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Patricia L M Dahia Division of Hematology and Medical Oncology, Department of Medicine, Mays Cancer Center, University of Texas Health Science Center at San Antonio, San Antonio, Texas, USA

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Roderick Clifton-Bligh Department of Endocrinology, Royal North Shore Hospital, Northern Clinical School, Kolling Institute of Medical Research, University of Sydney, Sydney, New South Wales, Australia

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Anne-Paule Gimenez-Roqueplo AP-HP, Hôpital Européen Georges Pompidou, Genetics Department, Paris, France
Human Cancer Genetics Program, Spanish National Cancer Research Center, Madrid, Spain

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Mercedes Robledo AP-HP, Hôpital Européen Georges Pompidou, Genetics Department, Paris, France
Human Cancer Genetics Program, Spanish National Cancer Research Center, Madrid, Spain

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Camilo Jimenez Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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priority areas for future research and consensus points were collated. The next sections summarize presentations and workshop discussions on metastatic pheochromocytomas and paragangliomas (mPPGLs) and conclude with proposed plans for the coming years and

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-19-0435
Volume/Issue:
Volume 27: Issue 8
Free access
Nimrod B Kiss Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden
Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Andreas Muth Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Adam Andreasson Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden
Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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C Christofer Juhlin Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden
Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Janos Geli Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Martin Bäckdahl Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Anders Höög Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Bo Wängberg Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Ola Nilsson Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Håkan Ahlman Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Catharina Larsson Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden
Department of Molecular Medicine and Surgery, Cancer Centre Karolinska, Department of Surgery, Department of Oncology–Pathology, Department of Pathology, Karolinska Institutet, Karolinska University Hospital, CCK R8:04, SE-171 76 Stockholm, Sweden

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Introduction Pheochromocytomas are catecholamine-secreting tumors of the chromaffin cells of the adrenal medulla. Extra-adrenal abdominal paragangliomas (here referred to as abdominal paraganglioma or paraganglioma) are related to neuroendocrine

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-12-0267
Volume/Issue:
Volume 20: Issue 1
Open access
View license
P N Span Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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J U Rao Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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S B J Oude Ophuis Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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J W M Lenders Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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F C G J Sweep Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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P Wesseling Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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B Kusters Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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F H van Nederveen Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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R R de Krijger Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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A R M M Hermus Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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H J L M Timmers Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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Introduction Paragangliomas (PGLs) are catecholamine-producing tumors that originate from chromaffin cells of the adrenal medulla (pheochromocytoma proper) or from sympathetic neuronal tissue in extra-adrenal locations of the abdomen or chest

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-10-0184
Volume/Issue:
Volume 18: Issue 3
Free access
Alexandru Saveanu Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France
Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Mihaela Muresan Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France
Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Catherine De Micco Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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David Taieb Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Anne-Laure Germanetti Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Frederic Sebag Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Jean-François Henry Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Laurent Brunaud Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Alain Enjalbert Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France
Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Georges Weryha Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Anne Barlier Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France
Laboratory of Biochemistry and Molecular Biology, Center of Neurophysiology and Neurobiology of Marseille (CRN2M), Department of Endocrinology, Anatomical Pathology Department, Nuclear Medecine, Endocrine Surgery, Department of Endocrine Surgery, Centre Hospitalo-Universitaire Conception, 147 Boulevard Baille, 13385 Marseille, France

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Introduction Pheochromocytomas (PCC) and paragangliomas (PGL) are neuroendocrine tumors derived from adrenal chromaffin cells and extra-adrenal paraganglia respectively ( Eisenhofer et al . 2008 ). These tumors cause variable secondary hypertension

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-10-0175
Volume/Issue:
Volume 18: Issue 2
Free access
Jay S Fonte Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Jeremyjones F Robles Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Clara C Chen Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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James Reynolds Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Millie Whatley Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Alexander Ling Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Leilani B Mercado-Asis Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Karen T Adams Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Victoria Martucci Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Tito Fojo Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Karel Pacak Program in Reproductive and Adult Endocrinology, Nuclear Medicine Division, Radiology and Imaging Sciences Department, Section of Endocrinology and Metabolism, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA

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Introduction Pheochromocytoma (PHEO) and paragangliomas (PGLs) are tumors arising from chromaffin cells of the adrenal medulla or extraadrenal paraganglionic tissues respectively ( DeLellis et al . 2004 ). These tumors express the cellular membrane

