Kai-Ling Yong, Timothy J Beckman, Matthew Cranstoun, Timothy J Sullivan
To evaluate the clinical features, management, and outcomes of orbital schwannomas.
Retrospective study analyzing 20 orbital schwannomas in 18 patients managed in an orbital service over 26 years. Clinical, radiological, histological, and surgical procedural data were analyzed.
Mean age of the study population was 40.89 ± 20.84 years. The most common nerve of origin was frontal nerve (50%), and majority of patients (70%) had a superior and/or posteriorly located tumor. Five patients (27.8%) had optic neuropathy at presentation, and 3 of them showed improved vision after intervention. Surgical resection was performed for all except for 1 patient who underwent fractionated stereotactic radiotherapy. Six subjects had extension of tumor into the cavernous sinus, and 3 of them underwent combined neurosurgical intervention with stereotactic neuronavigation. Outcomes were largely favorable with majority achieving complete excision. Two out of 6 incomplete resections experienced regrowth requiring second surgical intervention. Surgical complications include decreased vision, diplopia, ptosis, and mydriasis.
Orbital schwannomas are uncommon slow-growing tumors which can result in visual loss. Prognosis can be favorable even in presence of pre-operative optic neuropathy if there is early intervention. Schwannomas confined to the orbit can usually be completely excised with good outcomes. Schwannomas extending intra-cranially may undergo incomplete excision to preserve vital structures, leaving residual disease, but generally have satisfactory results. Stereotactic neuronavigation and frozen section are useful intra-operative tool to aid management and minimize complications. Fractionated radiotherapy can be considered as an alternative or adjunctive treatment for patients not amenable for surgical resections
Jason D. Wesolosky, Gabriela Campos-Baniak, Vikas Sharma, Ezekiel Weis
Traditionally, fast-growing tumours are thought to be of malignant origin.1 Schwannomas are relatively rare and are typically slow growing. They constitute approximately 1% of all intraorbital tumours. They typically originate from the intraorbital divisions of trigeminal V1, although other sensory nerves can be involved.2 Schwannoma of oculomotor origin is rarer, with just 40 cases in adults and children described in the literature3 and with only 1 case previously published of a schwannoma of the inferior oblique branch of the inferior division of the oculomotor nerve.4 We present a case of a fast-growing schwannoma in a 6-year-old male who presented with a progressive unilateral proptosis and rapidly compressive optic neuropathy secondary to a tumour of the inferior oblique branch of the inferior division of the oculomotor nerve.
Rachna Agarwal, Shabari Pal, Nandita Chaudhary, Kumudini Sharma, Vikas Kanaujia
Schwannomas involving the perilimbal conjunctiva is an extremely rare clinical entity and has been reported scantily in literature. In this case, a 27-year-old healthy female presented with a pinkish smooth globular mass involving the limbus in the superonasal quadrant of the left eye. The mass was completely excised and histopathology revealed an encapsulated tumor, with cells displaying elongated nuclei, bland chromatin, and few Verocay bodies with palisaded nuclei, suggestive of schwannoma. Immunohistochemistry showed strong S100 positivity. This case thus highlights schwannoma as a differential diagnosis of limbal masses.
Sai Divya Jajapuram, Dilip K Mishra, Swathi Kaliki
Schwannoma is a rare benign tumor, which is more commonly found elsewhere in the body along myelin-producing peripheral nerves and is rarely reported within the eye. In this report, we describe two adult patients presenting with a choroidal mass lesion and neovascular glaucoma who underwent enucleation with clinical differential diagnoses of choroidal melanoma and choroidal hemangioma. Histopathology confirmed the diagnosis of choroidal schwannoma. This case series highlights the variable presentation of intraocular schwannoma and how they masquerade as other choroidal tumors.
Stevenson, Louis J.; McElnea, Elizabeth M.; McKelvie, Penelope A.; Hardy, Thomas G. Less
A 68-year-old female presented for assessment of a space occupying lesion of her right orbit, demonstrated on MRI. An upper lid crease anterior orbitotomy was performed and the lesion excised completely. Postoperatively, she had reduced sensation in the distribution of the supraorbital nerve. Histopathologic examination of the excised lesion revealed a hybrid neurofibroma/schwannoma. This represents the fourth reported case of such a lesion arising within the orbit.
Donghun Lee, Won Jae Kim, Myung Mi Kim
Isolated schwannomas of motor nerves to extraocular muscles are uncommon. In addition, most previous studies on oculomotor nerve schwannoma discuss adult patients, and pediatric cases are rare. We report a 10-year-old girl who developed recurrent oculomotor nerve palsy caused by schwannoma without any vascular malformation. Although the incidence is rare in pediatric patient, the recurrent isolated oculomotor nerve palsy due to schwannoma can develop, and it should be considered in the differential diagnosis of ocular motility disorders in pediatric patients. Careful imaging evaluation is needed to identify schwannoma due to its small size, deep location in the brain, and rarity.
