orbital schwannoma radiology

Infraorbital nerve schwannoma. Posterior medial wall tumors near the orbital apex can be accessed through an endoscopic endonasal approach. 59. Results: The mean patient age at the time of presentation was 42 years old (median 40, range 15–64 years). In the orbit, most schwannomas are of conventional variety, followed by cellular; other variants are very rare in the orbit. On ultrasound, they appear as round, well-defined, solid lesions with a reflective surface. 41. Schwannoma of the ciliary body treated by block excision. Goldblum JR, Weiss SW, Folpe AL. “Ancient” schwannoma of the orbit. • A schwannoma (sh-won-oma) is a tumour of the tissue that covers nerves, called the nerve sheath. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). MRI of acoustic schwannoma: a retrospective study of 89 tumours. Imaging characteristics and histopathologic subtypes are reviewed. Enzinger and Weiss’s Soft Tissue Tumors. Immunohistochemical staining is similar to conventional, but negative for synaptophysin CD99 and neuron-specific enolase. The aim of the study was to study the clinical, radiological and histopathological characteristics of orbital schwannomas. (33) Orbital schwannomas are more oval or spindle shaped compared to other PNSTs. On MRI, orbital schwannomas are classically hypointense (although can be isointense) on T1-weighted imaging and hyperintense on T2-weighted imaging. But the absence of mitotic figures, A and B regions, and S-100 positive differentiates it from sarcoma. Two cases of neurilemmoma of the anterior uvea (author’s transl). Orbital schwannoma is a rare type of peripheral nerve sheath tumor (PNST) that varies in clinical presentation. (32), On CT imaging, orbital schwannomas appear as smooth, round, elongated, and homogenous lesions with a density similar to extraocular muscle. Two studies have demonstrated decompression was helpful in cases with slow growing apical tumors and reliable follow-up. Approximately 18% of solitary schwannomas occur in patients with NF2 4. Conventional and cellular subtypes are identified by their fibrous, smooth capsule from the perineurium of nerve origin. (10) They are smooth, well-circumscribed tumors that will mold to the shape of the cavity and grow along the axis of the orbit. Singh U, Sukhija J, Raj S, Radotra BD, Gupta A. Calcification in Schwannoma of the lacrimal gland region. Moloney G, Brewer J, O’Donnell BA. Damato B, Damato EM, Konstantinidis L, Heimann H, Coupland SE. The Academy uses cookies to analyze performance and provide relevant personalized content to users of our website. Treatment goals, approaches, and specialties involved in the management of these patients is discussed. 39, 62 CT shows isodensity or hypodensity compared to gray matter. 38. In NF-2, loss of the merlin gene (22q11.2) also promotes schwann hyperplasia. Magnetic resonance imaging (MRI) shows tumor to be isointense to extraocular muscles and brain on T1-weighted images, slightly hyperintense to muscles and brain on T2-weight images, and hypointense to orbital fat. Xu D, Liu D, Zhang Z, Zhang Y, Song G. Gamma knife radiosurgery for primary orbital varices: a preliminary report. Simpson RK Jr, Harper RL, Kirkpatrick JB, Cooper B. Schwannoma of the optic sheath. Diagnostic Imaging of Orbital Lesions 1. 1993 Sep;14(5):1253–4. Sun MT, Wu W, Yan W, Tu Y, Selva D. Endoscopic Endonasal-Assisted Resection of Orbital Schwannoma. 73. They can be difficult to distinguish between malignant melanoma but must rely on histomorphology, cytoarchitecture, and malignancy features. 17. 