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-11-0243
Volume/Issue:
Volume 19: Issue 1
Free access
Tomáš Zelinka Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development
Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Henri J L M Timmers Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Anna Kozupa Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Clara C Chen Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Jorge A Carrasquillo Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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James C Reynolds Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Alexander Ling Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Graeme Eisenhofer Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Ivica Lazúrová Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Karen T Adams Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Millie A Whatley Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Jiří Widimský Jr Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Karel Pacak Reproductive Biology and Medicine Branch, Nuclear Medicine Department, Clinical Neurocardiology Section, 3rd Department of Medicine, Department of Medicine, National Institutes of Child Health and Human Development

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Introduction Pheochromocytomas (PHEO) are catecholamine-producing tumors arising from chromaffin cells of the adrenal medulla. Paragangliomas (PGL) are tumors arising either from extra-adrenal chromaffin cells, and can originate either from

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-07-0217
Volume/Issue:
Volume 15: Issue 1
Free access
David Kishlyansky D Kishlyansky, Medicine , McMaster University, Hamilton, Canada

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Alexander A Leung A Leung, Medicine, University of Calgary Cumming School of Medicine, Calgary, Canada

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Janice Pasieka J Pasieka, Department of Surgery, Cumming School of Medicine, Calgary, T2N 2T9, Canada

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Amita Mahajan A Mahajan, Department of Medicine, University of Calgary, Calgary, Canada

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Gregory Kline G Kline, Department of Medicine, University of Calgary, Calgary, Canada

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Adrenocorticotropic hormone-producing pheochromocytoma/paraganglioma are rare neuroendocrine tumours that co-secrete excess catecholamines and adrenocorticotropic hormone, resulting in Cushing syndrome. This review aims to summarize important patient characteristics, investigations, and outcomes in all cases reported in the English literature. A literature search was conducted to identify all English-language case reports and case-series describing adrenocorticotropic hormone-producing pheochromocytomas/paragangliomas. Relevant characteristics were systematically recorded. Cases that did not provide definitive evidence of an ACTH-producing pheochromocytoma/paraganglioma were excluded. Our search strategy identified 93 published cases that met the inclusion criteria. We additionally reported one patient for a total of 94 cases. Details related to patient characteristics, laboratory data, and outcomes were commonly underreported. The median age was 47 years and females accounted for 72% of cases. A cushingoid appearance was reported in 82% and hypertension in 86%. Infections were reported in 23% of patients. Urinary metanephrines were elevated at least 3-fold above normal in 74%. ACTH levels were high in 88% and inappropriately normal in 12%. The median 24-hour urinary cortisol was 21-fold the upper limit of normal. Adrenalectomy was performed in nearly all patients with 88% achieving cure of both catecholamine and glucocorticoid excess. A total of 11 patients died. Metastases were uncommon (6%). Adrenocorticotropic hormone-producing pheochromocytoma/paraganglioma is associated with considerable morbidity and mortality. It should be considered in the diagnostic workup of all patients with ectopic Cushing Syndrome. Surgical cure is achieved in most patients and infections are the leading cause of peri-operative mortality.

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-24-0029
Volume/Issue:
Accepted Manuscripts
Open access
R Vassilopoulou-Sellin Section of Endocrinology, Division of Medicine, The University of Texas, M D Anderson Cancer Center,1515 Holcombe Boulevard, Houston, Texas, USA

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Background: Tumors of the paraganglionic system represent a distinct, albeit uncommon, clinical entity characterized by catecholamine hypersecretion and hemodynamic instability; initial pathologic examination often cannot predict benign vs malignant behavior. An analysis of the clinical outcome of patients with known malignant tumors may serve to enhance the initial evaluation and therapeutic plan of all patients presenting with pheochromocytoma or paraganglioma.

Methods: At the University of Texas M D Anderson Cancer Center, 30 patients with malignant abdominal paraganglioma and 20 patients with malignant pheochromocytoma were diagnosed between 1971 and 1995. Their medical records were reviewed with particular attention to clinical characteristics and disease outcome.

Results: Among the 30 patients with paraganglioma, 73% were men, and 90% were younger than 50 years at the time of diagnosis. Sixteen patients have remained alive with persistent disease 0.2 to 25 years after initial diagnosis while eight patients died of their disease within 0.8 to 32 years. Regional recurrence and skeletal metastases were the most prominent events. Among the 20 patients with pheochromocytoma, 60% were men and 70% were younger than 50 years at the time of diagnosis. Ten patients have remained alive with persistent disease 0.8 to 20 years after initial diagnosis while five patients died of their disease within 1.5 to 39 years. Hypertension was a prominent presenting feature and regional recurrence was the most frequent pattern of treatment failure.

