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Concurrent bilateral pheochromocytoma and thoracic paraganglioma during pregnancy

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Abstract

Although hypertension occurring during pregnancies is not uncommon and its prognosis is generally excellent, some of its unusual causes can lead to catastrophic consequences, especially in undiagnosed cases. Here, we report a pregnant woman who presented with hypertension in her early pregnancy. It was subsequently found to be caused by bilateral pheochromocytoma. After removal of both tumors, catecholamine levels unexpectedly and unexplainably remained elevated. At 23 weeks of gestation, the fetus was found dead in utero. After the fetal death, additional studies were performed and revealed a thoracic paraganglioma. To our knowledge, this is the first report of a case of three catecholamine-producing tumors occurring concurrently during a pregnancy. Genetic analysis helped identify this unprecedented condition; the patient harbored a heterozygous missense mutation c.482G>A in exon 3 of the VHL gene, indicating von Hippel-Lindau syndrome. Physicians who care for hypertensive pregnant patients should be aware of this condition as its diagnosis would probably lead to a better outcome.

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Acknowledgments

The authors thank Dr. Apirak Santingamkun and Professor Dr. Henry Wilde for invaluable input to this study. This study was supported by grants from the Hormone Research Fund, Anandamahidol Foundation under the Royal Patronage of His Majesty the King of Thailand.and Ratchadapisaksompoch Fund of Chulalongkorn University, and Thailand Research Fund.

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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

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

  2. Department of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand

    Nattachet Plengvidhya

  3. Departments of Obstetrics and Gynecology, Chulalongkorn University, Bangkok, Thailand

    Wisan Sereepapong

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

    Vorasuk Shotelersuk

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  1. Thiti Snabboon
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  2. Wanee Plengpanich
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  3. Natnicha Houngngam
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  4. Patinut Buranasupkajorn
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  6. Wisan Sereepapong
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  7. Sarat Sunthornyothin
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  8. Vorasuk Shotelersuk
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Correspondence to Thiti Snabboon.

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Snabboon, T., Plengpanich, W., Houngngam, N. et al. Concurrent bilateral pheochromocytoma and thoracic paraganglioma during pregnancy. Endocr 37, 261–264 (2010). https://doi.org/10.1007/s12020-009-9292-x

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  • DOI: https://doi.org/10.1007/s12020-009-9292-x

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