Treatment of progressive metastatic pheochromocytomas and paragangliomas with ¹⁷⁷Lu-DOTATATE PRRT

Ziel/Aim Treatment of metastatic pheochromocytomas and paragangliomas (MPPG) remains challenging. In case of a local recurrence or localized metastses, surgery or radiotherapy represent treatment options. Systemic chemotherapy and radionuclide therapy using ¹³¹I-mIBG are of limited efficacy. Peptide receptor radionuclide therapy (PRRT) using somatostatin analogues was proposed as an alternative therapeutic approach.

Methodik/Methods We report preliminary data of the prospective study aimed to assess the efficacy of 4 cycles of PRRT using ¹⁷⁷Lu-DOTATATE. 7 patients with histologically confirmed PPGLs and metastatic progression after surgery are included. One of them had germline mutations in RET, two patientes had mutations in SDHB genes. The mean age of our cohort was 56 years (range 46–73); 3 females and 4 males. Radiological response utilized RECIST 1.1 criteria and biochemical response was evaluiated.

Ergebnisse/Results Partial response (PR) was achieved in 2 and stable disease (SD) in 3 patients. In 2 patients, progressive disease (PD) was observed. One of these 2 patient had treatment refractory with disease progression and dramatic increase of chromogranin A concentration (+268%). Biochemical response (> 50 % decrease of chromogranin A) was observed in 2/7 patients and of catecholamines in 3/7 patients. No hematological or kidney toxicity grade 3–4 was registered. Median overall survival and median progression-free survival rate will be reported after the end of the study.

Schlussfolgerungen/Conclusions PRRT appears promising for treatment of MPPG with improvement of clinical symptoms and/or disease control in the setting of retrospective reports or case series. However, larger prospective studies are required to confirm these findings.

Publication History

Article published online:
08 April 2021