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.
Rachna Agarwal, Vikas Kanaujia, Priyadarshini Mishra, R. V. Phadke & Kumudini Sharma
We report a case of partial third nerve palsy resulting from a cystic lesion located at the orbital apex. Imaging was suggestive of cystic schwanomma but histopathology of the lesion confirmed epidermoid cyst, which is a rare tumour of the orbit.
Rubinstein, Tal J. M.D.; Repp, Daniel J. M.D.; Newell, David W. M.D.; Sires, Bryan S. M.D., Ph.D.
A 62-year-old male presented with a 1-year history of horizontal diplopia that was worse in left gaze. He was initially diagnosed with left abducens nerve paresis by a strabismus specialist. The strabismus specialist noted −1 abduction deficit of the left eye and 8 PD of esotropia at primary gaze. He additionally had 1.5 mm of relative exophthalmos of the left eye. An MRI of the orbits is shown in Figure 1. T1W coronal MRI identifies a heterogeneous, cystic-appearing anterior portion of a mass lesion superior to the superior rectus (Fig. 1A, arrow). T1W coronal MRI of the orbital apex shows a more homogenous, hyperintense portion of the tumor (Fig. 1B). T2W coronal MRI of the anterior portion of the lesion shows a homogenous hyperintensity, as often seen in cystic structures (Fig. 1C). T1W sagittal MRI with contrast depicts the posterior aspect of the tumor in the orbital apex (Fig. 1D, arrow). A craniotomy-approached orbitotomy was performed to completely excise the tumor. Pathological analysis revealed that the mass was a schwannoma. The origin was thought to be the frontal nerve based on its location in the orbit. Postoperatively, the patient’s diplopia completely resolved with almost complete resolution of his abduction deficit. Based on the tumor’s location outside both the muscle cone and cavernous sinus, the abduction deficit may have been caused by the tumor’s effect on the orbital fascial and pulley system with mechanical restriction or possibly by mechanical distortion of the abducens nerve or lateral rectus at the orbital apex.