Treatment of Recurrent Congenital Mesoblastic Nephroma

Patients with most forms of recurrent childhood cancer should consider enrolling in phase I and phase II clinical trials. Other treatment options for recurrent congenital mesoblastic nephroma are discussed below.

Palliative care remains a central focus of management regardless of whether disease-directed therapy is pursued at the time of progression. This ensures that quality of life is maximized while attempting to reduce symptoms and stress related to the terminal illness.

Relapses were reported in 4% of patients with congenital mesoblastic nephroma, and all relapses occurred within 12 months after diagnosis. Most relapses occurred locally, although metastatic relapses have been reported.[4] About 70% of patients who relapsed survived with individualized treatment comprising combinations of surgery, chemotherapy, and radiation therapy.[4]

Targeted therapy should be considered for patients with recurrent or refractory disease containing the ETV6::NTRK3 fusion. Larotrectinib and entrectinib are NTRK inhibitors that are approved for adult and pediatric patients with solid tumors that have an NTRK gene fusion without a known acquired resistance variant, who are either metastatic or when surgical resection is likely to result in severe morbidity, and who have no satisfactory alternative treatments or whose cancer has progressed after treatment.[15,16]