Rosai-Dorfman disease mimicking testicular malignancy in a patient with marginal zone lymphoma of the lung

Lee-Diaz E.; Vaughan V.C.; Zhang L.; Pan Z.; La Rosa F.G. · BMJ Case Reports (2026) · DOI: 10.1136/bcr-2025-265657

Resumen

Rosai-Dorfman-Destombes disease (RDD) is a rare, benign histiocytic disorder, most often involving lymph nodes but showing extranodal manifestations in nearly 50% of cases, particularly in the skin and upper respiratory tract. A hallmark histological feature is emperipolesis—large CD68+ andS100+ histiocytes containing intact inflammatory cells. We report a unique case of bilateral testicular RDD in a man with prior extranodal marginal zone lymphoma of the lungs. Three years after lymphoma remission, he presented with bilateral testicular enlargement. Ultrasound and positron emission tomography/CT showed diffuse hypoechoic parenchyma with metabolic activity suggestive of malignancy. Bilateral orchiectomy revealed tan-yellow, fleshy masses infiltrating the testicular parenchyma. Histology confirmed RDD with emperipolesis and S100+/CD68+/CD1a− phenotype. Testicular RDD is extremely uncommon, with only ~15 cases described to date, and its association with haematological malignancy remains poorly understood. Recognition of this entity is essential to avoid unnecessary oncological treatment. © BMJ Publishing Group Limited 2026. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.

Instituciones: Universidad Peruana Cayetano Heredia