Testicular Lymphoma

1
Testicular Lymphoma

• Nirav Dhruva
• Morning Report
• January 10, 2006

2
Background

• NHL was responsible for 19,200 deaths in US last
  year and 56,390 cases were diagnosed in 2005
• NHL of the testicle is a rare extranodal
  presentation of NHL as it represents only about
  1 of all lymphomas
• It accounts for 5 of all testicular neoplasms

3
Background

• Most are of intermediate or high grade according
  to Working Formulation or Diffuse Large B-cell
  lymphoma in the REAL classification. 52/64 cases
  reported by Ferry et al. were diffuse large cell
  lymphoma. Some estimate 80-90.
• Usually occurs in age gt 60, most frequent
  testicular neoplasm in this age group

4
Presentation

• Can occur in only the affected testicle, but
  extranodal presentation is very common.
• Common extranodal sites are the contralateral
  testicle, CNS, skin, and Waldeyer ring

5
Disease Course

• Testicular lymphomas have a high rate of
  recurrence and act aggressively. The ability and
  propensity to disseminate to extranodal areas
  makes testicular lymphoma unique from its nodal
  cousins. ? Of expression of CD44/other adhesion
  molecules to facilitate dissemination.

6
Disease Course

• Mayo Retrospective Study 62 patients with
  testicular lymphoma. Median age 62. 88
  patients had no residual disease after primary
  tx, 80 had recurrence. Treatment did not affect
  recurrence rate. Median f/u of 2.7 years 60
  died. 13 patients had CNS recurrence (11
  parenchymal) 8 patients had recurrence at CL
  testicle. 79 had localized or regional disease
  at presentation. Systemic symptoms 5 at
  presentation. Patients who had a CR, DFS could
  not be predicted based on stage, age, bm, or type
  of tx

7
Outcome and Prognostic Factors in Primary Large
Cell Lymphoma of Testes (International Extranodal
Lymphoma Study Group)

• Retrospective survey. 373 patients. Median age
  66yrs. Most present with local dx.
  Anthracycline chemo 68, prophylactic intrathecal
  chemo 18. Median survival 4.8 yrs. Favorable
  IPI, no B sx, use of anthracycline, and prophyl
  scrotal xrt assoc with longer survival at
  multivariate analysis. Failures usually occur
  within 1-3 years. In this study, even localized
  disease benefited from anthracycline chemo
  (longer PFS and OS). More CNS failures in
  parenchyma.

8
Treatment

• Orchiectomy is the initial primary therapeutic
  and diagnostic intervention.
• Systemic Chemotherapy in the past, the standard
  of tx was xrt for local and regional spread and
  chemo for distant metastasis
• However, given the poor prognosis of even stage
  I/II disease, combined modality including
  chemotherapy has become more frequent as initial
  therapy. Several studies indicate anthracycline
  containing regimen is favorable smaller ones
  exist showing no effect.

9
Treatment

• Prophylactic Irradiation of Contralateral
  Testicle - people feel it is a reservoir for
  extranodal disease because of the blood/testes
  barrier.
• In the Mayo study, no one who underwent
  contralateral xrt had recurrence at that site
  initially, but one case at 120 months after.
• Low complication risk reasonable to consider,
  especially in elder men.

10
Intrathecal Chemotherapy

• Tendency to relapse in CNS (? Blood / brain
  barrier)
• Many argue for CNS prophylaxis with intrathecal
  agents
• Used for patients with meningeal involvement
• Mayo study had high rate of parenchymal
  recurrence ???

11
NK/T cell

• NK cells are CD 56 cytotoxic/cytolytic cells
  usually expressing one or more of NK assoc
  antigens (CD 11b, CD 16, CD 56, and CD 57)
• Cells usually are CD2, CD56, surface CD 3- and
  cyctoplasmic CD3. Most express cytotoxic
  granule proteins, such as granzyme-B and TIA-1.
  Lack surface TCR. Share features with T cell
  (CD2, CD 7, CD3e, CD43).
• Usually can distinguish NK cell from T cell by
  lack of surface CD3 and lack of TCR gene
  arrangements

12
NK/Tcell

• Usually in upper aerodigestive tract possible
  act as first line of defense
• Very rare type approximately literature
  affecting testicle 9-10 cases reported (at the
  most)

13
NK/Tcell

• Appears to be more common in Asian, Mexican, or
  South American descent
• Strongly associated with EBV virus
• Usually extranodal presentation with angiocentric
  growth and large granular lymphocyte morphology
• Genetics loss of heterozygosity at 6q occurs at
  high frequency

14
NK/Tcell

• B symptoms are usually uncommon
• Poor prognosis 4 patients described in
  literature with testicular involvement died
  within 6 months of diagnosis

15
NK/Tcell

• Neoplastic cells surrounding and infiltrating
  vessel wall

16
Conclusion

• Testicular lymphoma has a low incidence rate with
  a high rate of recurrence.
• More information is needed in order to manage
  these patients but the low incidence rate makes
  prospective trials quite difficult

17
References

• Sasai K, Yamabe H et al. Primary Testicular
  Non-Hodgkins Lymphoma A Clinical Study and
  Review of the Literature. Am J Clin Oncol,
  Volume 20 (1). February 1997. 59-62
• Ballereau Charles, Leroy X, et al. Testicular
  natural killer T-cell lymphoma. International
  Journal of Urology (2005). 12, 223-224.
• Up to Date. Clinical and pathological features
  of mature peripheral T and NK cell lymphomas.
• Totonchi K, Engel G, et al. Testicular Natural
  Killer/T Cell Lymphoma, Nasal Type, of True
  Natural Killer-Cell Origin. Arch Pathol Lab Med.
  Vol 126. December 2002.
• Fonseca R, Habermann T, et al. Testicular
  Lymphoma is Associated with a High Incidence of
  Extranodal Recurrance. Cancer. Vol 88 1. Jan
  2000.
• Zucca E, Conconi A, et al. Patterns of Outcome
  and Prognostic Factors in Primary Large-Cell
  Lymphoma of the Testis in a Survey by the
  International Extranodal Lymphoma Study Group.
  Journal of Clinical Oncology Vol 21, January
  2003. P 20-27.
• Hasselblom S, Ridell B, et al. Testicular
  Lymphoma. Acta Oncologica. Vol 43. P 758-765,
  2004.