Treatment of inoperable or metastatic paragangliomas and pheochromocytomas with peptide receptor radionuclide therapy using 177Lu-DOTATATE

Wouter T Zandee*, Richard A Feelders, Daan A Smit Duijzentkunst, Johannes Hofland, R Mick Metselaar, Rogier A Oldenburg, Anne van Linge, Boen L R Kam, Jaap J M Teunissen, Esther Korpershoek, Johanna M Hendriks, Huda Abusaris, Cleo Slagter, Gaston J H Franssen, Tessa Brabander, Wouter W De Herder

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

6 Citations (Scopus)


Objectives: Inoperable or metastatic paragangliomas (PGLs) and malignant pheochromocytomas (PCCs) are rare tumours with limited options for systemic treatment. Aim of this study was to assess the safety and efficacy of the radiolabelled somatostatin analogue (177LutetiumDOTA0-Tyr3)octreotate (177Lu-DOTATATE) for the treatment of PGLs and PCCs.

Methods: Patients with histologically proven inoperable or malignant PGLs and PCCs treated with 177Lu-DOTATATE at our centre were retrospectively analysed. Patients were treated with up to four cycles of 177Lu-DOTATATE with an intended dose of 7.4 Gb per cycle. Response was assessed with use of RECIST 1.1.

Results: Thirty patients were included: 17 with parasympathetic, 10 with sympathetic PGLs and 3 with PCCs. Grade 3/4 subacute haematotoxicity occurred in 6 (20%) of patients. A reversible subacute adverse event due to cardiac failure following possible catecholamine release occurred in two patients. Best tumour response was partial response in 7 (23%) and stable disease in 20 (67%), whereas 3 (10%) patients had progressive disease. In 20 patients with baseline disease progression, tumour control was observed in 17 (85%); the median progression-free survival was 91 months in patients with parasympathetic PGLs, 13 months in patients with sympathetic PGLs and 10 months in patients with metastatic PCCs.

Conclusion: This study suggests that PRRT with 177Lu-DOTATATE is a safe and effective treatment option for patients with inoperable or malignant PGL and PCC.

Original languageEnglish
Pages (from-to)45-53
Number of pages9
JournalEuropean Journal of Endocrinology
Issue number1
Publication statusPublished - Jul-2019
Externally publishedYes


  • Adrenal Gland Neoplasms/radiotherapy
  • Adult
  • Aged
  • Female
  • Humans
  • Male
  • Middle Aged
  • Neoplasm Metastasis
  • Octreotide/analogs & derivatives
  • Organometallic Compounds/therapeutic use
  • Paraganglioma/radiotherapy
  • Pheochromocytoma/radiotherapy
  • Radiation Dosage
  • Radioisotopes/therapeutic use
  • Receptors, Peptide/radiation effects
  • Retrospective Studies
  • Treatment Outcome

Cite this