Testicular involvement in pediatric lymphoid tumors: a review of the literature and a series of clinical observations

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Abstract

Lymphoid tumors with testicular involvement in childhood are rare and heterogeneous. The disease may manifest with uni- or bilateral scrotal enlargement. Comprehensive examination includes evaluation of all lymph nodes involvement, as well as ultrasound examination, magnetic resonance imaging and positron emission tomography. A diagnosis is made on basis of morphological and immunohistochemical verification. Determination of lymphoid tumor variant and stage, is recommended to perform chemotherapy according to prognostic risk group, and, in some cases, transplantation of hematopoietic stem cells is required as consolidation therapy.

We present three rare clinical cases of follicular lymphoma with testicular involvement, T-lymphoblastiс progenitor cell lymphoma, and B-lineage acute lymphoblastic leukemia (ALL) relapse. Different schemes of chemotherapy, combined with orchiectomy (in 2 of 3 cases) resulted in prolonged complete remission. In the first case, due to treatment-refractory B-lineage ALL, the disease was incurable.

Our data on clinical, morphological, immunohistochemical and therapeutic features of lymphoid tumors with testicular involvement make it necessary to form multidisciplinary teams, including pediatric urologists, hematologic oncologists and surgeons for timely diagnosis and successful treatment.

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About the authors

M. S. Korneeva

N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia

Author for correspondence.
Email: margokorneeva@mail.ru
ORCID iD: 0000-0001-9805-9972

Hematologist at the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 2 of the Research Institute of Pediatric Oncology and Hematology at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

Russian Federation, Moscow

N. A. Batmanova

N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia

Email: batmanova_nataly@mail.ru
ORCID iD: 0000-0002-3005-2085

Ph.D., Head of the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 2 of the Research Institute of Pediatric Oncology and Hematology at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

Russian Federation, Moscow

T. R. Panferova

N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia

Email: tizmailova@gmail.com
ORCID iD: 0000-0003-2240-069X

Ph.D., Senior Researcher of the Department of Radiology of the Department of Radiation Methods for the Diagnosis and Treatment of Tumors of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

Russian Federation, Moscow

T. T. Valiev

N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia

Email: timurvaliev@mail.ru
ORCID iD: 0000-0002-1469-2365

Ph.D., MD, Head of the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 1 of the Research Institute of Pediatric Oncology and Hematology at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia

