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VOLUME 10 , ISSUE 3 ( September-December, 2018 ) > List of Articles


Chromaffin-cell Tumors in Pregnancy: A Case Series and Systematic Review

Maximilien Rappaport, Paul Skierczynski, Lauren Dungy-Poythress, Tara Benjamin, Brian D Saunders, Andrew A Wagner, Benjamin C James

Keywords : Adrenal tumor, Maternal hypertension, Paragangliomas, Pheochromocytoma, Pregnancy, Pregnant

Citation Information : Rappaport M, Skierczynski P, Dungy-Poythress L, Benjamin T, Saunders BD, Wagner AA, James BC. Chromaffin-cell Tumors in Pregnancy: A Case Series and Systematic Review. World J Endoc Surg 2018; 10 (3):163-169.

DOI: 10.5005/jp-journals-10002-1240

License: CC BY-NC 4.0

Published Online: 01-12-2018

Copyright Statement:  Copyright © 2018; The Author(s).


Aim: We describe three chromaffin-cell tumors managed during pregnancy as well as systematically review case reports published from 2014 to 2018. Chromaffin-cell tumors are rare catecholamine-producing tumors that can arise from the adrenal medulla, where they are referred to as pheochromocytomas, or from extra-adrenal chromaffin tissue, referred to as paragangliomas. The incidence of chromaffin-cell tumors identified during pregnancy is extremely rare, with an incidence of 0.32 cases per 100,000 pregnancy years. Cases: We describe diagnosis and management during pregnancy of a 25-year-old with a 7.3 cm right pheochromocytoma, a 23-year-old with metastatic paraganglioma and SDHB mutation, and a 28-year-old with MEN2A and a left pheochromocytoma. We performed a systematic review of cases utilizing MEDLINE, EMBASE and Google Scholar with the terms (pheochromocytoma or paraganglioma) and (pregnancy or pregnant) within the timeframe 2014 through 2018 (searched on April 9th, 2018). We found that emergency cesarean section delivery (p < 0.05), maternal heart failure or pulmonary edema (p<0.05) and fetal or neonatal death (p < 0.05) were more common in women with a late or postpartum diagnosis of a chromaffin-cell tumor compared to women with diagnosis during or before pregnancy. Conclusion: Chromaffin-cell tumors are rare during pregnancy. However, morbidity is severe and requires an early diagnosis for the best possible outcomes. Hypertension during pregnancy is the most common presenting symptom of these catecholamineproducing tumors. Severe hypertension, labile hypertension or hypertension before 20 weeks, without proteinuria or lower extremity edema, should raise suspicion for a chromaffin-cell tumor. Management should consist of an experienced multidisciplinary team at a tertiary referral hospital to ensure the best outcomes.

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  1. Harrington JL, Farley DR, van Heerden JA, Ramin KD. Adrenal tumors and pregnancy. World journal of surgery. 1999 Feb 21;23(2):182-186.
  2. Quartermaine G, Lambert K, Rees K, Seed PT, Dhanjal MK, Knight M, et al. Hormone-secreting adrenal tumours cause severe hypertension and high rates of poor pregnancy outcome; a UK Obstetric Surveillance System study with case control comparisons. BJOG: An International Journal of Obstetrics & Gynaecology. 2018 May;125(6):719-727.
  3. Berends AM, Buitenwerf E, de Krijger RR, Veeger NJ, van der Horst-Schrivers AN, et al. Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review. European journal of internal medicine. 2018 May 1;51:68-73.
  4. Biggar MA, Lennard TW. Systematic review of phaeochromocytoma in pregnancy. British journal of surgery. 2013 Jan;100(2):182-190.
  5. Prete A, Paragliola RM, Salvatori R, Corsello SM. Management of catecholamine-secreting tumors in pregnancy: a review. Endocrine Practice. 2016 Mar;22(3):357-370.
  6. Wing LA, Conaglen JV, Meyer-Rochow GY, Elston MS. Paraganglioma in pregnancy: a case series and review of the literature. The Journal of Clinical Endocrinology & Metabolism. 2015 Aug 1;100(8):3202-3209.
  7. Buitenwerf E, Osinga TE, Timmers HJ, Lenders JW, Feelders RA, Eekhoff EM, et al. Randomized trial comparing phenoxybenzamine and doxazosine for preoperative treatment of patients with a pheochromocytoma (PRESCRIPT). In20th European Congress of Endocrinology 2018 May 8 (Vol. 56). Bio Scientifica.
  8. Versmissen J, Koch BC, Roofthooft DW, Bosch-Dijksman W, Meiracker AH, Hanff LM, et al. Doxazosin treatment of phaeochromocytoma during pregnancy: placental transfer and disposition in breast milk. British journal of clinical pharmacology. 2016 Aug 1;82(2):568-569.
  9. Schenker JG, Granat M. Phaeochromocytoma and pregnancy. an updated appraisal. Australian and New Zealand Journal of Obstetrics and Gynaecology. 1982 Feb;22(1):1-10.
  10. Plu I, Sec I, Barres D, Lecomte D. Pregnancy, cesarean, and pheochromocytoma: a case report and literature review. Journal of forensic sciences. 2013 Jul;58(4):1075-1079.
  11. Junglee N, Harries SE, Davies N, Scott-Coombes D, Scanlon MF, Rees DA. Pheochromocytoma in pregnancy: when is operative intervention indicated?. Journal of Women's Health. 2007 Nov 1;16(9):1362-1365.
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