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Pheochromocytoma diagnosed during pregnancy: lessons learned from a series of ten patients

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A Correction to this article was published on 20 March 2018

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Abstract

Background

Pheochromocytoma (PHEO) in pregnancy is a life-threatening condition. Its management is challenging with regards to the timing and type of surgery.

Methods

A retrospective review of the management of ten patients diagnosed with pheochromocytoma during pregnancy was performed. Data were collected on the initial diagnostic workup, symptoms, treatment, and follow-up.

Results

PHEO was diagnosed in ten patients who were between the 10th and the 29th weeks of pregnancy. Six patients had none to mild symptoms, while four had complications of paroxysmal hypertension. Imaging investigations consisted of MRI, CT scan and ultrasounds. All had urinary metanephrines, measured as part of their workup. Three patients had MEN 2A, one VHL syndrome, one suspected SDH mutation. All patients were treated either with α/β blockers or calcium channel blockers to stabilize their clinical conditions. Seven patients underwent a laparoscopic adrenalectomy before delivery. Three out of these seven patients had a bilateral PHEO and underwent a unilateral adrenalectomy of the larger tumor during pregnancy, followed by a planned cesarean section and a subsequent contralateral adrenalectomy within a few months after delivery. Three patients had emergency surgery for maternal or fetal complications, with C-section followed by concomitant or delayed adrenalectomy. All newborns from the group of planned surgery were healthy, while two out three newborns within the emergency surgery group died shortly after delivery secondary to cardiac and pulmonary complications.

Conclusions

PHEO in pregnancy is a rare condition. Maternal and fetal prognosis improved over the last decades, but still lethal consequences may be present if misdiagnosed or mistreated. A thorough multidisciplinary team approach should be tailored on an individual basis to better manage the pathology. Unilateral adrenalectomy in a pregnant patient with bilateral PHEO may be an option to avoid the risk of adrenal insufficiency after bilateral adrenalectomy.

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Change history

  • 20 March 2018

    The original article was updated to correct the listing of A. Hamy’s name; it is correct as displayed above.

References

  1. Harrington JL, Farley DR, van Heerden JA, Ramin KD (1999) Adrenal tumors and pregnancy. World J Surg 23(2):182–186

    Article  PubMed  CAS  Google Scholar 

  2. Lenders JW, Eisenhofer G, Mannelli M, Pacak K (2005) Phaeochromocytoma. Lancet 366(9486):665–675 (Review)

    Article  PubMed  Google Scholar 

  3. Brunt LM (2001) Phaeochromocytoma in pregnancy. Br J Surg 88(4):481–483

    Article  PubMed  CAS  Google Scholar 

  4. Biggar MA, Lennard TW (2013) Systematic review of phaeochromocytoma in pregnancy. Br J Surg. https://doi.org/10.1002/bjs.8976

    Article  PubMed  Google Scholar 

  5. Lenders JW (2012) Pheochromocytoma and pregnancy: a deceptive connection. Eur J Endocrinol. https://doi.org/10.1530/EJE-11-0528. (Epub 2011 Sep 2. Review, Feb, 2012)

    Article  PubMed  Google Scholar 

  6. Lamb MN, Farley DR (2005) Adrenal tumors and pregnancy. In: Linos D, van Heerden JA, Adrenal glands. Springer-Verlag, Berlin Heidelberg, pp 265–273

    Chapter  Google Scholar 

  7. Oliva R, Angelos P, Kaplan E, Bakris G (2010) Pheochromocytoma in pregnancy: a case series and review. Hypertension. https://doi.org/10.1161/HYPERTENSIONAHA.109.147579

    Article  PubMed  Google Scholar 

  8. Dahia PL, Hayashida CY, Strunz C, Abelin N, Toledo SP (1994) Low cord blood levels of catecholamine from a newborn of a pheochromocytoma patient. Eur J Endocrinol 130(3):217–219

    Article  PubMed  CAS  Google Scholar 

  9. Lansdown A, Rees DA. (2011) Endocrine oncology in pregnancy. Best Pract Res Clin Endocrinol Metab. https://doi.org/10.1016/j.beem.2011.06.011. (Review, Dec, 2011)

    Article  PubMed  Google Scholar 

  10. Ahn J, Hibbard J, Chapa J (2003) Atypical presentation of pheochromocytoma as part of multiple endocrine neoplasia IIa in pregnancy. Obstet Gynecol 102:1202–1205