Russian Federation, Moscow

References

  1. Kevin N. Heller, Julie Teruya-Feldstein, Michael P. La Quaglia, Leonard H. Wexler. Primary follicular lymphoma of the testis: excellent outcome following surgical resection without adjuvant chemotherapy. J Pediatr Hematol Oncol. 2004 Feb;26(2):104–107. doi: 10.1097/00043426-200402000-00007.
  2. Manuel Nistal, Ricardo Paniagua, Pilar González-Peramato, Miguel Reyes-Múgica. Perspectives in Pediatric Pathology, Chapter 25. Testicular and Paratesticular Tumors in the Pediatric Age Group. Pediatr Dev Pathol. 2016 Nov/Dec;19(6):471–492. doi: 10.2350/16-09-1829-PER.1.
  3. Umberto Vitolo 1., Andrés J.M. Ferreri, Emanuele Zucca, et al. Primary testicular lymphoma. Critical Reviews in Oncology/Hematology. 2008 Feb;65(2):183–189. doi: 10.1016/j.critrevonc.2007.08.005.
  4. Jacob D. Gundrum, Michelle A. Mathiason, Derek B. Moore, and Ronald S. Go. 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 Nov 1;27(31):5227–5232. doi: 10.1200/JCO.2009.22.5896.
  5. Menter T., Ernst M., Drachneris J., et al. Phenotype profiling of primary testicular diffuse large B-cell lymphomas. Hematol Oncol. 2014 Jun;32(2):72–81. doi: 10.1002/hon.2090.
  6. Epstein A.S., Hedvat C., Habib F., Hamlin P. Testis-isolated mantle cell lymphoma: a unique case. Clin Lymphoma Myeloma Leuk. 2011 Oct;11(5):439–441. doi: 10.1016/j.clml.2011.06.013.
  7. Licci S., Morelli L., Covello R. Primary mantle cell lymphoma of the testis. Ann Hematol.2011 Apr;90(4):483–484. doi: 10.1007/s00277-010- 1049-3.
  8. Kemmerling R., Stintzing S., Mühlmann J., Dietze O., Neureiter D. Primary testicular lymphoma: a strictly homogeneous hematological disease? Oncol Rep. 2010 May;23(5):1261–1267. doi: 10.3892/or_00000759.
  9. Liang D.N., Yang Z.R., Wang W.Y., et al. Extranodal nasal type natural killer/T-cell lymphoma of testis: report of seven cases with review of literature. Leuk Lymphoma. 2012 Jun;53(6):1117–1123. doi: 10.3109/10428194.2011.645209.
  10. Pileri S.A., Sabattini E., Rosito P. et al. Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics. J Clin Pathol. 2002 Sep;55(9):684–688. doi: 10.1136/jcp.55.9.684.
  11. Yavuz Köksal, Bilgehan Yalçin, Aysegül Uner, Canan Akyüz, Unsal Han, Münevver Büyükpamukçu. Primary testicular Burkitt lymphoma in a child. Pediatr Hematol Oncol. 2005 Dec;22(8):705–709. doi: 10.1080/08880010500278822.
  12. Yongren Wang, Jian Li, Yongjun Fang. Primary testicular T-lymphoblastic lymphoma in a child: A case report. Medicine (Baltimore). 2020 Jun 26;99(26):e20861. doi: 10.1097/MD.0000000000020861.
  13. Hoa Thi Kim Nguyen, Michael A Terao, Daniel M Green , Ching-Hon Pui, Hiroto Inaba. Testicular involvement of acute lymphoblastic leukemia in children and adolescents: Diagnosis, biology, and management. Cancer. 2021 Sep 1;127(17):3067–3081. d=doi: 10.1002/cncr.33609.
  14. Srisuwan T., Muttarak M., Kitirattrakarn P., Ya-in C. Clinics in diagnostic imaging (134). Testicular lymphoma., Singapore Med J, 2011 Mar;52(3):204–208.
  15. Tsili A.C., Argyropoulou M.I., Giannakis D., Sofikitis N., Tsampoulas K. Primary diffuse large B-cell testicular lymphoma: magnetic resonance imaging findings., Andrologia. 2012 May;44 Suppl 1:845–847. doi: 10.1111/j.1439-0272.2011.01236.x.
  16. Ponti G, Ponzoni M, Ferreri AJ, Foppoli M, Mazzucchelli L, Zucca E. The impact of histopathologic diagnosis on the proper management of testis neoplasms., Nat Clin Pract Oncol. 2008 Oct;5(10):619–622. doi: 10.1038/ncponc1218.
  17. Chan Y. Cheah, Andrew Wirth, John F. Seymour. Primary testicular lymphoma. Blood. 2014 Jan 23;123(4):486–493. doi: 10.1182/blood-2013-10-530659.
  18. Umberto Vitolo, Annalisa Chiappella, Andrés J.M. Ferreri, et al. 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 Jul 10;29(20):2766–2772. doi: 10.1200/JCO.2010.31.4187.
  19. Kourosh Pakzad, Gregory T. MacLennan, et al. Follicular large cell lymphoma localized to the testis in children. J Urol. 2002 Jul;168(1):225–228.
  20. Felice Francavilla, Riccardo Santucci, Arcangelo Barbonetti, Sandro Francavilla. Naturally-occurring antisperm antibodies in men: interference with fertility and clinical implications. An update. Front Biosci. 2007 May;12:2890–28911. doi: 10.2741/2280.
  21. Hijiya N., Liu W., Sandlund J.T., et al. Overt testicular disease at diagnosis of childhood acute lymphoblastic leukemia: lack of therapeutic role of local irradiation. Leukemia. 2005 Aug;19(8):1399–1403. doi: 10.1038/sj.leu.2403843.