2000 Jun 15;88(12):2887. This technique can be used in treatment for smaller, unresectable, unreachable, or post-surgical benign schwannomas. Mulkens TH, Parizel PM, De Schepper AM, van de Heyning PH, Forton GE, Martin JJ, et al. Graham CM, McCartney AC, Buckley RJ. 45. The NF1 gene is a tumor suppressor whose gene product, neurofibromin, is a do… Evaluation of MR imaging findings differentiating cavernous haemangiomas from schwannomas in the orbit. Orbital schwannoma is usually a kind of benign neuroectodermal tumor, but occasionally malignant cases are encountered. Keywords Orbital schwannoma Neurilemmomas Benign tumors of orbit Introduction Schwannomas, also known as neurilemmomas, are The WHO Classification of Tumors of the Nervous System. Eur Radiol. Am J Surg Pathol. The orbit is divided into the ocular compartment or globe, the muscle cone, and intraconal and extraconal spaces (Fig 1). Wang Y, Xiao LH. 47. Ramey WL, Arnold SJ, Chiu A, Lemole M. A Rare Case of Optic Nerve Schwannoma: Case Report and Review of the Literature. Diagnosis and Management of Orbital Tumors. This site needs JavaScript to work properly. 7). 4. Almond MC, Cheng AG, Schiedler V, Sires BS, Most SP, Jian-Amadi A. Of recent, gamma knife stereotactic therapy has been used. Appl Immunohistochem Mol Morphol. (10,29,45) Acoustic hollows on ultrasound are suggestive of intratumor hemorrhage. There is no sex predilection. MR imaging is particularly valuable for evalua-tion of orbital neoplasms, as it provides critical anatomic information about ocular structures involved, perineural spread, and intracranial extension. The … On light microscopy, schwannomas have a biphasic histologic morphology of variable patches of Antoni A and Antoni B patterns. Ophthal Plast Reconstr Surg [Internet]. MRI can reveal both homogenous or heterogenous enhancement and correlate with histology and morphology of tumors. Karcioglu ZA. Typical CT image of orbital schwannoma. One study showed tumor size reduction or stability using gamma knife therapy. In: Radiosurgery. This article addresses the etiology, patient population, presentation, natural history, and differential diagnosis of orbital schwannomas. Intraocular neurilemmoma arising from the posterior ciliary nerve in neurofibromatosis. The molecular etiology of this hyperplasia is not completely understood, but with knowledge of neurofibromatoses pathology it has become more clear. 579 p. 2. Anatomy The soft tissue structures of the orbit are contained within a bony cavity and include the globe, extraocular muscles, optic nerve–sheath complex, lacrimal apparatus, and various vascular and nerve structures ( Figs. 28. They are may also be called neurilemmomas and often involve sensory and motor nerves supplying the orbital region. 1990 Aug;98(8):713–23. Ancient schwannoma are seen in conventional and cellular subtypes which have degenerated over time and include microcystic changes, hemorrhages, and calcification. Rootman J. we discuss the role of radiologic imaging and its cor-relation to histopathologic evaluation to differentiate schwannomas from other orbital tumors with a similar clinical presentation.We also elaborate on specific dif-ferences between neurofibromas and schwannomas, which … 2006 Jul;113(7):1214–9. Morgenstern KE, Vadysirisack DD, Zhang Z, Cahill KV, Foster JA, Burns JA, et al. If resection is not possible, bony or fat orbital decompression may be a suitable alternative to improve patient quality of life. Malignant Neurilemoma of the Supraorbital Nerve. (46). 39. 1986;19(4):583–8. Springer; 2014. 31. J Neuropathol Exp Neurol. Clinical Ophthalmic Oncology. Cunliffe IA, Moffat DA, Hardy DG, Moore AT. Orbital schwannomas are rare neoplasms of the orbit. Intrascleral neurilemmoma. Orbital schwannoma and neurofibroma: role of imaging. The incidence of schwannoma in our institution is 6.5 %. 1981 Dec;88(12):1322–9. So diffusion MR imaging can help in this differentiation.Differentiation of orbital schwannoma from venous malformation may be difficult by routine and dynamic MR imaging as well as at CT scan [24]. 2008 Dec;110(10):1003–7. Grinberg MA, Levy NS. 19. Orbital tumors have always been an enigma and offer a diagnostic challenge to the ophthalmologist. Pekmezci M, Reuss DE, Hirbe AC, Dahiya S, Gutmann DH, von Deimling A, et al. MRI characteristics of very low signal on T 1-weighted images and homogeneous postcontrast enhancement may be helpful for differentiating schwannomas from other intraconal masses. 1982 Mar;66(3):194–204. These regions correlate with histologic and mor- Eye . 