CASE REPORTS
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A 60-year-old male with B-cell testicular lymphoma presenting as epididymo-orchitis
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The case of a man presenting with a non-resolving epididymo-orchitis, based on clinical evaluation and ultrasonographical evidence, which subsequently was diagnosed as primary testicular lymphoma.
Corresponding Author: McDonald, JR. Wrightington, Wigan and Leigh NHS Foundation Trust, UK. jawad.husain@wwl.nhs.uk, jrmcdonald@doctors.org.uk
Additional Authors: Husain, J; Thompson, A; Dauleh, MI
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Primary testicular lymphoma (PTL) is the most common form of testicular cancer in men over the age of 60 (1), and accounts for 7% of all testicular tumours (2). It usually presents as a unilateral, painless scrotal swelling. In this study we describe the case of a man presenting with a non-resolving epididymo-orchitis, based on clinical evaluation and ultrasonographical evidence, which subsequently was diagnosed as primary testicular lymphoma.
Case report: A 60-year-old male was referred acutely by the primary care physician to a regional district general urology department in the UK. He presented with a 2 month history of a swollen left hemi-scrotum that had become worse over the last 2 weeks. It was intermittently tender, responding partially to repeated courses of oral antibiotics. He had no associated symptoms of a lower urinary tract infection. He had been diagnosed and treated as having severe epididymo-orchitis.
An ultrasound of the scrotum, requested by the general practitioner 4 weeks prior to referral, had shown diffuse heterogenicity throughout most of the left testicle with hyperaemia. The findings were consistent with significant acute epididymo-orchitis (Figures 1 & 2)
Following examination of the testicle by a senior urologist, the diagnosis of a non-resolving epididymo-orchitis was made and an ultrasound performed to rule out a testicular abscess. The ultrasound showed severe epididymo-orchitis extending along the spermatic cord. There was a small hydrocoele but no abscess. There was adequate perfusion on colour Doppler (Figures 3 & 4).
Intravenous antibiotics were commenced to treat the suspected infection. Tumour markers were performed (AFP=2, BHCG<1). The pain resolved on day 8 of the admission, but on the morning of intended discharge he developed a new, severe left sided renal colic.
Subsequent intravenous urogram revealed an obstructed left ureter with extravasation of the contrast. A left hydronephrosis was seen with no intra-ureteric calculi (Figures 5 & 6). CT examination revealed a ureteric obstruction secondary to para-aortic lymph nodes (Figure 7). The patient remained apyrexial throughout the admission.
In view of these findings, a primary testicular neoplasm was suspected and the patient underwent a left orchidectomy and insertion of left JJ stent, to relieve the hydronephrosis. Histological examination revealed Non-Hodgkin’s B cell lymphoma which had completely replaced the normal testicular tissue. Staining revealed an atypical B cell phenotype that stained positive for CD5, CD20, bel-2, MUM 1 2 microglobulin (focal and cytoplasmic), and negative for CD3, CD10, CD23, P53. The proliferative fraction was 80-90%. He was staged at IIEA using the Ann Arbour Classification for lymphoma. He subsequently underwent 8 cycles of R-CHOP chemotherapy regimen, with 4 cycles of intrathecal methotrexate and radiotherapy to the contralateral testicle.
Comment: Primary testicular lymphoma (PTL) is a rare cancer representing 7% of all testicular tumours (2). It accounts for 1% of all non-Hodgkin’s Lymphomas (3) and represents up to 38% of bilateral tumours (4). It is the most common testicular tumour in the patient age range 60-80 (1) and has a mean age presentation of 64 (5). 80-90% of PTLs are diffuse large B-cell type (DLCL)1,3,4,6, with Burkitt and Burkitt-like cell type making up the remainder. Rarely T-cell or follicular cell tumours have been reported (10,11). Lymphatic spread is to the para-aortic lymph nodes with systemic spread to the CNS (6% - 16%), skin (0% - 35%), Waldeyer’s ring (5%), lung, pleura and soft tissue3.
