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Schwannoma or neurilemmomas are benign tumours of neural origin. They arise from the Schwann cells present in the peripheral myelinated nerves. The head and neck region is the most common site of occurrence for schwannomas, followed by flexor aspects of limbs and trunk.
We describe an extremely rare case of a epibulbar nodular mass that was histopathologically confirmed as schwannoma in a 17-year-old Indian male patient.
The patient presented to our oculoplasty clinic with history of mass in the left inferior conjunctival region for the previous 2 years. The mass was painless and gradually increasing in size. There was no history of frequent redness, watering, itching, or any bleeding episodes. Systemic history was unremarkable.
On examination, his best-corrected visual acuity was 20/20 OU. Slit-lamp examination of the left eye revealed a well-circumscribed oval mass located at the 5 o’clock position over the inferotemporal bulbar conjunctiva (Fig. 1A). The mass was firm, nontender, freely mobile, and not adherent to the underlying sclera. The rest of the ophthalmic examination in both eyes was unremarkable. There was no lymphadenopathy.
Based on the findings a differential diagnosis of conjunctival inclusion cyst, conjunctival granuloma, allergic reactive lymphoid hyperplasia, or a parasitic cyst was considered. The patient underwent complete excision of the mass. Peroperatively the mass was found to be located subconjuctivally without any underlying scleral adhesions and was easily removed in toto. Conjunctiva was approximated with the help of electrocautery.
Histopathologic examination revealed a well-encapsulated tumour composed of spindle cells intermixed with tiny blood vessels. Numerous Verocay bodies could be seen. There was no nuclear atypia. The findings were consistent with schwannoma (Fig. 1B). Immunohistochemistry was strongly positive for S-100, confirming the diagnosis of schwannoma (Fig. 1C).
The patient did not report for a follow-up after surgery and hence was contacted over phone after 6 months. There was no recurrence.
Conjunctival schwannoma is an extremely rare entity. No case of conjunctival schwannoma was found in a review of 1643 conjunctival tumours by Shields et al.,
did not have any clinical details. The rest of the reported cases did not show any sex predilection with 6 being female and 4 male. Patient age ranged from 10 to 72 years. Seven cases had bulbar involvement, 2 were palpebral, and 1 case involved the fornix. Our case also involved the bulbar conjunctiva. Except for 2 cases,
reported a series of 3 cases of conjunctival schwannoma in 1997. Interestingly, all the cases in the series were females. Five out of total 12 reported cases (41.66%; including the present case) were below 20 years of age, signifying a predilection for adolescent age group. The details of all the cases reported to date have been summarized in Table 1.
Table 1Table depicting the details of all reported cases of conjunctival schwannomas to date
Histopathological features of conjunctival schwannomas are no different from schwannomas affecting the rest of the body. Two distinct patterns (i.e., Antony A and Antony B) are seen. The former is more cellular and consists of tightly packed spindle cells with spindle-shaped nuclei forming Verocay bodies, whereas the latter has sparse cells and more of myxoid stroma.
Immunohistochemically, they are positive for the neural crest marker S-100.
Complete excision is the treatment of choice for conjunctival schwannoma. A double freeze–thaw cryo can be applied to the underlying sclera bed if the mass is found to be adherent to underlying sclera peroperatively.
To conclude, conjunctival schwannomas are rare tumours with very few cases described in the literature. They should be considered in the differential diagnosis of any subconjunctival nodular mass. Complete excision offers cure in most of the patients without any recurrence.