    PubMed  Google Scholar 

  11. Guerrero MA, Schreinemakers JM, Vriens MR, Suh I, Hwang J, Shen WT, Gosnell J, Clark OH, Duh QY (2009) Clinical spectrum of pheochromocytoma. J Am Coll Surg. https://doi.org/10.1016/j.jamcollsurg.2009.09.022

    Article  PubMed  Google Scholar 

  12. Takai Y, Seki H, Kinoshita K (1997) Pheochromocytoma in pregnancy manifesting as hypertensive crisis induced by metoclopramide. Int J Gynecol Obstet 59:133–137

    Article  CAS  Google Scholar 

  13. Hudsmith JG, Thomas CE, Browne DA (2006) Undiagnosed phaeochromocytoma mimicking severe preeclampsia in a pregnant woman at term. Int J Obstet Anesth 15(3):240–245

    Article  PubMed  CAS  Google Scholar 

  14. Petrie J, Lockie C, Paolineli A, Stevens M, Smith M, Mitchell C, Dubrey SW (2012) Undiagnosed phaeochromocytoma masquerading as eclampsia. BMJ Case Rep. https://doi.org/10.1136/bcr.10.2011.4922

    Article  PubMed  PubMed Central  Google Scholar 

  15. Desai AS, Chutkow WA, Edelman E, Economy KE, Dec GW Jr (2009) Clinical problem-solving: a crisis in late pregnancy. N Engl J Med. https://doi.org/10.1056/NEJMcps0708258

    Article  PubMed  Google Scholar 

  16. Kondziella D, Lycke J, Szentgyörgyi E (2007) A diagnosis not to miss: pheochromocytoma during pregnancy. J Neurol 254(11):1612–1613

    Article  PubMed  Google Scholar 

  17. Salazar-Vega JL, Levin G, Sansó G, Vieites A, Gómez R, Barontini M (2014) Pheochromocytoma associated with pregnancy: unexpected favourable outcome in patients diagnosed after delivery. J Hypertens. https://doi.org/10.1097/HJH.0000000000000215

    Article  PubMed  Google Scholar 

  18. Oishi S, Sato T (1994) Pheochromocytoma in pregnancy: a review of the Japanese Literature. Endocr J 41(3):219–225

    Article  PubMed  CAS  Google Scholar 

  19. Orioli L, Debiève F, Donckier J, Mourad M, Lois F, Maiter D (2017) Pheochromocytoma during pregnancy: Case report and review of recent literature. Ann Endocrinol (Paris). https://doi.org/10.1016/j.ando.2017.05.004

    Article  Google Scholar 

  20. Wing LA, Conaglen JV, Meyer-Rochow GY, Elston MS, Paraganglioma in pregnancy: a case series and review of the literature. J Clin Endocrinol Metab. https://doi.org/10.1210/jc.2015-2122

  21. Cecchi R, Frati P, Capri O, Cipolloni L (2013) A rare case of sudden death due to hypotension during cesarean section in a woman suffering from pheochromocytoma and neurofibromatosis. J Forensic Sci. https://doi.org/10.1111/1556-4029.12279

    Article  PubMed  Google Scholar 

  22. Madani R, Al-Hashmi M, Bliss R, Lennard TW (2007) Ectopic pheochromocytoma: does the rule of tens apply? World J Surg 31(4):849–854

    Article  PubMed  CAS  Google Scholar 

  23. Neumann HP, Bausch B, McWhinney SR, Bender BU, Gimm O et al (2002) Freiburg-Warsaw-Columbus Pheochromocytoma Study Group. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 346(19):1459–1466

    Article  PubMed  CAS  Google Scholar 

  24. Dong D, Li H (2014) Diagnosis and treatment of pheochromocytoma during pregnancy. J Matern Fetal Neonatal Med. https://doi.org/10.3109/14767058.2014.880883

    Article  PubMed  PubMed Central  Google Scholar 

  25. Cammarano WB, Gray AT, Rosen MA, Lim KH (1997) Anesthesia for combined cesarean section and extra-adrenal pheochromocytoma resection: a case report and literature review. Int J Obstet Anesth 6(2):112–117

    Article  PubMed  CAS  Google Scholar 

  26. Zuluaga-Gómez A, Arrabal-Polo M, Arrabal-Martín M, Lahoz-García C, Escobar-Jiménez F, Torres-Vela E, Saz T (2012) Management of pheochromocytoma during pregnancy: laparoscopic adrenalectomy. Am Surg 78(3):E156-8 (No abstract available)