Clinical presentation is usually of a painless, unilateral testicular swelling (1,2,6). Symptoms of occasional sharp pain have been documented, with cases of abdominal pain with ascites being reported due to large retroperitoneal lymph nodes (7). Associated B symptoms (Fever, night sweats, weight loss) usually present only in advanced stages, accounting for 25% to 41% of patients at diagnosis8. An Ovid-Medline search failed to reveal a documented case report of PTL presenting with the same clinical features and sonographic evidence as epididymo-orchitis. The search included the key words: Lymphoma and Epididymo-orchitis (in the English Language). There were 287 positive matches; the abstracts of each being reviewed where available.
PTL is diagnosed by histological assessment following radical orchidectomy. Ultrasound is often the first diagnostic procedure performed in patients with suspected testicular malignancy, along with being the investigation of choice in un-resolving epididymo-orchitis. However, typical gray-scale findings of lymphoma do not allow confident differentiation from primary testicular tumours or focal orchitis since the appearances of the neoplastic and inflammatory processes are non-specific12.
Mazzu et al (1995)12 reviewed eight patients with lymphoma (n=6) and leukaemia (n=2) with gray-scale ultrasound and colour Doppler, before reviewing the available literature. They found enlargement of the testis in all cases and reduced echogenicity in four cases. Colour Doppler sonography allows simultaneous interrogation of gray-scale tissue appearance and evaluation of blood flow (12). This allows assessment of vascularity which correlates with tumour size (13) and permits evaluation of round cell infiltration (12). Diffuse round cell infiltration becomes more apparent with increased vascularity. This helps to differentiate the neoplastic and the inflammatory. Ultrasound findings must be correlated with clinical evidence and, as in the case presented here, the diagnosis of epididymo-orchitis remains high in the differential diagnosis in the presence of pain and epididymal enlargement (12).
Following staging, using the Ann Arbour staging for Lymphoma, radical orchidectomy is performed, in order to gain local tumour control and eliminate the blood-testis barrier (9), thus increasing the efficacy of subsequent chemotherapy. Adjuvant chemotherapy is offered regardless of stage. The exact chemotherapy regimen remains controversial but is usually based on an anthracycline based regimen such as R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone). Localised disease (stage IE) has a two year relapse rate of 50% (3) with a 5-year survival of 70-79%1. Adjuvant chemotherapy for stage IAE PTL improves relapse free survival from 50% to 75% at five years (14) though overall prognosis remains poor16. Survival worsens with increased systemic disease involvement15. The presence of disseminated disease carries a bleak outlook with a median survival of less than one year after diagnosis4.
Primary testicular lymphoma is a rare cancer with a poor outcome. It usually presents between ages 60-80 as a painless, unilateral scrotal swelling. In this report a case of Non-Hodgkin’s B cell lymphoma of the testicle is presented following treatment for acute epididymo-orchitis, based both on the clinical finding and radiological evidence. This presentation of PTL has not been previously reported, as far as we are aware.
Initial diagnosis may be alluded to by ultrasonography; however, the appearances are difficult to differentiate from that of inflammation, especially if the history and clinical findings suggest this to be the most likely differential. Colour Doppler ultrasound can aid in diagnosis and assessment of vascularity can assist in distinguishing neoplastic from inflammatory processes. However, this has limitations and strong clinical suspicion may be a more reliable guide to diagnosis than ultrasonographical evidence. This report raises the question as to whether elderly male patients, with non-resolving epididymo-orchitis, should undergo early CT investigation to rule out testicular malignancy, in spite of ultrasonographical evidence of inflammation.
