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Paraganglioma and Pheochromocytoma

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Neuroendocrine Tumors

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Abstract

Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare neuroendocrine tumors arising from adrenal medulla and extra-adrenal sympathetic or parasympathetic paraganglia, respectively. Parasympathetic PGL are predominantly non-secreting and located in the head and neck and at the base of the skull (HNPGL), while sympathetic PGL are catecholamine-secreting and located in the abdomen and pelvis. Diagnostic work-up includes plasma or urinary metanephrines measurement, computed tomography or magnetic resonance and functional imaging. Genetic testing is recommended in all patients. Surgical resection is the only curative strategy. Minimally invasive adrenalectomy is the preferred approach for PHEO and most abdominal PGL. Surgery for HNPGL is the treatment of choice to prevent local destruction of adjacent nerves. Lifelong follow-up is suggested to detect recurrent or metastatic disease.

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Authors and Affiliations

  1. Endocrine Surgery Unit, Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy

    Maurizio Iacobone & Francesca Torresan

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  1. Maurizio Iacobone
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  2. Francesca Torresan
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Correspondence to Maurizio Iacobone .

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  1. Division of Surgical Oncology, Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA

    Jordan M. Cloyd

  2. Division of Surgical Oncology, Department of Surgery, The Ohio State University Wexner Medical Center, Columbus, OH, USA

    Timothy M. Pawlik

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Iacobone, M., Torresan, F. (2021). Paraganglioma and Pheochromocytoma. In: Cloyd, J.M., Pawlik, T.M. (eds) Neuroendocrine Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-62241-1_15

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