Lymphomatoid granulomatosis presenting as granulomatous orchitis

Abstract

Background Lymphomatoid granulomatosis (LYG) is an angio-centric and angiodestructive lymphoproliferative disorder which involves the lungs, skin, central nervous system, liver, spleen and kidneys. Only a single case of testicular LYG has been reported. LYG is an EBV driven process, with demonstration of EBV positive large B cells required for diagnosis. Case presentation We report a case of a 51-year-old man with a testicular mass. At orchidectomy, a polymorphous lymphoid infiltrate was present incorporating scattered, large, atypical cells with a vaguely granulomatous appearance. A polyclonal B cell population was demonstrated on flow cytometry. The case was reported as a granulomatous orchitis. Five months later he developed leg ulcers and bilateral pulmonary infiltrates. He was diagnosed with LYG and responded to treatment with R-CHOP. Five years later he developed further nodular infiltrates within the lungs and leg ulceration. A wedge biopsy of the lung showed an angiocentric lymphoid infiltrate with numerous large, atypical, CD20 positive B cells showing EBV positivity. Thus, a histological diagnosis of LYG was made. Conclusion Lymphomatoid granulomatosis is a rare lymphoproliferative disorder, with only a single case report of testicular involvement. It may be considered in the differential diagnosis of granulomatous orchitis or testicular lymphoma.