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Publications

Journal Article Ahead of print

PubMed: 42454478 DOI: 10.1530/ERC-26-0036

Molecular Imaging and Radioligand Therapies in Pheochromocytomas and Paragangliomas.

Endocr Relat Cancer

Authors: Jorge H Hernandez-Felix, Mahshid Golagha, Abhishek Jha, Diana Varghese, Simona Grozinsky-Glasberg, Rachel Riechelmann, Nicola Fazio, Marianne Pavel, Frank I Lin, David Taïeb, Karel Pacak, Jaydira Del Rivero

Affiliations

  • Developmental Therapeutics Clinic, National Cancer Institute (NCI), National Institutes of Health , Bethesda, MD, USA. Departament of Hematology and Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán , Mexico City, Mexico.
  • Developmental Therapeutics Clinic, National Cancer Institute (NCI), National Institutes of Health , Bethesda, MD, USA.
  • Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health , Bethesda, MD, USA.
  • Neuroendocrine Tumor Unit, ENETS Center of Excellence, Division of Medicine, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem , Jerusalem, Israel.
  • Department of Clinical Oncology, AC Camargo Cancer Center , São Paulo, Brazil.
  • European Institute of Oncology (IEO), IRCCS, Division of Gastrointestinal Medical Oncology and Neuroendocrine Tumors , MilanItaly.
  • Department of Medicine 1, Uniklinikum Erlangen and Comprehensive Cancer Center CCC-EMN, Friedrich Alexander University Erlangen-Nürnberg , Erlangen, Germany.
  • Molecular Imaging Branch, National Cancer Institute, National Institutes of Health , Bethesda, MD, USA.
  • Nuclear Medicine, La Timone University Hospital, Aix-Marseille University , Marseille, France.
  • Center for Adrenal Endocrine Tumors, AKESO , Prague 5, Czech Republic.

Abstract

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors of neural crest origin that exhibit wide clinical and biological heterogeneity. While surgical resection remains the only curative option, many patients have unresectable or metastatic progressive disease and/or with uncontrolled secretion requiring alternative management strategies. Advances in genetics have revealed germline pathogenic variants in up to 30-40% of cases, refining tumor classification and guiding the use of molecularly targeted imaging. Functional imaging modalities like 68Ga or 64Cu-DOTA-SSA (somatostatin analogs) , 18F-FDOPA, 18F-FDG, and 123I-MIBG, exploit tumor biology such as somatostatin receptor expression, catecholamine biosynthesis, and altered glucose metabolism. These approaches not only improve diagnostic accuracy but also help in prognosis and selection to therapies. Radioligand therapies have emerged as pivotal option in advanced PPGLs. High-specific-activity 131I-MIBG has demonstrated durable disease control, symptomatic improvement, and favorable safety in multicenter studies, leading to FDA approval for metastatic PPGL, although currently not commercially available. More recently, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE has shown disease control rates of 80-100% in prospective and retrospective trials, with outcomes enhanced by standardized dosing strategies. Ongoing studies are evaluating PRRT in genetically defined subgroups and exploring synergistic combinations, including radiosensitizing approaches.

MeSH

Paraganglioma, Somatostatin, Pheochromocytoma, Neoplasms, Therapeutics, Molecular Imaging, Endocrine System Diseases