Skip to main content
SpringerLink
Log in

Bilateral pheochromocytoma during the postpartum period

  • Case Report
  • Published:
Archives of Gynecology and Obstetrics Aims and scope Submit manuscript

Abstract

Background

Pheochromocytoma manifesting during pregnancy is uncommon but it is responsible for a high maternal and fetal mortality rate, especially when unrecognized. Most cases of pheochromocytoma are sporadic but they can be part of hereditary autosomal dominant syndromes.

Case

We describe a case of bilateral pheochromocytoma in a term-pregnant patient with a previous history of medullary thyroid carcinoma (MTC). Her genetic study revealed a heterozygous mutation, c.1900T>C, in the RET proto-oncogene which confirmed the diagnosis of multiple endocrine neoplasia type 2A (MEN2A). Unrecognized, the tumors caused a crisis with fatal outcome in the mother during the postpartum period. This event might have been prevented if the tumor had been detected previously.

Conclusion

MEN2A affected pregnancy is an unusual condition. This syndrome should be suspected when a pregnant patient has a history of MTC. Early detection and appropriate management can prevent serious maternal and fetal complications. We also reviewed the literature of MEN2A-affected pregnancies.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Lenders JW, Eisenhofer G, Mannelli M, Pacak K (2005) Phaeochromocytoma. Lancet 366:665–675. doi:10.1016/S0140-6736(05)67139-5

    Article  PubMed  Google Scholar 

  2. Phupong V, Witoonpanich P, Snabboon T, Tharavej C, Ultchaswadi P (2005) Bilateral pheochromocytoma during pregnancy. Arch Gynecol Obstet 271:276–279

    PubMed  Google Scholar 

  3. Mannelli M, Bemporad D (2002) Diagnosis and management of pheochromocytoma during pregnancy. J Endocrinol Invest 25:567–571

    CAS  PubMed  Google Scholar 

  4. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA Jr, Marx SJ (2001) Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671. doi:10.1210/jc.86.12.5658

    Article  CAS  PubMed  Google Scholar 

  5. Donis-Keller H, Dou S, Chi D, Carlson KM, Toshima K, Lairmore TC, Howe JR, Moley JF, Goodfellow P, Wells SA Jr (1993) Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet 2:851–856. doi:10.1093/hmg/2.7.851

    Article  CAS  PubMed  Google Scholar 

  6. Machens A, Niccoli-Sire P, Hoegel J, Frank-Raue K, van Vroonhoven TJ, Roeher HD, Wahl RA, Lamesch P, Raue F, Conte-Devolx B, Dralle H, European Multiple Endocrine Neoplasia (EUROMEN) Study Group (2003) Early malignant progression of hereditary medullary thyroid cancer. N Engl J Med 349:1517–1525. doi:10.1056/NEJMoa012915

    Article  CAS  PubMed  Google Scholar 

  7. Kim J, Reutrakul S, Davis DB, Kaplan EL, Refetoff S (2004) Multiple endocrine neoplasia 2A syndrome presenting as peripartum cardiomyopathy due to catecholamine excess. Eur J Endocrinol 151:771–777. doi:10.1530/eje.0.1510771

    Article  CAS  PubMed  Google Scholar 

  8. Moran ME, Rosenberg DJ, Zornow DH (2006) Pheochromocytoma multisystem crisis. Urology 67:846e19–846e20

    Article  Google Scholar 

  9. Chodankar CM, Abhyankar SC, Deodhar KP, Shanbhag AM (1982) Sipple’s syndrome (multiple endocrine neoplasia) in pregnancy—case report. Aust N Z J Obstet Gynaecol 22:243–244. doi:10.1111/j.1479-828X.1982.tb01455.x

    Article  CAS  PubMed  Google Scholar 

  10. Moraca-Kvapilová L, Op de Coul AA, Merkus JM (1985) Cerebral haemorrhage in a pregnant woman with a multiple endocrine neoplasia syndrome (type 2A or Sipple’s syndrome). Eur J Obstet Gynecol Reprod Biol 20:257–263

    Article  PubMed  Google Scholar 

  11. van der Vaart CH, Heringa MP, Dullaart RP, Aarnoudse JG (1993) Multiple endocrine neoplasia presenting as phaeochromocytoma during pregnancy. Br J Obstet Gynaecol 100:1144–1145

    PubMed  Google Scholar 

  12. Wax JR, Eggleston MK Jr, Teague KE (1997) Pregnancy complicated by multiple endocrine neoplasia type IIA (Sipple’s syndrome). Am J Obstet Gynecol 177:461–462. doi:10.1016/S0002-9378(97)70217-5

