Primary Testicular Lymphoma

  • Keisuke SasaiEmail author


Primary testicular lymphoma (PTL) usually occurs as a diffuse large B-cell lymphoma in elderly males. The first diagnostic and therapeutic procedure in a patient with PTL is inguinal orchiectomy. The guidelines of the International Lymphoma Radiation Oncology Group recommend rituximab combined with CHOP or more aggressive regimens as the first-line systemic treatment, with prophylactic intrathecal or intravenous methotrexate to avoid disease relapse in the CNS, the most common site of tumor recurrence. Because the testis is a sanctuary, the tumor in this organ is not responsive to systemic chemotherapy. The contralateral testis should be irradiated to prevent recurrence.


Blood–testicular barrier Prophylaxis of CNS relapse R-CHOP Radiation therapy of the contralateral testis 


  1. 1.
    Brouwer CL, Wiesendanger EM, van der Hulst PC, van Imhoff GW, Langendijk JA, Beijert M. Scrotal irradiation in primary testicular lymphoma: review of the literature and in silico planning comparative study. Int J Radiat Oncol Biol Phys. 2013;85:298–308.CrossRefPubMedGoogle Scholar
  2. 2.
    Koukourakis G, Kouloulias V. Lymphoma of the testis as primary location: tumour review. Clin Transl Oncol. 2010;12:321–5.CrossRefPubMedGoogle Scholar
  3. 3.
    Olszewskia AJ, Winerb ES, Castilloca JJ. Improved survival with rituximab-based chemoimmunotherapy in older patients with extranodal diffuse large B-cell lymphoma. Leuk Res. 2014;38:866–73.CrossRefGoogle Scholar
  4. 4.
    Vidyavathi K, Prabhakar K, Harendra KM. Primary testicular lymphoma with rupture: an unusual presentation. J Nat Sci Biol Med. 2013;4:232–5.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Deng L, Xu-Monette ZY, Loghavi SN, Manyam GC, Xia Y, et al. Primary testicular diffuse large B-cell lymphoma displays distinct clinical and biological features for treatment failure in rituximab era: a report from the International PTL Consortium. Leukemia. 2015; doi: 10.1038/leu.2015.237.Google Scholar
  6. 6.
    Cheah CY, Wirth A, Seymour JF. Primary testicular lymphoma. Blood. 2014;123:486–93.CrossRefPubMedGoogle Scholar
  7. 7.
    Gundrum JD, Mathiason MA, Moore DB, Go RS. Primary testicular diffuse large B-cell lymphoma: a population-based study on the incidence, natural history, and survival comparison with primary nodal counterpart before and after the introduction of rituximab. J Clin Oncol. 2009;27:5227–32.CrossRefPubMedGoogle Scholar
  8. 8.
    Ahmad SS, Idris SF, Follows GA, Williams MV. Primary testicular lymphoma. Clin Oncol. 2012;24:358–65.CrossRefGoogle Scholar
  9. 9.
    Riemens A, Missotten T, Baarsma S, Vidovic-Valentincic N, Novac-Andrejcic K, et al. A diffuse large B-cell lymphoma in immunoprivileged sites: association of vitreoretinal, testicular and central nervous system lymphoma. Acta Ophthalmol. 2014;92:e159–60. doi: 10.1111/aos.12236.CrossRefPubMedGoogle Scholar
  10. 10.
    Yahalom J, Illidge T, Specht L, Hoppe RT, Li YX, Tsang R, Wirth A, International Lymphoma Radiation Oncology Group. Modern radiation therapy for extranodal lymphomas: field and dose guidelines from the International Lymphoma Radiation Oncology Group. Int J Radiat Oncol Biol Phys. 2015;92:11–31.CrossRefPubMedGoogle Scholar
  11. 11.
    Al-Abbadi MA, Hattab EM, Tarawneh MS, Amr SS, Orazi A, Ulbright TM. Primary testicular diffuse large B-cell lymphoma belongs to the nongerminal center B-cell-like subgroup: a study of 18 cases. Mod Pathol. 2006;19:1521–7.CrossRefPubMedGoogle Scholar
  12. 12.
    Wright DH. Pathology of extra-nodal non Hodgkin lymphomas. Clin Oncol. 2012;24:319–28.CrossRefGoogle Scholar
  13. 13.
    Magnoli F, Ricotti I, Novario M, Mazzucchelli L, Dainese E, Ambrosiani L, Sessa F, Uccella S. Primary testicular diffuse large B-cell lymphoma: morphological and immunophenotypical study with characterization of the T-cell component of the tumor microenvironment. Leuk Lymphoma. 2015; doi: 10.3109/10428194.2015.1055488.PubMedGoogle Scholar
  14. 14.
    Bacon CM, Ye H, Diss TC, McNamara C, Kueck B, Hasserjian RP, Rohatiner AZ, Ferry J, Du MQ, Dogan A. Primary follicular lymphoma of the testis and epididymis in adults. Am J Surg Pathol. 2007;31:1050–8.CrossRefPubMedGoogle Scholar
  15. 15.
    Seymour JF. X. Extra-nodal lymphoma in rare localisations: bone, breast and testes. Hematol Oncol. 2013;31(Suppl. 1):60–3.CrossRefPubMedGoogle Scholar
