An 88-years old man comes to the urology department because of 3 weeks persisting oedema and itching of the right testicle, without any associated signs or symptoms. On examination right testicle appeared enlarged, tender and sensitive on palpation. Left testicle was asymptomatic.
First US scan revealed enlarged right testicle and epididymis with heterogenous sonographic appearance and hyperperfusion on Power Doppler US. Minimal hydrocele was observed. Left testicle appeared unaffected, except for 6 mm cystic formation in the head of epididymis.
5 weeks later the second US scan revealed progression of changes with spreading to the spermatic cord and persisting minimal hydrocele.
Third US scan (2 weeks after the second one) revealed obvious spreading of changes along the funiculus and testicular vessels to the abdominal cavity reaching the caudal pole of left kidney.
Contrast enhanced CT of abdomen demonstrated soft tissue mass within the right testicle, extending through the right spermatic cord into the retroperitoneal space along the testicular vessels. Axial diameter of the tumor at the level of the kidney vessels was measured to cca 50 x 40 mm. Around this area multiple pathologically enlarged (up to 20 mm) lymphatic nodes were visible. Tumor infiltration of the right ureter caused moderate right-sided ureterohydronephrosis.
Primary testicular non-Hodgkin’s lymphoma (PTL) is a rare extranodal lymphoma accounting for 1–9% of testicular malignancies and 1–2% of non-Hodgkin's lymphomas. Histologically the most common subtype of PTL is diffuse large B-cell lymphoma accounting for 80-90 % of cases, other subtypes include Burkitt and follicular lymphoma. As was also seen in our case it usually affects males aged over 60 years [1-3].
As in our case the most common clinical presentation of PTL is a firm, painless testicular mass without preference for either side, inseparable from the affected testicle. Constitutional symptoms including weight loss, night sweats, anorexia, fever, and weakness are infrequent. Associated hydrocele is observed in 40 % of patients. Synchronous bilateral involvement occurs in around 35 % of cases. [5, 6] Central nervous system, skin and pleura are commonly reached due to clear extranodal tropysm of PTL .
Initial diagnostic modality is usually ultrasonography, which demonstrates an enlarged testicle with focal or diffuse areas of hypo-echogenicity with increased blood flow on Power Doppler. These changes were indicative for orchyepididymis therefore antibiotic therapy with Ciprofloxacin followed by Amoxicillin/Clavulanate was prescribed. US controls showed progression of the disease, consequently, CT scan was indicated, enabling further bilateral evaluation, visualization of the paratesticular spaces and spermatic cord, hematogenous spread and staging [1, 7]. For staging of PTL, the Ann Arbor system is used. The diagnosis of PTL is usually confirmed through orchiectomy or testicular biopsy . Our patient’s biopsy report confirmed large B-cell lymphoma, non-GCB type which has the worst progression-free survival and overall survival .
PTL is an aggressive malignant with a poor prognosis, especially in cases of relapse. Treatment usually consists of orchidectomy (diagnostic and therapeutic procedure), followed by chemotherapy, radiotherapy or a combination of both [1, 3]. Prophylactic radiotherapy of the contralateral testicle and central nervous system prophylaxis should be considered [5, 7]. In most patients even with initial stage disease relapse still occurs in the first 2 years, affecting mainly these organs . A possible explanation for this special tropism to the immune-privileged sides is due to special immune escape phenotype of the lymphoma which may enable evasion of the host antitumor response .
Teaching points: Primary testicular non-Hodgkin’s lymphoma most commonly occurs patients older than 60 years, presenting with usually unilateral, unpainful swelling of the testicle. US appearance of enlarged, heterogenous, hyperemic testicle suggests the diagnosis, which is confirmed by testicular biopsy or orchidectomy. CT scan or MRI are important for assessment of tumour spread and staging.
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