    PubMed  Google Scholar 

  27. Holthausen UH, Mettler L, Troidl H (1999) Pregnancy: a contraindication? World J Surg 23(8):856–862

    Article  PubMed  CAS  Google Scholar 

  28. Junglee N, Harries SE, Davies N, Scott-Coombes D, Scanlon MF, Rees DA (2007) Pheochromocytoma in Pregnancy: when is operative intervention indicated? J Womens Health (Larchmt) 16(9):1362–1365

    Article  CAS  Google Scholar 

  29. Kitayama K, Kashiwagi S, Amano R, Noda S, Ohira G, Yamazoe S, Kimura K, Hamamoto K, Hamuro A, Ohsawa M, Onoda N, Hirakawa K (2015) A case of bilateral pheochromocytoma during pregnancy. BMC Surg. https://doi.org/10.1186/s12893-015-0041-1

    Article  PubMed  PubMed Central  Google Scholar 

  30. Pearl JP, Price RR, Tonkin AE, Richardson WS, Stefanidis D (2017) SAGES guidelines for the use of laparoscopy during pregnancy. Surg Endosc. https://doi.org/10.1007/s00464-017-5637-3

    Article  PubMed  Google Scholar 

  31. Pacak K, Jochmanova I, Prodanov T, Yang C, Merino MJ, Fojo T, Prchal JT, Tischler AS, Lechan RM, Zhuang Z (2013) New syndrome of paraganglioma and somatostatinoma associated with polycythemia. J Clin Oncol. https://doi.org/10.1200/JCO.2012.47.1912

    Article  PubMed  PubMed Central  Google Scholar 

  32. Jochmanova I, Lazurova I (2014) A new twist in neuroendocrine tumor research: Pacak-Zhuang syndrome, HIF-2α as the major player in its pathogenesis and future therapeutic options. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. https://doi.org/10.5507/bp.2014.021

    Article  PubMed  Google Scholar 

  33. Langlois F, Lim DST, Fleseriu M (2017) Update on adrenal insufficiency: diagnosis and management in pregnancy. Curr Opin Endocrinol Diabetes Obes. https://doi.org/10.1097/MED.0000000000000331

    Article  PubMed  Google Scholar 

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Funding

This research did not receive any specific grant from any funding agency in the public, commercial, or not-for-profit sector.

Author information

Authors and Affiliations

  1. Department of General and Endocrine Surgery, CHU Poitiers, 2 Rue de la Miletrie, 86021, Poitiers, France

    G. Donatini & J. L. Kraimps

  2. Department of General and Endocrine Surgery, CHU Nantes, Nantes, France

    C. Caillard & E. Mirallie

  3. Department of Obstetrics and Gynaecology, CHU Poitiers, Poitiers, France

    F. Pierre

  4. Department of General and Endocrine Surgery, CHU Tours, Tours, France

    Loïc De Calan

  5. Department of General and Endocrine Surgery, CHU Angers, Angers, France

    A. Hamy

  6. Department of Endocrine Surgery, Ukrainian Scientific and Practical Center for Endocrine Surgery of Public Health Ministry of Ukraine, Kiev, Ukraine

    O. Larin, O. Tovkay & S. Cherenko

Authors
  1. G. Donatini
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  2. J. L. Kraimps
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  3. C. Caillard
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  4. E. Mirallie
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  5. F. Pierre
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  6. Loïc De Calan
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  7. A. Hamy
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  8. O. Larin
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  9. O. Tovkay
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  10. S. Cherenko
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Corresponding author

Correspondence to G. Donatini.

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Disclosure

Gianluca Donatini, Jean-Louis Kraimps, Cecile Caillard, Eric Mirallie, Fabrice Pierre, Loïc De, Antoine Hamy, Oleg Larin, Oleg Tovkay, and Sergii Cherenko have nothing to disclose.

Additional information

The ​original ​version ​of ​this ​article ​was ​revised: The original article was updated to correct the listing of A. Hamy’s name; it is correct as displayed above.

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Donatini, G., Kraimps, J.L., Caillard, C. et al. Pheochromocytoma diagnosed during pregnancy: lessons learned from a series of ten patients. Surg Endosc 32, 3890–3900 (2018). https://doi.org/10.1007/s00464-018-6128-x

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  • DOI: https://doi.org/10.1007/s00464-018-6128-x

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