Table 3.
Recommended management of testicular involvement of acute lymphoblastic leukemia
| Evaluation at diagnosis and relapse | ||
|---|---|---|
| Physical examination of testes | ||
| Ultrasound examination of suspected cases | ||
| Biopsy to confirm testicular disease for patients with isolated testicular involvement at diagnosis or relapse or for those with equivocal testicular involvement at the time of concurrent bone marrow or central nervous system relapse | ||
| Treatment | ||
| Patients with newly diagnosed ALL | ||
| Induction chemotherapy | ||
| Biopsy for patients with persistent enlargement or abnormal ultrasound imaging after induction therapy (if involvement is unequivocal, biopsy may not be necessary) | ||
| Testicular irradiation (24 Gy) for persistent leukemia involvement | ||
| Isolated testicular relapse | ||
| –Early relapse (<18 months after diagnosis) in B-ALL and relapse at any time in T-ALL | ||
| Intensive chemotherapy* with hematopoietic cell transplantation | ||
| Testicular boost (12 Gy) with total body irradiation (12 Gy)** | ||
| Chimeric antigen receptor T cells for patients with B-ALL# | ||
| –Intermediate relapse (18–36 months after diagnosis) and late relapse (≥36 months after diagnosis) in B-ALL | ||
| Systemic chemotherapy*†‡ | ||
| Bilateral: testicular irradiation (24 Gy) or orchiectomy | ||
| Unilateral: orchiectomy of the affected testis and prophylactic irradiation (15 Gy) of the biopsy-negative contralateral testis OR testicular irradiation (24 Gy) | ||
| Chimeric antigen receptor T cells for patients with B-ALL# | ||
| Combined testicular and bone marrow relapse | ||
| –Early relapse (<18 months after diagnosis) and intermediate relapse (18–36 months after diagnosis) in B-ALL and relapse at any time in T-ALL | ||
| Intensive chemotherapy* with hematopoietic cell transplantation | ||
| Testicular boost (12 Gy) with total body irradiation (12 Gy)** | ||
| Chimeric antigen receptor T cells for patients with B-ALL# | ||
| –Late relapse (≥36 months after diagnosis) in B-ALL | ||
| Systemic chemotherapy*†‡ | ||
| Bilateral: testicular irradiation (24 Gy) or orchiectomy | ||
| Unilateral: orchiectomy of the affected testis and prophylactic irradiation (15 Gy) of the biopsy-negative contralateral testis OR testicular irradiation (24 Gy) | ||
| Chimeric antigen receptor T cells for patients with B-ALL# | ||
Immunotherapy (e.g., blinatumomab) can be used in B-ALL although the efficacy of blinatumomab for treatment of testicular relapse is not established.
For patients who do not receive total body irradiation, testicular radiation (24 Gy) or orchiectomy can be considered.
The number of patients with testicular relapse who have been treated with chimeric antigen receptor T cells is limited.
When intensified chemotherapy including high-dose methotrexate is given, some treatment regimens omit testicular irradiation or orchiectomy if no residual disease is observed after the induction regimen.
Patients with MRD of ≥0.1% at the end of induction therapy should be treated in the same way as those with early relapse. Even in cases of isolated testicular relapse, MRD in the bone marrow can be positive at relapse.
The radiation dose, schedule, and timing may vary between treatment regimens.
Abbreviation: ALL, acute lymphoblastic leukemia; MRD, minimal residual disease