PRIMARY UNILATERAL CONJUNCTIVAL SMALL LYMPHOCYTIC LYMPHOMA IN A CHILD: A RARE CASE REPORT

Ocular adnexal lymphomas, or orbital lymphomas, are extranodal lymphomas primarily involving the ocular adnexa, which includes the conjunctiva. Ocular adnexal lymphomas are considered primary if involvement of lymphoma is confined to ocular adnexa alone and secondary if there is lymphoma of identical type present at another site. Conjunctival lymphomas are uncommon in children, with very few reported cases in published literature, none of which were small lymphocytic lymphomas. We report a case of small lymphocytic lymphoma confined to the conjunctiva, in a 15-year-old child, with full paediatric oncology examination. To our knowledge, this is first case to be reported of a child with primary unilateral conjunctival small lymphocytic lymphoma.

A 15-year-old male presented with a 2-month history of a painless, slowly growing conjunctival peribulbar lesion in the left eye. His medical history was otherwise unremarkable except for G6PD deficiency. He had a prior ocular history of childhood chronic viral keratoconjunctivitis which was successfully treated. The external examination of the left eye was notable for a perilimbal, mobile, elevated, salmon-coloured, globoid temporal conjunctival lesion, measuring 10x7mm, surrounded by dilated vessels. Rest of ocular examination was unremarkable. Ultrasound biomicroscopy (UBM) was done and showed no scleral extension.

Then the patient underwent a complete excisional biopsy using the “no touch” technique with 4 mm conjunctival margin, followed by cryotherapy of the conjunctival margins. The pathology revealed a nodular proliferation of small lymphoid cells with clumped chromatin and scant cytoplasm confirming the diagnosis of conjunctival small lymphocytic lymphoma (SLL). Paediatric oncology evaluation was unremarkable. A positron emission tomography, scan revealed moderate fluorodeoxyglucose avidity in bilateral upper cervical lymph nodes which were considered to reflect an inflammatory process rather than nodal lymphoma. A final clinical diagnosis of primary unilateral conjunctival small lymphocytic lymphoma was made. The patient is currently on a regular follow up.

Small lymphocytic lymphomas of the ocular adnexa are rare, especially in the conjunctiva. Lymphomas are one of the most common malignant conjunctival neoplasms, however they may be clinically missed due to lack of symptoms. Therefore, ophthalmologists must be vigilant for the distinctive appearance of these neoplasms and keep a low clinical threshold for suspecting lymphoma in patients with chronic conjunctival lesions. Tissue biopsy and histopathology examination are critical for diagnosis. Staging and systemic evaluation are important for formulating a proper treatment plan. And finally, long-term follow-up is necessary for conjunctival lymphomas, as systemic lymphoma may develop after many years.