Background: Angiocentric lymphoma developed in a 6.5-year-old boy with acute lymphoblastic leukemia of thymic origin in remission. He presented with unilateral testicular enlargement 17 months after diagnosis.
Methods and results: Biopsy of the testis revealed an angiocentric, angiodestructive polymorphous cellular infiltrate accompanied by necrosis. The lymphoid component showed a mature T-cell immunophenotype (positive for CD2, CD3, CD5, CD7, CD4, CD8).
Conclusions: Angiocentric lymphoma is a rare entity included in the spectrum of angiocentric immunoproliferative lesions, which preferentially involves extranodal sites. Although four cases of angiocentric immunoproliferative lesions have been reported in pediatric patients with acute lymphoblastic leukemia, this is the first reported occurrence of testicular involvement.