  22. Sirvent N., Suciu S., Bertrand Y., Uyttebroeck A., Lescoeur B., Otten J. Overt testicular disease (OTD) at diagnosis is not associated with a poor prognosis in childhood acute lymphoblastic leukemia: results of the EORTC CLG Study 58881. Pediatr Blood Cancer. 2007 Sep;49(3):344–348. doi: 10.1002/pbc.20716.
  23. Ching-Hon Pui, William E Evans. A 50-year journey to cure childhood acute lymphoblastic leukemia. Semin Hematol. 2013 Jul;50(3):185–196. doi: 10.1053/j.seminhematol.2013.06.007.
  24. David R. Freyer, Meenakshi Devidas, Mei La, William L. Carroll, Paul S. Gaynon, Stephen P. Hunger, Nita L. Seibel. Postrelapse survival in childhood acute lymphoblastic leukemia is independent of initial treatment intensity: a report from the Children’s Oncology Group. Blood. 2011 Mar 17;117(11):3010–3010. doi: 10.1182/blood-2010-07-294678.
  25. Eric C. Larsen, Meenakshi Devidas, Si Chen, Wanda L. Salzer, Elizabeth A. Raetz, Mignon L. Loh et all. Dexamethasone and High-Dose Methotrexate Improve Outcome for Children and Young Adults With High-Risk B-Acute Lymphoblastic Leukemia: A Report From Children’s Oncology Group Study AALL0232. J Clin Oncol 2016 Jul 10;34(20):2380–2838. doi: 10.1200/JCO.2015.62.4544.
  26. Yousif Matloub, Bruce C. Bostrom, Stephen P. Hunger, Linda C. Stork, Anne Angiolillo, Harland Sather, et al. Escalating intravenous methotrexate improves event-free survival in children with standard-risk acute lymphoblastic leukemia: a report from the Children’s Oncology Group. Blood. 2011 Jul 14;118(2):243–251. doi: 10.1182/blood-2010-12-322909.
  27. K Nguyen 1, M Devidas, S-C Cheng, et al. Children’s Oncology Group. Factors influencing survival after relapse from acute lymphoblastic leukemia: a Children’s Oncology Group study. Leukemia. 2008 Dec;22(12):2142–2150. doi: 10.1038/leu.2008.251.
  28. Lisieux Eyer de Jesus, Samuel Dekermacher, Glaucia Campos Resende, Renata Rangel Justiniano. Testicular involvement in pediatric acute lymphocytic leukemia: what to do about it? Int Braz J Urol. 2022 Nov-Dec;48(6):981–987. doi: 10.1590/S1677-5538.IBJU.2022.0318.
  29. Parker C, Waters R, Leighton C, et al. Effect of mitoxantrone on outcome of children with first relapse of acute lymphoblastic leukaemia (ALL R3): an open-label randomised trial. Lancet. 2010 Dec 11;376(9757):2009–2017. doi: 10.1016/S0140-6736(10)62002-8. Epub 2010 Dec 3.
  30. Locatelli F, Schrappe M, Bernardo ME, Rutella S. How I treat relapsed childhood acute lymphoblastic leukemia. Blood. 2012 Oct 4;120(14):2807–2816. doi: 10.1182/blood-2012-02-265884.
  31. Hunger SP, Raetz EA. How I treat relapsed acute lymphoblastic leukemia in the pediatric population. Blood. 2020 Oct 15;136(16):1803–1812. doi: 10.1182/blood.2019004043.
  32. Barredo JC, Hastings C, Lu X, et al. Isolated late testicular relapse of B-cell acute lymphoblastic leukemia treated with intensive systemic chemotherapy and response-based testicular radiation: a Children’s Oncology Group study. Pediatr Blood Cancer. 2018 May;65(5):e26928. doi: 10.1002/pbc.26928.
  33. Castillo LA, Craft AW, Kernahan J, Evans RG, Aynsley-Green A. Gonadal function after 12-Gy testicular irradiation in childhood acute lymphoblastic leukaemia. Med Pediatr Oncol. 1990;18(3):185–189. doi: 10.1002/mpo.2950180304.
  34. Wasilewski-Masker K., Seidel K.D., Leisenring W., et al. Male infertility in long-term survivors of pediatric cancer: a report from the Childhood Cancer Survivor Study. J Cancer Surviv. 2014 Sep;8(3):437–447. doi: 10.1007/s11764-014-0354-6.

Supplementary files

Supplementary Files
Action
1. JATS XML
2. Fig. 1. Appearance of the affected scrotum in relapsed B-lineage OLL

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3. Fig. 2. Ultrasound. Longitudinal scan of the right testis. Increased size, inhomogeneous decrease in echogenicity

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4. Fig. 3. Ultrasound. Thickening, unclear contours of the body of the right testicular appendage (marked with cursor)

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5. Fig 4. Longitudinal ultrasound scan of the right testis with CDC. Significant enhancement of the vascular pattern

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6. Fig 5. Transverse ultrasound scan of the right testis with CCC. Enhanced vascular pattern

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