# Extranodal natural killer/T-cell lymphoma with tonsil involvement: a case report

## TL;DR

A rare case of a type of lymphoma involving the tonsils in a child was successfully treated with chemotherapy.

## Contribution

This paper reports a rare pediatric case of ENKTL with tonsil involvement and its successful treatment with the SMILE regimen.

## Key findings

- A 13-year-old girl was diagnosed with stage II ENKTL involving the tonsils.
- The patient achieved complete response after six cycles of SMILE chemotherapy and remained recurrence-free.
- Immunohistochemistry and genetic testing confirmed the diagnosis of ENKTL.

## Abstract

Extranodal natural killer/T-cell lymphoma (ENKTL) with tonsil involvement is not common, especially in children.

A 13-year-old girl presented with an unexplained sore throat for more than 2 months, together with intermittent fever and suppurative tonsilitis. Nasopharyngoscopy revealed a pharyngeal mass. Enhanced computed tomography (CT) scan showed tonsillar hypertrophy and punctate calcification. Chronic pyogenic granulomatous inflammation with pseudoepithelial squamous epithelial hyperplasia was observed in left tonsil, and pyogenic granulomatous inflammation and a small number of T-lymphoid cells were detected in the right tonsil. The immunohistochemical results showed CD2+, CD3+, CD4+, CD5+, CD8+, granzyme B+, and TIA-1+. The Ki-67 proliferation index was 20%. The case showed T cell receptor gene rearrangement. Finally, the case was diagnosed as ENKTL of stage II with tonsil involvement. The patient received 6 cycles of chemotherapy with SMILE regimen, and showed complete response with no recurrence in the follow-up.

We presented a rare case of ENKTL with tonsil involvement in a child. The patient showed complete response to the SMILE chemotherapy with no recurrence.