Surgical approaches and results of treatment for hereditary paragangliomas

Best Pract Res Clin Endocrinol Metab. 2019 Oct;33(5):101298. doi: 10.1016/j.beem.2019.101298. Epub 2019 Jul 27.


Paragangliomas (PGL) are rare neuroendocrine tumours; parasympathetic PGL are predominantly non-secreting and located at the skull base and neck, while sympathetic PGL are typically catecholamine-secreting and located at abdomino-pelvic level. Approximately 40% of PGL may be caused by germline mutations; hereditary variants should be suspected especially in case of positive family history, early onset, multifocal, or recurrent PGL. Significant genotype-phenotype correlation has been recognized, including syndromic presentation, location, multifocality and risk of malignancy. Surgical resection remains the only curative strategy, but the outcomes may be unsatisfactory because of surgical morbidity and recurrence rate. However, due to the rarity of the disease, most data derive from case-report or limited series. This paper was aimed to review the available literature on the epidemiology, diagnosis, clinical features, treatment of PGL in order to discuss the surgical approach and the results of treatment in hereditary PGL.

Keywords: hereditary paraganglioma; pheochromocytoma/paragangliomas syndromes; surgical resection.

Publication types

  • Review

MeSH terms

  • Adrenal Gland Neoplasms / diagnosis
  • Adrenal Gland Neoplasms / genetics
  • Adrenal Gland Neoplasms / surgery*
  • Endocrine Surgical Procedures / adverse effects
  • Endocrine Surgical Procedures / methods*
  • Endocrine Surgical Procedures / standards
  • Germ-Line Mutation
  • Humans
  • Pheochromocytoma / diagnosis
  • Pheochromocytoma / genetics
  • Pheochromocytoma / surgery*
  • Postoperative Complications / epidemiology