Neurofibromatosis Type 2: Optic Nerve Sheath Meningioma in one Orbit, Intramuscular Schwannoma in the Other

Kıratlı, Hayyam; Yıldız, Sibel; Söylemezoğlu, Figen

doi:10.1080/01676830802350356

Cited by 17 publications

(4 citation statements)

References 9 publications

(19 reference statements)

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“…Half of the schwannoma specimens displayed previously described classic features [2, 14], including a capsule, hyalinized vessels, and coexisting Antoni A and Antoni B patterns. Most schwannomas of the orbit appear to be sporadic, but some have occurred in association with NF2 [27]. We did not see that association in any of the cases in this study.…”

Section: Discussioncontrasting

confidence: 58%

Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa

et al. 2017

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Peripheral nerve sheath tumors (PNSTs) are known to occur in the orbit and comprise 4% of all orbital tumors, but have not been well-studied in contemporary literature. Ninety specimens involving the eye and ocular adnexa (1979–2015) from 67 patients were studied. The mean age was 32.5 years. Locations included orbit (58.9%), eyelid (60.0%) and other ocular adnexa. A large majority of specimens were neurofibromas (70.0%), followed by schwannomas (11.1%), neuromas (11.1%), granular cell tumors (n=4), nerve sheath myxomas (n=2), and malignant peripheral nerve sheath tumor (n=1). Fifty-six (88.9%) neurofibroma cases were neurofibromatosis 1 (NF1)-associated. Among neurofibromas, 31.7% were localized, 38.1% were plexiform, 25.4% were diffuse, and 4.8% were diffuse and plexiform. These tumors involved skin (31.7%), soft tissue (11.1%), skeletal muscle (22.2%), peripheral nerve (63.0%), lacrimal gland (20.6%) and choroid (n=1). Other histologic findings included pseudo-Meissner corpuscles (27%), Schwann cell nodules (4.8%), prominent myxoid component (7.9%), melanin-like pigment (3.2%), and inflammation (14.3%). Available immunostains included S100 (+ in 15/15 cases), EMA (+ in 2/4 cases), CD34 (+ in 4/4 cases), and Ki67 (<1% in 4/4 cases). Among 10 schwannomas, 8 were conventional and 2 were plexiform. Observed features included capsule (n=5), hyalinized vessels (n=5), Verocay bodies (n=7), and Antoni B pattern (n=5). Immunostaining included S100+ in 4/4 cases, collagen IV+ and Ki67<1% in 3/3 cases. Neurofibromas are the most common PNST involving the eye and ocular adnexa and the majority are associated with NF1. Plexiform and diffuse patterns and the presence of pseudo-Meissner corpuscles are relatively frequent in this area.

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Section: Discussioncontrasting

confidence: 58%

Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa

et al. 2017

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“…Possible interventions include (1) resection of tumor; (2) decompression of the orbit and/ or optic canal; and (3) radiation therapy/SRS. In our opinion resection of meningiomas arising or involving the optic nerve sheath is associated with unacceptable risk of vision loss ( 9 , 10 ). Although not the object of this study, resection may be a reasonable option for meningiomas extending into the orbit secondarily (e.g., anterior clinoid process or sphenoid wing meningiomas with extension into the orbit).…”

Section: Discussionmentioning

confidence: 84%

Intra-Orbital Meningioma Causing Loss of Vision in Neurofibromatosis Type 2: Case Series and Management Considerations

et al. 2018

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Objectives: Little evidence exists regarding the management of orbital meningioma causing vision loss in the setting of neurofibromatosis Type 2 (NF2). We review here our experience with patients at risk for blindness due to intra-orbital meningioma.Design/Setting/Participants/Main Outcome Measures: The charts of patients with NF2 presenting for evaluation of intra-orbital meningioma and vision impairment between 2008 and 2013 were retrospectively reviewed in accordance with institutional IRB policies. Patients with primarily extra-orbital tumors and minimal intra-orbital extension were excluded. Charts were analyzed for the presence and/or imaging progression of intra-orbital tumor, presence of other optic apparatus pathology, presence and/ or progression of vision impairment, and intervention performed (if any).Results: Seven patients with intra-orbital meningioma on MRI and bilateral vision loss and/or unilateral visual impairment due to tumor and contralateral blindness of any etiology were identified. Patients without salvageable vision in either eye were excluded (n = 3). Diagnosis of meningioma was obtained either by biopsy or based on imaging assessment.Conclusion: Conservative management of orbital tumors in NF2 may be preferred in asymptomatic patients but may not be acceptable in patients with progressive visual decline. Radiation is a reasonable option for meningiomas of the orbit and optic nerve sheath. Finally, although the benefit of cranial nerve decompression in NF2 for preservation of facial nerve and hearing has previously been established, the role of optic nerve decompression for preservation of vision in NF2 remains poorly defined.

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“…The differential diagnosis for a solitary optic nerve tumor includes an optic nerve sheath meningioma and an optic glioma. Meningioma is commonly associated with neurofibromatosis type 2 ( 27 ) and optic glioma is commonly associated with neurofibromatosis type 1 ( 28 ), while hemangioblastoma is commonly associated with the VHL disease. The patient had the VHL disease with a germline mutation of the VHL gene.…”

Section: Discussionmentioning

confidence: 99%

VHL-Associated Optic Nerve Hemangioblastoma Treated with Stereotactic Radiosurgery

Kanno

Osano²,

Shinonaga

2018

jkcvhl

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Central nervous system hemangioblastomas are generally restricted to the cerebellum, spinal cord, and brainstem. Supratentorial hemangioblastomas are uncommon, and optic nerve hemangioblastomas are extremely rare, with fewer than 25 reports including this case. In this report, we present the case of a 36-year-old woman with von Hippel–Lindau (VHL) disease who presented with progressive diminution of vision in the left eye due to a retrobulbar optic nerve hemangioblastoma. The patient had a history of cerebellar/spinal hemangioblastomas and pancreatic cysts, and her father and brother were patients with VHL disease. Gadolinium-enhanced magnetic resonance imaging showed intraorbital retrobulbar–enhanced mass on the left optic nerve. The optic nerve hemangioblastoma was treated with fractionated stereotactic radiosurgery using Novalis. Eighteen months after the stereotactic radiosurgery, the tumor volume decreased although the patient lost vision. This report presents an extremely rare case of optic nerve hemangioblastoma, which is the first case treated with stereotactic radiosurgery.

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Neurofibromatosis Type 2: Optic Nerve Sheath Meningioma in one Orbit, Intramuscular Schwannoma in the Other

Cited by 17 publications

References 9 publications

Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa

Clinicopathological features of peripheral nerve sheath tumors involving the eye and ocular adnexa

Intra-Orbital Meningioma Causing Loss of Vision in Neurofibromatosis Type 2: Case Series and Management Considerations

VHL-Associated Optic Nerve Hemangioblastoma Treated with Stereotactic Radiosurgery

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