  16. 16.
    Williams PL, Warwick R, et al. Testis. In: Williams PL, Warwick R, editors. Gray’s anatomy. 36th ed. Edinburgh: Churchill Livingstone; 1980. p. 1410–9.Google Scholar
  17. 17.
    Sobin LH, Gospodarowicz MK, Wittekind C, editors. UICC TNM classification of malignant tumours. 7th ed. Hoboken, NJ: Wiley-Blackwell; 2009. p. 249–54.Google Scholar
  18. 18.
    Bart J, Groen HJ, van der Graaf WT, Hollema H, Hendrikse NH, Vaalburg W, Sleijfer DT, de Vries EG. An oncological view on the blood-testis barrier. Lancet Oncol. 2002;3:357–63.CrossRefPubMedGoogle Scholar
  19. 19.
    Cronin CG, Swords R, Truong MT, Viswanathan C, Rohren E, Giles FJ, O'Dwyer M, Bruzzi JF. Clinical utility of PET/CT in lymphoma. Am J Roentgenol. 2010;194:W91–W103.CrossRefGoogle Scholar
  20. 20.
    Mazloom A, Fowler N, Medeiros LJ, Iyengar P, Horace P, Dabaja BS. Outcome of patients with diffuse large B-cell lymphoma of the testis by era of treatment: the M.D. Anderson Cancer Center experience. Leuk Lymphoma. 2010;51:1217–24.CrossRefPubMedGoogle Scholar
  21. 21.
    Kim J, Yoon DH, Park I, Kim S, Park JS, Lee SW, Huh J, Park CS, Suh C. Treatment of primary testicular diffuse large B cell lymphoma without prophylactic intrathecal chemotherapy: a single center experience. Blood Res. 2014;49:170–6.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Vitolo U, Chiappella A, Ferreri AJM, Martelli M, Baldi I, Balzarotti M, Bottelli C, Conconi A, Gomez H, Lopez-Guillermo A, Martinelli G, Merli F, Novero D, Orsucci L, Pavone V, Ricardi U, Storti S, Gospodarowicz MK, Cavalli F, Sarris AH, Zucca E. First-line treatment for primary testicular diffuse large B-cell lymphoma with rituximab-CHOP, CNS prophylaxis, and contralateral testis irradiation: final results of an international phase ii trial. J Clin Oncol. 2011;29:2766–72.CrossRefPubMedGoogle Scholar
  23. 23.
    Guirguis HR, Cheung MC, Mahrous M, Piliotis E, Berinstein N, Imrie KR, Zhang L, Buckstein R. Impact of central nervous system (CNS) prophylaxis on the incidence and risk factors for CNS relapse in patients with diffuse large B-cell lymphoma treated in the rituximab era: a single centre experience and review of the literature. Br J Haematol. 2012;159:39–49.CrossRefPubMedGoogle Scholar
  24. 24.
    McMillan A, Ardeshna KM, Cwynarski K, Lyttelton M, McKay P, Montoto S. Guideline on the prevention of secondary central nervous system lymphoma: British Committee for Standards in Haematology. Brit J Haematol. 2013;163:168–81.Google Scholar
  25. 25.
    Zucca E, Conconi A, Mughal TI, Sarris AH, Seymour JF, Vitolo U, Klasa R, Ozsahin M, Mead GM, Gianni MA, Cortelazzo S, Ferreri AJM, Ambrosetti A, Martelli M, Thie’blemont C, Moreno HG, Pinotti G, Martinelli G, Mozzana R, Grisanti S, Provencio M, Balzarotti M, Laveder F, Oltean G, Callea V, Roy P, Cavalli F, Gospodarowicz MK. Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group. J Clin Oncol. 2003;21:20–7.CrossRefPubMedGoogle Scholar
  26. 26.
    Wang Y, Li ZM, Huang JJ, Xia Y, Li H, Li YJ, Zhu YJ, Zhao W, Xia XY, Wei WX, Huang HQ, Lin TY, Jiang WQ. Three prognostic factors influence clinical outcomes of primary testicular lymphoma. Tumour Biol. 2013;34:55–63.CrossRefPubMedGoogle Scholar
  27. 27.
    Ichikawa K, Noguchi M, Koike M, Aritaka N, Sekiguchi Y, Sunami Y, Tsutsui M, Hosone M, Hirano T, Gotoh A, Komatsu N. Rituximab plus a CHOP-like regimen, central nervous system prophylaxis, and contralateral testicular irradiation for localized primary testicular diffuse large B-cell lymphoma lead to prolonged progression-free survival. Int J Hematol. 2014;100:370–8.CrossRefPubMedGoogle Scholar
  28. 28.
    Mihaljevic B, Vukovic V, Smiljanic M, Milic N, Todorovic M, Bila J, Andjelic B, Djurasinovic V, Jelicic J, Antic D. Single-center experience in the treatment of primary testicular lymphoma. Oncol Res Treat. 2014;37:239–42.CrossRefPubMedGoogle Scholar
  29. 29.
    Sasai K, Yamabe H, Tsutsui K, Dodo Y, Ishigaki T, Shibamoto Y, Hiraoka M. Primary testicular non-Hodgkin’s lymphoma: a clinical study and review of the literature. Am J Clin Oncol. 1997;20:59–62.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Japan 2017

Authors and Affiliations

  1. 1.Department of Radiation OncologyJuntendo University, Graduate School of MedicineTokyoJapan

Personalised recommendations