Primary testicular lymphoma

Blood ◽  
2014 ◽  
Vol 123 (4) ◽  
pp. 486-493 ◽  
Author(s):  
Chan Y. Cheah ◽  
Andrew Wirth ◽  
John F. Seymour

Abstract Primary testicular lymphoma (PTL) is a rare, clinically aggressive form of extranodal lymphoma. The vast majority of cases are histologically diffuse large B-cell lymphoma, but rarer subtypes are clinically important and must be recognized. In this review, we discuss the incidence, clinical presentation, and prognostic factors of PTL and present a summary of the recent advances in our understanding of its pathophysiology, which may account for the characteristic clinical features. Although outcomes for patients with PTL have historically been poor, significant gains have been made with the successive addition of radiotherapy (RT), full-course anthracycline-based chemotherapy, rituximab and central nervous system–directed prophylaxis. We describe the larger retrospective series and prospective clinical trials and critically examine the role of RT. Although rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone given every 21 days with intrathecal methotrexate and locoregional RT is the current international standard of care, a substantial minority of patients progress, representing an unmet medical need. Finally, we discuss new treatment approaches and recent discoveries that may translate into improved outcomes for patients with PTL.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2697-2697
Author(s):  
Ali Mazloom ◽  
Nathan Fowler ◽  
Puneeth Iyengar ◽  
Bouthaina S. Dabaja

Abstract Abstract 2697 Poster Board II-673 Background: Primary testicular lymphoma (PTL) is the most common testicular neoplasm in males over the age of 60, representing 1% to 7% of all testicular malignancies. Histologically, the most common subtype of PTL is diffuse large B-cell lymphoma (DLBCL) representing 80–90% of diagnosed tumors. Primary testicular lymphoma has a tendency to disseminate to the contralateral testis and the central nervous system. Treatment approaches can include loco-regional treatment regimens (orchiectomy, radiotherapy) and systemic chemotherapy with or without intra-thecal chemoprophylaxis. The purpose of this study is to determine the clinical characteristics, patterns of failure, and survival of patients with PTL at our institution and to determine any correlation between outcome and treatment strategies. Methods: We retrospectively reviewed the medical records of patients with diffuse large B cell lymphoma of the testis. All patients were diagnosed and managed at the University of Texas MD Anderson Cancer Center between 1964 and 2008. Pathological diagnosis of DLBCL of the testis was made from orchiectomy specimens or fine needle aspiration of the testis. Factors analyzed included: age, stage at diagnosis, presence of b-symptoms, serum lactate dehydrogenase (LDH), beta-2 microglobulin, and modality of treatment. Cox proportional hazards model was used to generate hazard ratios for prognostic factors that affect the overall survival (OS) and disease free survival (DFS). Estimates of survival were calculated using the Kaplan-Meier method and the log-rank test was used to compare OS and DFS by the type of treatment. Results: Seventy-five patients with DLBCL of the testis were identified. The median age at diagnosis was 64 years (range 22 – 82). Ann Arbor stage I was present in 34 (45%), stage II in 13 (17%), stage III in 4 (5%), stage IV in 23 (31%), and unknown in 1 (1%). On univariate analysis, patients with advanced stage disease (stage III and IV; p = 0.042), elevated serum LDH levels (p = 0.029), B-symptoms at presentation (p = 0.003), and high-intermediate and high IPI score (p = 0.013), had a significantly decreased OS and DFS. The 5-years OS and DFS for all patients was 53.3%, and 45.9%, respectively. Treatment details are described in Table 1. A greater proportion of patients who received trimodality regimen (doxorubicin based chemotherapy, scrotal RT, and intrathecal methotrexate) had limited stage disease compared to those receiving less than a trimodality approach, however this variation was not statistically significant (71% vs 60%; p = 0.374, chi-square test). The 5-year OS and DFS for those treated with trimodality regimen was 90.4% and 79.5%, respectively, while for those treated with doxorubicin-based chemotherapy and testicular irradiation but without intrathecal chemotherapy it was 63.5%, and 55.6%, respectively, and for those treated with doxorubicin-based chemotherapy alone it was 39.3%, and 28.6% (p = 0.009 for OS and p = 0.012 for DFS). Figures 1 & 2 Conclusion: Prognostic factors for DLBCL of the testis include stage, LDH, B-symptoms, and IPI score. Patients with DLBCL of the testis who received trimodality regimen (doxorubicin based chemotherapy combined with scrotal RT and intrathecal chemotherapy) had significantly improved OS and DFS, which support the use of this approach as standard of care. Disclosures: No relevant conflicts of interest to declare.


Folia Medica ◽  
2020 ◽  
Vol 62 (1) ◽  
pp. 200-203
Author(s):  
Yavuz Guler ◽  
Burak Ucpinar ◽  
Akif Erbin

Introduction: Testicular cancers detected in older males are mostly testicular lymphomas. Primary testicular lymphoma (PTL) is a rare, clinically aggressive form of extranodal lymphoma. In population-based studies, the  incidence of PTL is 0.09-0.26/100.000. The vast majority of PTL are diffuse large B-cell lymphoma (DLBCL).   Case presentation: We present a case of PTL diagnosed in a 68-year-old male patient and also, we discuss the incidence, clinical presentation, prognostic factors and management of this rare pathology in the light of current literature. Histopathological and immunohistochemical exams of the patient confirmed the diagnosis of DLBCL after radical orchiectomy. According to the internal prognostic index (IPI), patients’ IPI score was evaluated as 5 and according to Ann Arbor staging, patients’ stage was interpreted as grade 3E. Cyclophosphamide, vincristine, etoposide, and prednisolone chemotherapy was planned for the patient and until now, the patient received his first chemotherapy regimen.   Discussion: Primary testicular lymphoma should be kept in mind for every patient who admits with a testicular mass, especially in advanced age.  Misinterpreatation of the clinical findings can delay the definitive diagnosis. Primary testicular lymphoma should be managed with a multi-disciplinary team including urologists, medical and radiation oncologists. 


Author(s):  
Eduardo Poblano Olivares ◽  
Jorge L. Olmos Gonzalez

Primary testicular lymphoma is a rare disease that has a higher incidence in patients over 60 years of age, presenting as an increase in volume in the inguinal region, which is usually painless and slow-growing. In the case that we present, it is a patient who was initially diagnosed with an indirect inguinal hernia due to the findings on examination and ultrasound, without presenting relevant findings in the laboratory studies, during the trans-operative we found testicular tumor compatible with diffuse large B-cell lymphoma, this being the most common variant of testicular lymphoma. This case emphasizes on importance of pre-operative suspicion in older age patients with increased volume in the groin region and without a clear diagnosis.


2010 ◽  
Vol 43 (1) ◽  
pp. 237-240 ◽  
Author(s):  
Suhail Al-Salam ◽  
Ahmad Shaaban ◽  
Maha Alketbi ◽  
Naveed U. Haq ◽  
Samra Abouchacra

Haematologica ◽  
2018 ◽  
Vol 104 (6) ◽  
pp. e256-e259 ◽  
Author(s):  
Luca Aresu ◽  
Serena Ferraresso ◽  
Laura Marconato ◽  
Luciano Cascione ◽  
Sara Napoli ◽  
...  

2010 ◽  
Vol 6 (1) ◽  
pp. 47-57 ◽  
Author(s):  
Celso Arrais Rodrigues ◽  
Poliana Alves Patah ◽  
Yana A. S. Novis ◽  
Chitra Hosing ◽  
Marcos de Lima

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