Pointdujour-Lim, Renelle; Lally, Sara E.; Shields, Jerry A.; Eagle, Ralph C. Jr; Shields, Carol L.
Purpose: To evaluate the relationship between radiographic and histopathologic features of orbital schwannoma.
Methods: Retrospective review of 15 patients with orbital schwannoma managed at an ocular oncology service.
Results: The mean patient age at the time of presentation was 42 years old (median 40, range 15–64 years). The orbital schwannoma was found incidentally (n = 2) or with symptoms of proptosis (n = 2), blurred vision (n = 3), pain (n = 3), eyelid swelling (n = 2), diplopia (n = 2), or headache (n = 1). The mean duration of symptoms was 15 months (median, 9; range 1–60 months). The tumor occupied the superior (n = 11) or inferior (n = 4) orbit. Antero-posterior tumor location involved the anterior (n = 2), middle (n = 3), posterior (n = 4), or entire (n = 6) orbit. MRI was performed in 12 patients (80%) and CT was the only form of imaging in 3 patients (20%). The T1-weighted MRI (n = 11) showed the mass as isointense (n = 10) or hyperintense (n = 1) to the extraocular muscles). On T2-weighted MRI (n = 10), the mass demonstrated hyperintensity (n = 9) or hypointensity (n = 1). Histopathologic assessment demonstrated Antoni A (n = 12) and Antoni B (n = 12) patterns. Antoni A pattern correlated with hyperintensity on T1-weighted MRI and hypointensity on T2-weighted MRI. Antoni B pattern correlated with hypointensity on T1-weighted MRI and hyperintensity on T2-weighted MRI. As Antoni B approached >50% of the solid mass (n = 8), both T1- and T2-weighted MRI images were more likely to be heterogeneous (n = 7, 88% and n = 6, 75%, respectively).
Conclusion: Orbital schwannomas are nearly always benign, well-encapsulated slowly progressive tumors. Due to the biphasic distribution of Antoni A and Antoni B pattern, the appearance on MRI has a variable degree of heterogeneity. Antoni A pattern correlated with hyperintensity and Antoni B pattern correlated with hypointensity on T1-weighted MRI.
Yufei Tu, Frederick A. Jakobiec, Katherine Leung & Suzanne K. Freitag
An orbital neoplasm in children is an uncommon clinical finding. Clinical suspicion should be based on many factors, including its location, the nature of onset, associated systemic signs and symptoms, family and social histories, examination findings, and radiographic characteristics. We present two cases of young children of similar age with a rapid-onset orbital mass. In both cases, a circumscribed round lesion was found in the superomedial orbit. An orbital schwannoma, a benign and usually slow growing tumor, was found in the first patient. In contrast, the biopsy of the second patient, who was nearly asymptomatic, revealed a rhabdomyosarcoma. In this review, we have explored the differential diagnosis of relatively common circumscribed round orbital tumors in the pediatric population from both the radiographic (magnetic resonance imaging, MRI) and histopathologic perspectives. A review of highly unusual orbital tumors in children is also provided.
Szymanski L.J, Sibug Saber M.E., Kim J.W, Go J.L, Zada G, Rao N, Hurth K.M
Purpose: The aim of this study was to describe a 34-year-old male with hereditary bilateral retinoblastoma treated with radiotherapy as a child who developed 4 distinct tumors within the radiation field. Methods: A 34-year-old male with bilateral retinoblastoma status postradiation therapy and recurrence requiring enucleation presented with left-eye visual acuity changes. Magnetic resonance imaging demonstrated a left orbital mass and a right parasellar complex lobulated mass (right sphenoid and right cavernous sinus). Two weeks later, the patient underwent excision of the orbital mass and biopsy of an upper-lid nodule. This was followed by craniotomy for removal of the complex mass. Results: Histology revealed 4 distinct tumors, including an undifferentiated pleomorphic sarcoma (left orbit), a radiation-induced meningioma (right sphenoid), a schwannoma (right cavernous sinus), and a basal-cell carcinoma (left lid). Conclusion: Although occurrence of a second neoplasm is a well-known outcome following radiation treatment in patients with hereditary retinoblastoma, the diagnosis of 4 additional neoplasms is rare. Pleomorphic sarcoma, radiation-induced meningioma, and schwannoma are uncommon tumors and not well represented in the literature describing irradiated retinoblastoma patients. Secondary malignancies are a leading cause of early death in retinoblastoma survivors, and long-term follow-up is crucial for patient care.