Peptide Receptor Radionuclide Therapy in Patients with Neurofibromatosis Type 2 – Initial Experience

Ziel/Aim Neurofibromatosis type 2 (NF2) is a genetic disorder that is associated with multiple tumors of the nervous system and approximately one-half of patients present with meningiomas. For patients with multifocal disease, somatostatin receptor (SSTR)-targeted peptide receptor radionuclide therapy (PRRT) might be a suitable systemic treatment option.

Methodik/Methods Between March 2015 and August 2017, eleven NF2 patients (7 females and 4 males; mean age, 39 ± 12 years) with multifocal, progressive meningiomas underwent a median of 4 cycles of PRRT (range, 2 – 6 cycles). Acute and chronic adverse events were recorded according to NIH Common Toxicity Criteria (CTC) version 5.0. Follow-up magnetic resonance imaging (every 3 to 6 months) using the Response Assessment in Neuro-Oncology response criteria for meningiomas were used to assess treatment responses.

Ergebnisse/Results PRRT was well tolerated in all patients without any relevant acute adverse effects. Chronic adverse events in the form of hematologic toxicity (CTC grade 3) were observed in 2 subjects. SSTR-directed radiopeptide therapy resulted in disease stabilization in 5 out of 11 patients.

Schlussfolgerungen/Conclusions PRRT is feasible and well-tolerated in NF2 patients. It might be a suitable treatment option in subjects with multifocal disease.