PRIMARY TESTICULAR LYMPHOMA AND CONTROLATERAL CRYPTORCHIDISM

Background: Testicular lymphoma accounts for 1%-9% of all testicular neoplasm and 1%-2% of all non-Hodgkin lymphomas, with an estimated incidence of 0.26/100,000 per year. Although uncommon in general, lymphomas of the testis are the most common testicular malignancy in men 60 years of age and older, and may be the primary and only manifestation of malignant lymphoma, that is the initial sign of a systemic disease. Secondary involvement of the testis in patients with lymphoma is fare more common than primary testicular lymphoma.Various reports have advocated prior trauma, cronic orchitis, cryptorchidism, and filariasis of the spermatic cord, as potential risk factors, but predisposing causes for the development of testicular lymphoma are still unclear. We describe a case of diffuse large B-cell testicular lymphoma associated with controlateral cryptorchidism. Case Report: A 63-year-old man, apparently in good health, came to our attention for left testicular/scrotal swelling of several months duration, associated with rare scrotal pain during sexual intercourse, previously treated with different courses of antibiotics. Scrotal examination demonstrated a firm, painless, palpable testicular mass within the left hemiscrotum. Ultrasound scan showed the left testicle to be enlarged, with two hypoechoic 3.4 and 2.7 cm heterogeneous vascular masses with no involvement of the adiacent epididymis, and an hypoplasic controlateral testis with an hyperechoic streak (microlithiasis) within an homogeneous hecotexture. Normal blood flow was found on Doppler ultrasound. Medical history revealed a previous surgical treatment for right cryptorchidism and the results of an accurate physical examination and blood testincluding testicular cancer markers, were no contributory. The patient underwent left orchidectomy and pathological features, according to the WHO classification system, showed the presence of diffuse large B-cell lymphoma (CD45+, CD20+, vimentin+), with no involvement of the epididymis, tunica albuginea or spermatic cord. Complete haematological and biochemical examinations, total-body computerized tomography, and bone marrow aspirate and biopsy did not show extratesticular involvement of the disease, and the case was classified according to the modified Ann Arbor Staging system as a stage I-E disease. The patient is receiving chemotherapy by means of R-CHOP (cyclophosphamide, doxorubicin, vincristine and radiation therapy). Conclusion: Primary non-Hodgkin lymphoma of the testis is a rare disease associated with a high incidence of recurrence, even years after complete response, and a poor prognosis. Diffuse large-B cell testicular lymphoma is the most common histotype in primary forms, and has a high incidence of bilateral involvement and shows propensity for extranodal spread to the skin, subcutaneous tissue, central nervous system, lung and Waldeyer’s ring. Stage and pathological grading are the most important predictive factors for outcome, and further optimal treatment following orchidectomy is still unclear because the rarity of this disease, and because of the lack of prospective or randomized studies. Concerning the risk factors, there are no well-documented predisposing causes for primary testicular lymphoma, and anectodal reports associated with trauma, cronic orchitis, and cryptorchidism have been published. It is interesting to note how the case described is associated with controlateral cryptorchidism and testicular microlithiasis, both considered predisposing factors for testicular cancer; therefore the patient should have short- and long-term follow-up due to the increased risk of controlateral recurrence of lymphoma. References 1 Lantz AG, Power N, Hutton B and Gupta R: Malignant lymphoma of the testis: a study of 12 cases. CUAJ 3(5): 393-398, 2009. 2 Vitolo U, Ferreri AJ and Zucca E: Primary testicular lymphoma. Crit Rev Oncol Hematol 65(2): 183-189, 2008. 3 Vural F, Cagirgan S, Saydam G, Hekimgil M, Soyer NA and Tombuloglu M: Primary testicular lymphoma. J Natl Med Assoc 99(11): 1277-1282.