Final Diagnosis -- Pediatric Testicular Follicular Lymphoma

DIAGNOSIS

Pediatric testicular follicular lymphoma

B-CELL CLONALITY: positive
B-Cell Receptor PCR (BIOMED-2):
IgH (VDJ) FR1: Polyclonal
IgH (VDJ) FR2: Clonal (281bp)
IgH (VDJ) FR3: Polyclonal
IgK-VJ: Polyclonal
IgK-DE: Clonal (280bp, 286bp)

FISH FOR BCL-2 REARRANGEMENT: Negative.

DIFFERENTIAL DIAGNOSIS: follicular hyperplasia in the course of chronic orchitis.

DISCUSSION

Primary testicular lymphomas are rare in children with only few case reports of pre-B lymphoblastic lymphoma, Burkitt and Burkitt-like lymphoma, diffuse large B-cell lymphoma and follicular lymphoma[1]. The case reported here represents a rare example of pediatric testicular follicular lymphoma. This tumor shows biological features that are different from the classic adult follicular lymphomas, lacking the BCL2/IGH translocation. By immunohistochemistry, the germinal center cells have a B-cell phenotype (CD20 positive) with expression of CD10 and Bcl6 proteins, but are Bcl2 protein negative. Similar to our case, published cases report that molecular genetic studies reveal immunoglobulin heavy chain (IGH) gene rearrangements , with no rearrangement involving the BCL2 gene [2, 3, 4, 5].

The most important differential diagnosis is with follicular hyperplasia in the course of chronic orchitis. The diagnosis of pediatric testicular follicular lymphoma should always be considered if there is back to back follicular growth pattern and no clinical or histologic evidence of infectious diseases with lack of inflammation or granulomas in the epididymis and non-affected testis. EBV and other infections process should be used to exclude, and the detection of a monoclonal Ig gene rearrangement can be helpful [2, 5].

Pediatric testicular follicular lymphomas have been reported to be a localized disease. The clinical outcome is excellent with treatment, including complete surgical resection and short duration chemotherapy or by surgical excision alone [6].

REFERENCES

  1. Nistal M, Paniagua R, Gonzalez-Peramato P, Reyes-Mugica M. Testicular and paratesticular tumors in the pediatric age group. Pediatr Dev Pathol 2016;19:471-492.
  2. Liu Q, Salaverria I, Pittaluga S, Jegalian AG, Xi L, Siebert R, Raffeld M, Hewitt SM, Jaffe ES. Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma. Am J Surg Pathol. 2013 Mar;37(3):333-43.
  3. Lones MA, Raphael M, McCarthy K, Wotherspoon A, Terrier-Lacombe MJ, Ramsay AD, Maclennan K, Cairo MS, Gerrard M, Michon J, Patte C, Pinkerton R, Sender L, Auperin A, Sposto R, Weston C, Heerema NA, Sanger WG, von Allmen D, Perkins SL. Primary follicular lymphoma of the testis in children and adolescents. J Pediatr Hematol Oncol. 2012 Jan;34(1):68-71.
  4. Pileri SA, Sabattini E, Rosito P, Zinzani PL, Ascani S, Fraternali-Orcioni G, Gamberi B, Piccioli M, Vivenza D, Falini B, Gaidano G. Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics. J Clin Pathol. 2002 Sep;55(9):684-8.
  5. Finn LS, Viswanatha DS, Belasco JB, Snyder H, Huebner D, Sorbara L, Raffeld M, Jaffe ES, Salhany KE. Primary follicular lymphoma of the testis in childhood. Cancer. 1999 Apr 1;85(7):1626-35.
  6. Heller KN, Teruya-Feldstein J, La Quaglia MP, Wexler LH. Primary follicular lymphoma of the testis: excellent outcome following surgical resection without adjuvant chemotherapy. J Pediatr Hematol Oncol. 2004 Feb;26(2):104-7.

Contributed by Claudia Maria Salgado MD, PhD and Miguel Reyes-Múgica MD



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