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Arthur S Tischler Department of Pathology and Laboratory Medicine, Tufts Medical Center and Tufts University School of Medicine, Boston, Massachusetts, USA

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Judith Favier Université Paris cité, Inserm UMR970 PARCC, Equipe Labellisée par la Ligue contre le cancer, Paris, France

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lacking for pheochromocytoma and paraganglioma (PGL). There are no human cell lines that faithfully represent these tumors although many attempts have been made to establish them. The failure may be explained in large part simply by the extremely low

Article Type:
Review Article
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Advances and Future Directions in Pheochromocytoma and Paraganglioma
DOI:
https://doi.org/10.1530/ERC-22-0405
Volume/Issue:
Volume 30: Issue 5
Free access
Karel Pacak Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Mark Kidd WREN Laboratories, Branford, Connecticut, USA

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Leah Meuter Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Irvin M Modlin Gastroenterological and Endoscopic Surgery, Yale University School of Medicine, New Haven, Connecticut, USA

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Introduction Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are neural crest-derived tumors that are most commonly found in the adrenal glands. These tumors typically arise from chromaffin cells, which are a group of cells that have the

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-21-0216
Volume/Issue:
Volume 28: Issue 11
Restricted access
Sophie Moog PARCC, INSERM UMR970, Equipe Labellisée par la Ligue contre le Cancer, Paris, France
Université de Paris, Paris, France

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Charlotte Lussey-Lepoutre PARCC, INSERM UMR970, Equipe Labellisée par la Ligue contre le Cancer, Paris, France
Department of Nuclear Medicine, Sorbonne University, Pitie-Salpetriere Hospital, Paris, France

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Judith Favier PARCC, INSERM UMR970, Equipe Labellisée par la Ligue contre le Cancer, Paris, France
Université de Paris, Paris, France

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Introduction Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from the adrenal medulla or extra-adrenal paraganglia, respectively. Paragangliomas can be found in the sympathetic nervous system of the thorax

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-20-0346
Volume/Issue:
Volume 27: Issue 12
Free access
Rodrigo A Toledo Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Yuejuan Qin Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Subramanya Srikantan Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Nicole Paes Morales Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Qun Li Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Yilun Deng Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Sang-Woo Kim Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Maria Adelaide A Pereira Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Sergio P A Toledo Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Xiaoping Su Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Ricardo C T Aguiar Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA
Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Patricia L M Dahia Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA
Division of Hematology and Medical Oncology, Greehey Children Cancer Research Institute, University of São Paulo School of Medicine, Department of Bioinformatics and Computational Biology, Department of Medicine, Cancer Therapy and Research Center at the University of Texas Health Science Center, 7703 Floyd Curl Drive, MC 7880, San Antonio, Texas 78229-3900, USA

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Introduction Pheochromocytomas and paragangliomas are sympathetic neural-derived neoplasms. Over one third of these tumors carry germline mutations in one of the ten distinct genes ( Jafri & Maher 2012 ). Remarkably, somatic events in

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-13-0101
Volume/Issue:
Volume 20: Issue 3
Free access
Rodrigo Almeida Toledo Division of Hematology and Medical Oncology, Department of Medicine, Cancer Therapy and Research Center, University of Texas Health Science Center at San Antonio (UTHSCSA), San Antonio, Texas, USA

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clinical features, i.e., renal carcinoma alone, renal carcinoma associated with pheochromocytoma, and pheochromocytoma alone ( Crossey et al. 1995 , Zbar et al. 1996 ). Pheochromocytomas and paragangliomas (PPGLs) are neural

Article Type:
Article Commentary
DOI:
https://doi.org/10.1530/ERC-16-0479
Volume/Issue:
Volume 24: Issue 9
Free access
Ales Vicha
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David Taieb Department of Pediatric Hematology and Oncology, Service Central de Biophysique et de Médecine Nucléaire, Département d'Oncologie Moléculaire, Program in Reproductive and Adult Endocrinology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic
Department of Pediatric Hematology and Oncology, Service Central de Biophysique et de Médecine Nucléaire, Département d'Oncologie Moléculaire, Program in Reproductive and Adult Endocrinology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic

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Karel Pacak Department of Pediatric Hematology and Oncology, Service Central de Biophysique et de Médecine Nucléaire, Département d'Oncologie Moléculaire, Program in Reproductive and Adult Endocrinology, 2nd Medical School, Charles University and University Hospital Motol, Prague, Czech Republic