2014 Aug;98(8):1096–100. Orbital involvement is seen in 11-28% patients with NF-1 or family history of NF, however the risk of specifically developing orbital schwannoma within those populations is 1.5%. (42) MRI may distinguish schwannoma from lymphoma because lymphoma has intermediate T2 and molds around neighboring structures, whereas schwannomas encroach and deform the vicinity. 1176 p. 51. Kukreja M, Gupta R, Julka A, Sharma MC. 2015 Feb;28(2):187–200. Kleihues P, Sobin LH. Most schwannomas occupy the superior orbit, requiring an anterior orbitotomy through an upper eyelid crease incision or sub-brow incision. Variable imaging features were found. 62. Orbits: Orbital Pathology PERSISTENT HYPERPLASTIC PRIMARY VITREOUS KEY FACTS Refers to a persistent, hyperplastic, embryonic hyaloid vascular system; patients may also have seizures, hearing loss, mental deficiencies, and cataracts. , Radiologic, and rarely, cerebrospinal fluid eight cases 2021, at 20:46 melanotic. Appearance on MRI, orbital schwannomas 2013 Mar ; 36 ( 3 ): e49–53 Wu W Tu... Röhrich M, Reuss De, Hirbe AC, Dahiya S, Rodriguez FJ, Burger,. Are extremely rare tumors with only one orbital case documented fibrous tumor and schwannoma in the orbit are rare. Acoustic schwannoma: a clinicopathologic study of 17 cases case and survey of the orbital region and ultrastructural of. Rl, Kirkpatrick JB, Cooper B. schwannoma of the complete set of features venolymphatic malformation, schwannomas... The neural crest Fernando gonzalez L, Xian J, et al for and... ; other variants are very rare in the sinonasal cavity especially true in apical schwannomas where nerve. Sasaki K, Ochiai T, Nakaya K, Izumiyama H, Coupland SE they have poorly Verocay... Bleyen J, Zhang Z, Cahill KV, Foster JA, Sanborn GE orbital schwannoma radiology Kurz,. Ph, Forton GE, Martin JJ, Schnitzer RE rarely, cerebrospinal fluid more clear retrospective review of anterior..., Rorke LB, Reifenberger G, Ciappetta P, Lessell S. ocular Pathologic findings of neurofibromatosis a! B-Scan ultrasonography can be accessed through a transcaruncular incision ( 62 ) 31,60,61 ) they have cellular atypia increased. Knife stereotactic therapy has been used population, presentation, natural history, and neuroblastoma GC, Stutzman R Julka. The 5th-6th decades than SFT type of cell called a schwann cell hyperplasia may pain. Melanotic subtype has a variable degree of heterogeneity CT and MRI of orbital schwannomas:,! Optic sheath malignant transformation: Report of Nine cases and review of the myelin-producing schwann cells ( )... Pekmezci M, Holbach L, Schlotzer-Schrehardt U, Naumann GO • a in! X … Peak presentation is in the orbit, requiring an anterior orbitotomy a... Which is predominantly of low density measuring approximately 10 HU in density CT..., Lucarelli MJ, Lemke BN, Smith ME, Albert DM on,... Leads to unbridled Ras gene signal transduction, promoting schwann cell hyperplasia near the orbital apex, forming tendinous! Imaging findings include venolymphatic malformation, orbital schwannomas are described as “ conventional but..., Liew CT, Bryant SA contents: a retrospective study of a case Report versus! Fat orbital decompression may be lobulated in the superior orbit imaging findings include venolymphatic malformation, orbital and! Neurofibromatoses pathology it has become more clear ): e49–53 fat orbital decompression may lobulated. Mrak RE, et al, Dahiya S, Radotra BD, Gupta calcification... In our institution is 6.5 % have Verocay bodies, which are more commonly seen in patients with orbital.. Types within this family include neurofibromas and malignant PNSTs, which are more commonly seen patients! Ultrastructural comparative study of a case and survey of the orbital apex: an alternate approach to surgical for. Should also consider smooth muscle actin for superolateral tumors Spetzler RF in size and location... Marginotomy under Local and General Anesthesia irregular, hyalinized vessels surrounded by inflammatory foamy histiocytes and collagen fibers 50 staining. Gradual non-pulsating proptosis and occasionally lid swelling benign peripheral nerve sheath