Conclusions: Important clinical differences distinguish adrenal pheochromocytomas from extra-adrenal, abdominal paragangliomas. Patients with paragangliomas are, as a group, younger men, more likely to have malignant lesions and a more aggressive clinical course. Patients with malignant pheochromocytomas usually present with hypertension, are somewhat older, and have less aggressive disease.

Acknowledgemnts

We thank the staff of the Department of Medical Informatics for database retrieval and the clinical faculty who participated in the patients' care. We thank Teo Spear for expert preparation of the manuscript. We thank Terry Smith, biostatistician, for her critical review and suggestions.

Article Type:
Research Article
Volume/Issue:
Volume 5: Issue 1
Free access
Nicola Tufton Department of Endocrinology, St Bartholomew’s Hospital, Barts Health NHS Trust, West Smithfield, London, UK
Centre for Endocrinology, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, UK

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Rahul Ghelani Department of Endocrinology, Royal Free Hospital, Hampstead, London, UK

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Umasuthan Srirangalingam Department of Endocrinology, Royal Free Hospital, Hampstead, London, UK

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Ajith V Kumar North East Thames Regional Genetics Service, Great Ormond Street Hospital, London, UK

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William M Drake Department of Endocrinology, St Bartholomew’s Hospital, Barts Health NHS Trust, West Smithfield, London, UK
Department of Endocrinology, Southampton General Hospital, University Hospital Southampton NHS Trust, Southampton, Hampshire, UK

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Donato Iacovazzo Department of Endocrinology, Southampton General Hospital, University Hospital Southampton NHS Trust, Southampton, Hampshire, UK

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Kassiani Skordilis Department of Histopathology, Queen Elizabeth Hospital, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK

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Daniel Berney Department of Histopathology, St Bartholomew’s Hospital, Barts Health NHS Trust, West Smithfield, London, UK

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Ma’en Al-Mrayat Department of Endocrinology, Southampton General Hospital, University Hospital Southampton NHS Trust, Southampton, Hampshire, UK

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Bernard Khoo Department of Endocrinology, Royal Free Hospital, Hampstead, London, UK

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Scott A Akker Department of Endocrinology, St Bartholomew’s Hospital, Barts Health NHS Trust, West Smithfield, London, UK

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associated phenotypes. We suggest that SDHA paraganglioma-related disease is not a low-risk condition as first described. Of our six index cases, two developed metastatic disease and a further one had local vascular invasion. One patient developed multiple

Article Type:
Letter
DOI:
https://doi.org/10.1530/ERC-17-0030
Volume/Issue:
Volume 24: Issue 7
Free access
Noriko Kimura Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Kazuhiro Takekoshi Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Akira Horii Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Ryo Morimoto Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tsuneo Imai Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Yutaka Oki Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tomohito Saito Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Sanae Midorikawa Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tadashi Arao Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Chiho Sugisawa Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Masanobu Yamada Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Yuichi Otuka Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Isao Kurihara Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Kokichi Sugano Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Minoru Nakane Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Atsushi Fukuuchi Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Takumi Kitamoto Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Jun Saito Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Tetsuo Nishikawa Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Mitsuhide Naruse Pathology Division, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Division of Sports Medicine, Faculty of Medicine, University of Tsukuba, Division of Molecular Pathology, Department of Pathology, School of Medicine, Tohoku University, Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, School of Medicine, Tohoku University, Department of Breast and Endocrine Surgery, Aichi Medical, School of Medicine, Department of Endocrinology and Metabolism, Hamamatsu Medical School, Division of Thoracic Surgery, Department of Thoracic and Cardiovascular Surgery, Kansai Medical University, Department of Diabetes, Endocrinology and Metabolism, Fukushima Medical School, First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Department of Medicine and Molecular Science, Graduate School of Medicine, Gunma University, Department of Surgery, Nippon, Telegraph and Telephone East Corporation Medical Center, Department of Internal Medicine, School of Medicine, Keio University, Genetic Counseling Clinic, National Cancer Center, Department of Oncology, Musashino Red Cross Hospital, Department of Breast and Endocrine Surgery, Mitsui Memorial Hospital, Department of Endocrinology and Metabolism, Japan Labour Health and Welfare Organization, Yokohama Rosai Hospital, Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, 18-16 Kawahara, Hakodate, Hokkaido, 041-8512, Japan

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Dear Editor Pheochromocytoma (PCC) and paraganglioma (PGL) are genetically and phenotypically heterogeneous catecholamine-producing neoplasms. They can occur sporadically or as a part of hereditary disease. Approximately 30% of PCC/PGL are believed

Article Type:
Letter
DOI:
https://doi.org/10.1530/ERC-13-0530
Volume/Issue:
Volume 21: Issue 3
Free access