    Article  CAS  PubMed  Google Scholar 

  13. Huang SM, Tao BL, Tzeng CC, Liu HT, Wang WP (1997) Prenatal molecular diagnosis of RET proto-oncogene mutation in multiple endocrine neoplasia type 2A. J Formos Med Assoc 96:542–544

    CAS  PubMed  Google Scholar 

  14. Joseph JV, Harrison R, Davis RS (2000) Multiple endocrine neoplasia type IIA (Sipple’s syndrome) presenting in pregnancy. Aust N Z J Obstet Gynaecol 40:473–474. doi:10.1111/j.1479-828X.2000.tb01188.x

    Article  CAS  PubMed  Google Scholar 

  15. Tewari KS, Steiger RM, Lam ML, Rutgers JK, Berkson RA, DiSaia PJ (2001) Bilateral pheochromocytoma in pregnancy heralding multiple endocrine neoplasia syndrome IIA. A case report. J Reprod Med 46:385–388

    CAS  PubMed  Google Scholar 

  16. Martínez Brocca MA, Acosta Delgado D, Quijada D, Navarro González E, Soto Moreno A, Gonzáles Duarte D, del Valle López de Ayala A, Astorga Jiménez R (2001) Pheochromocytoma in a pregnant woman with multiple endocrine neoplasia type 2a. Gynecol Endocrinol 15:439–442

    Article  PubMed  Google Scholar 

  17. Ahn JT, Hibbard JU, Chapa JB (2003) Atypical presentation of pheochromocytoma as part of multiple endocrine neoplasia IIa in pregnancy. Obstet Gynecol 102:1202–1205. doi:10.1016/S0029-7844(03)00627-6

    Article  PubMed  Google Scholar 

  18. Martinelli P, Maruotti GM, Pasquali D, Paladini D, Agangi A, Rippa E, Colantuoni V, Bellastella A, Sinisi AA (2004) Genetic prenatal RET testing and pregnancy management of multiple endocrine neoplasia Type II A (MEN2A): a case report. J Endocrinol Invest 27:357–360

    CAS  PubMed  Google Scholar 

  19. Langerman A, Schneider JA, Ward RP (2004) Pheochromocytoma storm presenting as cardiovascular collapse at term pregnancy. Rev Cardiovasc Med 5:226–230

    PubMed  Google Scholar 

  20. Frayssinet C, Vezzosi D, Huyghe E, Lorenzini F, Bennet A, Caron P (2008) Retroperitoneal laparoscopic adrenalectomy in a pregnant woman presenting MEN2a with a pheochromocytoma: case report and review of the literature. Ann Endocrinol (Paris) 69:53–57. doi:10.1016/j.ando.2007.10.005

    CAS  Google Scholar 

  21. Quayle FJ, Fialkowski EA, Benveniste R, Moley JF (2007) Pheochromocytoma penetrance varies by RET mutation in MEN 2A. Surgery 142:800–805. doi:10.1016/j.surg.2007.09.013

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors thank Dr. Wanla Kulwichit and Professor Dr. Henry Wilde for invaluable input to this study. The study was supported by the Anandamahidol Research Fund of Division of Endocrine and Metabolism Division, Department of Internal Medicine, Chulalongkorn University, Ratchadapisaksompoch Research Fund of Chulalongkorn University and Thailand Research Fund.

Conflict of interest statement

The authors declare that we have no conflict of interest.

Author information

Authors and Affiliations

  1. Division of Endocrine and Metabolism, Department of Internal Medicine, Faculty of Medicine, Chulalongkorn University, Rama IV Road, Patumwan, Bangkok, 10330, Thailand

    Lalita Wattanachanya, Wanee Plengpanich, Natnicha Houngngam, Patinut Buranasupkajorn, Sarat Sunthornyothin & Thiti Snabboon

  2. Department of Internal Medicine, Faculty of Medicine, Chulalongkorn University, Rama IV Road, Patumwan, Bangkok, 10330, Thailand

    Udomsak Bunworasate

  3. Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

    Vorasuk Shotelersuk

Authors
  1. Lalita Wattanachanya
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Udomsak Bunworasate
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Wanee Plengpanich
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Natnicha Houngngam
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Patinut Buranasupkajorn
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Sarat Sunthornyothin
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Vorasuk Shotelersuk
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Thiti Snabboon
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Thiti Snabboon.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wattanachanya, L., Bunworasate, U., Plengpanich, W. et al. Bilateral pheochromocytoma during the postpartum period. Arch Gynecol Obstet 280, 1055–1058 (2009). https://doi.org/10.1007/s00404-009-1057-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00404-009-1057-5

Keywords

Search

Navigation