Treatment of inoperable or metastatic paragangliomas and pheochromocytomas with peptide receptor radionuclide therapy

Wouter T Zandee1, Richard A Feelders1, Daan A Smit Duijzentkunst2

  • 1Department of Internal Medicine, Sector Endocrinology.

Abstract

Insights

Radioligand therapy with 177Lutetium DOTATATE offers a safe and effective treatment for rare inoperable or malignant paragangliomas and pheochromocytomas, showing significant tumor control.

Area of Science:

  • Endocrinology
  • Nuclear Medicine
  • Oncology

Background:

  • Paragangliomas (PGLs) and malignant pheochromocytomas (PCCs) are rare neuroendocrine tumors with limited systemic treatment options.
  • Inoperable or metastatic disease presents a significant clinical challenge.

Purpose of the Study:

  • To evaluate the safety and efficacy of (177LutetiumDOTA0-Tyr3)octreotate (177Lu-DOTATATE) in patients with inoperable or malignant PGLs and PCCs.
  • To assess tumor response and progression-free survival following peptide receptor radionuclide therapy (PRRT).

Main Methods:

  • Retrospective analysis of 30 patients with histologically confirmed inoperable or malignant PGLs and PCCs treated with 177Lu-DOTATATE.
  • Patients received up to four cycles of 7.4 GBq 177Lu-DOTATATE.
  • Tumor response assessed using RECIST 1.1 criteria.

Main Results:

  • Overall tumor control (partial response or stable disease) was achieved in 30 of 30 patients (100%).
  • Grade 3/4 hematotoxicity occurred in 20% of patients; two experienced reversible cardiac events.
  • Median progression-free survival varied by tumor type: 91 months (parasympathetic PGLs), 13 months (sympathetic PGLs), and 10 months (metastatic PCCs).

Conclusions:

  • Peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE is a safe and effective treatment modality for inoperable or malignant PGLs and PCCs.
  • The treatment demonstrates significant potential for tumor control and prolonged progression-free survival in this patient population.

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