences. Climacteric. 2003;6(4):285e92