Figures 1 & 2. Greyscale ultrasound and colour Doppler of left testicle prior to hospital admission
Figures 3 & 4. Greyscale ultrasound and colour Doppler of left testicle following hospital admission
Figures 5 & 6. Intravenous urogram at 7 & 15 mins post injection of contrast showing left hydronephrosis & extravasation of contrast
Figure 7. CT urogram at level of aortic bifurcation, showing large lymph node anterior to left psaos muscle
References
1. Miedler, J.D., MacLennan, G.T. Primary Testicular Lymphoma. J Urol (2007) Dec;178(6):2645
2. Russell, R.C.G., Williams, N.S., Bulstrode, C.J.K. Chapter 79. Testis and Scrotum in Bailey & Love’s Short Practice of Surgery 24th Edition. (2004) Pp 1403-1417. Eds: Russell, R.C.G., Williams, N.S., Bulstrode, C.J.K. Arnold Publishers, UK
3. Vural, F., Cagirgan, S., Saydam, G., Hekimgil, M., Soyer, N.A., Tombuloglu, M. Primary testicular Lymphoma J Nat Med Assoc (2007). 99(11):1277-82,
4. Vaid, A.K., Bhatia, K., Doval, D.C., Talwar, V., Prakash, N., Sethi, T.K Primary Testicular Lymphoma: A Case Report. Haema (2006); 9(2): 286-289
5. Zouhair, A., Weber, D., Belkacemi, Y., Ketterer, N., Dietrich, P.Y., Villa, S., Scandolaro, L., Bieri, S., Studer, G., Delacretaz, F., Girardet, C., Mirimanoff, R.O., Ozsahin, M., Rare Cancer Network. Outcome and patterns of failure in testicular lymphoma: a multicenter Rare Cancer Network study. Int J Radiat Oncol Biol Phys. (2002) 52(3):652-6
6. Al-Abbadi, M.A., Hattab, E.M, Tarawneh, M.S., Amr, S.S., Orazi, A., Ulbright, T.M. Primary testicular diffuse large B-cell lymphoma belongs to the nongerminal center B-cell-like subgroup: a study of 18 cases Mod Path (2006) 19, 1521–1527
7. Sampat, M.B., Sirsat, M.V., Kamat, M.R. Malignant lymphoma of the testis in Indians. Br J Urol (1974); 46: 569-575
8. Shahab, N., Doll, D.C. Testicular lymphoma. Semin Oncol (1999); 26: 259-269
9. Salem, Y.H., Miller, H.C. Lymphoma of genitourinary tract. J Urol (1994); 151: 1162-1170
10. Moertel, C.L., Watterson, J., McCormick, S.R., Simonton, S.C. Follicular large cell lymphoma of the testis in a child. Cancer (1995); 75: 1182-1186
11. Ferry, J.A., Harris, N.L., Young, R.H., Coen, J., Zietman, A., Scully, R.E. Malignant lymphoma of the testis, epididymis, and spermatic cord. A clinicopathologic study of 69 cases with immunophenotypic analysis. Am J Surg Pathol (1994); 18: 376-390
12. Mazzu, D., Jeffry Jr, R.B., Ralls, P.W. Lymphoma and Leukemia the Testicles: Findings on Gray- and Color Doppler Sonography. AJR (1995) 164:645-7
13. Horstrnan, W.G., Melson, G.L., Middleton, W.D., Andnole, G.L. Testicular tumors: findings with color Doppler US. Radiology (1992) ;185:733-737
14. Zietman, A.L., Coen, J.J., Ferry, J.A., Scully, R.E., Kaufman, D.S., McGovern, F.G. The management and outcome of stage IAE non Hodgkin's lymphoma of the testis. J Urol (1996) 155: 943-946
15. Ono, K., Arimoto, H., Wada, K., Takahara, T., Shirotani, T., Shimizu, A., Sakai, Y., Matsukuma, S., Hatanaka, K., Inohara, T. Multicentric involvement of non-Hodgkin's lymphoma in the central nervous system and testis--case report Neurol Med Chir (Tokyo) (2004) 44:493-6
16. Moorjani,V., Mashankar, A., Goel, S., Khandelwal, K., Patange, V., Merchant, N. Sonographic Appearance of Primary Testicular Lymphoma AJR (1991). 157:1225-1226,
Date added to bjui.org: 25/10/2009 (publication information)
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