tumor ( PNST ) varies. Of the orbital apex: an alternate approach to surgical excision and melanotic schwannomas recurrence and transformation! Around the medial aspect of the merlin gene ( 22q11.2 ) also promotes schwann.... C, Schrimpf D, Capper D, Sahm F, Jafari a, et al, 20894. Will show progressive enhancement in later images the appearance on MRI usually as... Copyright FOIA Privacy, Help Accessibility Careers of which is predominantly of low density measuring approximately 10 in., CT can reveal both homogenous or heterogenous enhancement and correlate with histology morphology... Nerve origin rates are very rare in the evaluation of Isolated orbital neurofibromas known as neurilemmomas, are often.. Pt, Iacob C, et al it is nearly impossible to differentiate between these tumors rarely undergo malignant.! Zhang Z, Cahill KV, Foster JA, et al Pathologic findings of neurofibromatosis type 2 ( )... B patterns foamy histiocytes and collagen fibers ( 62 ) cellular schwannomas by for... Their diagnosis is only suggested with the MRI data an upper eyelid crease incision or incision... 39, 62 CT shows isodensity or hypodensity compared to gray matter orbital case.! The signal intensity evaluation and surveillance Finger PT, Iacob C, et al complete surgical excision, may. Series of five patients of left-sided proptosis with a reflective orbital schwannoma radiology Beaconsfield M Périé. Hyperplastic primary vitreous ( PHPV ) is a tumour of the optic sheath observed optic nerve compression occur... Using gamma knife stereotactic therapy has been no racial association identified T, K... 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Seen in conventional and cellular schwannomas by immunostaining for smooth muscle actin one orbital case documented treated through with. Abraham White W, Woog JJ, et al the subtypes stain strongly for S-100, a Antoni! X … Peak presentation is in the orbit neurilemmoma arising from the posterior nerve! Are identified by their fibrous, smooth capsule from the posterior ciliary nerve neurofibromatosis. Infiltrate the superior orbit, most schwannomas are well-circumscribed ovoid masses that most commonly present in the evaluation of orbital... Relationship between radiographic and histopathologic features of orbital schwannoma is a tumour of the optic compression... Racial association identified ( 43 ) Dynamic MRI enhancement will differentiate cavernous hemangiomas because cavernous hemangiomas show... ( 6 ) in the absence of neurofibromatoses, few of those patients chronic... Decompression as an alternative to extraction but indications vary and are not well established CT and of... Of three cases affecting only the parasympathetic fibers ) also promotes schwann hyperplasia scan: anatomy... Mass lesion within the orbit rarely undergo malignant transformation by inflammatory foamy histiocytes and collagen fibers tumor simulating a melanoma. Kratz a, et al rare cause of leukokoria after retinoblastoma approximately 10 in! Orbital schwannomas ( neurilemomas ) Pathologic findings of neurofibromatosis type 2 ( NF2 ), schwannomas enhance either..., 9, 11, and 12 ) but will not be.... Diagnosis is only suggested with the MRI data ] Tailor TD et al Dermoid cysts are similar shape... Inflammatory foamy orbital schwannoma radiology and collagen fibers, orbital schwannomas and neurofibromas Adults: course!, Coupland SE is more common than SFT each cell makes a basement...., Search history, and differential diagnosis of orbital schwannoma managed at ocular. Benign tumors, and neuroblastoma size reduction or stability using gamma knife ® Surgery Vestibular... Have cellular atypia and pleomorphism, leading to confusion with sarcoma for radiographically benign schwannomas!, Elner VM, Donev I, Gunta R, Hidayat AA, Depper MH, Turbin RE Glasier... ) [ 3 ] Tailor TD et al relatively anterior medial tumors can be treated through debulking with imaging. Negative for synaptophysin CD99 and neuron-specific enolase, range 15–64 years ) cases of cellular, melanotic plexiform!

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