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these metabolic pathways or restoring altered pathways can lead to new approaches in cancer treatment. Pheochromocytoma and paraganglioma Pheochromocytomas (PHEOs)/paragangliomas (PGLs) are rare neuroendocrine tumors that produce catecholamines ( Lenders

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-13-0398
Volume/Issue:
Volume 21: Issue 3
Free access
David Taïeb Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France

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Abhishek Jha Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Giorgio Treglia Clinic of Nuclear Medicine and PET/CT Center, Ente Ospedaliero Cantonale, Bellinzona, Switzerland
Department of Nuclear Medicine and Molecular Imaging, Lausanne University Hospital, Lausanne, Switzerland
Health Technology Assessment Unit, General Directorate, Ente Ospedaliero Cantonale, Bellinzona, Switzerland

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Karel Pacak Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Learning points Know the molecular taxonomy of pheochromocytoma and/or paraganglioma (PPGL) and the relationship between various genotypes and imaging phenotypes; Describe imaging features of SDHx -related PPGL; Understand

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-19-0165
Volume/Issue:
Volume 26: Issue 11
Free access
William Beimers Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Megan Braun Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Kaleb Schwinefus Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Keenan Pearson Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Brandon Wilbanks Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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Louis James Maher Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA

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when oxygen is abundant ( Warburg 1956 ). Since this discovery, many cancers have been found to have other forms of altered metabolism besides the Warburg effect ( Pavlova & Thompson 2016 , Kozal et al. 2021 ). Familial paraganglioma (PGL) is a

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-21-0349
Volume/Issue:
Volume 29: Issue 6
Open access
Jacques Amar Department of Arterial Hypertension, Toulouse University, Hôpital Rangueil, Toulouse, France
INSERM UMR1048, I2MC, Toulouse, France

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Jeremy Brunel Department of Arterial Hypertension, Toulouse University, Hôpital Rangueil, Toulouse, France

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Catherine Cardot Bauters Service d’Endocrinologie, Hôpital Claude Huriez, CHU Lille, Lille, France

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Virginie Jacques Laboratory of Biochemistry and Molecular Biology, IFB-CHU, Toulouse, France
Laboratory of Biochemistry and Molecular Biology, IFB-CHU, Toulouse, France

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Clément Delmas INSERM UMR1048, I2MC, Toulouse, France
Department of Cardiology Toulouse University, Hôpital Rangueil, Toulouse, France

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Marie-Françoise Odou Service de Biochimie et Biologie Moléculaire ‘Hormonologie, Métabolisme-Nutrition, Oncologie’, CHU Lille, Lille, France
Univ. Lille, Inserm, CHU Lille, U1286 – Infinite – Institute for Translational Research in Inflammation, Lille, France

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Frédérique Savagner Laboratory of Biochemistry and Molecular Biology, IFB-CHU, Toulouse, France
Laboratory of Biochemistry and Molecular Biology, IFB-CHU, Toulouse, France

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Introduction Pheochromocytoma–paraganglioma (PPGL) are rare tumors ( Berends et al. 2018 ) that produce and store catecholamines. The release of excessive amounts of catecholamines can lead to life-threatening cardiovascular complications

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-21-0373
Volume/Issue:
Volume 29: Issue 5
Restricted access
Fredrika Svahn Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

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Karolina Solhusløkk Höse Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

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Adam Stenman Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital, Stockholm, Sweden

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Yaxuan Liu Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Breast Surgery, Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China

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Jan Calissendorff Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

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Emma Tham Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Clinical Genetics, Karolinska University Hospital, Stockholm, Sweden

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Ákos Végvári Division of Chemistry I, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden

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Roman A Zubarev Division of Chemistry I, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden

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Na Wang Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Medicine Huddinge, Karolinska Institutet, Huddinge, Sweden

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Reju Korah Yale Endocrine Neoplasia Laboratory, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, USA

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Tobias Carling Yale Endocrine Neoplasia Laboratory, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, USA
Carling Adrenal Center, Tampa, Florida, USA

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Jan Zedenius Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital, Stockholm, Sweden

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Robert Bränström Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital, Stockholm, Sweden

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C Christofer Juhlin Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Clinical Pathology and Cancer Diagnostics, Karolinska University Hospital Stockholm, Sweden

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Catharina Larsson Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

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Introduction Pheochromocytoma (PCC) is a neuroendocrine tumor that arises from chromaffin cells in the adrenal medulla, and abdominal paraganglioma (aPGL) exhibits a highly related cellular origin ( Mete et al. 2022 ). Based on their

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-23-0061
Volume/Issue:
Volume 31: Issue 9
Open access