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Paraganglioma

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Evidence-Based Endocrine Surgery

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Abstract

Paragangliomas are rare tumors that arise from the sympathetic ganglia of the chest and abdomen and the parasympathetic ganglia of the head and neck. They often are found incidentally on imaging that is performed for another reason, although they may also present with the classic symptoms of hypertension, anxiety, nausea and vomiting, or catecholamine crisis. Patients with suspicion of paraganglioma should undergo biochemical testing with plasma or urine metanephrines to confirm the diagnosis. Cross-sectional imaging with CT or MRI is necessary to localize the tumor and for surgical planning. If patients are at high risk for metastatic disease, functional imaging with 123I-MIBG, 18F-FDG, or 68Ga-DOTATATE may be performed to localize additional sites of disease. All patients with paraganglioma should be referred to a genetic counselor for consideration of genetic testing, since at least 30% of patients with paraganglioma may have a genetic mutation, most commonly in one of the SDH protein subtypes.

Preoperative alpha-blockade is essential before surgery. Open resection of paraganglioma is usually preferred, although a laparoscopic approach may be attempted if the surgeon is comfortable with the technique. Removal of the tumor without violating the capsule is essential to prevent local seeding and recurrence. After surgery, patients should be monitored yearly with metanephrine levels and cross-sectional imaging if biochemical testing shows evidence of recurrence. Surgical resection is the first line of therapy in patients with recurrence or metastases when possible and has the best outcomes. Local ablative therapy, chemotherapy, or ablation with radiolabeled peptides may be considered in patients with disease that is not amenable to resection. Patients with metastatic disease have a varying course but can often achieve good disease-free intervals and overall survival when treated by a multidisciplinary team experienced in treating this disease.

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Authors and Affiliations

  1. Weill Cornell Medicine, New York, NY, USA

    Toni Beninato

  2. University of California, San Francisco, CA, USA

    Quan-Yang Duh

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  1. Division of Endocrine Surgery, National University Hospital, Singapore, Singapore

    Rajeev Parameswaran

  2. Department of Endocrine Surgery, SGPGI Hospital, Lucknow, India

    Amit Agarwal

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Beninato, T., Duh, QY. (2018). Paraganglioma. In: Parameswaran, R., Agarwal, A. (eds) Evidence-Based Endocrine Surgery. Springer, Singapore. https://doi.org/10.1007/978-981-10-1124-5_30

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