| Pheochromocytoma and Von Hippel-Lindau in pregnancy. | |
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MedLine Citation:
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PMID: 19784914 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Pheochromocytoma is an infrequent but well-acknowledged primary cause of malignant hypertension in pregnancy. Although the majority of pheochromocytomas are sporadic, those that present as bilateral or multifocal tumors may be a manifestation of a rare cancer susceptibility syndrome, such as Von Hippel-Lindau (VHL). Gravidae with unrecognized pheochromocytoma are at risk for recurrent paroxysmal hypertensive crises with ensuant maternal and fetal risks. To further illustrate the challenges of management of pheochromocytoma and VHL in pregnancy, we present two illustrative cases. In the first, a multigravida presented with an intrauterine fetal demise and malignant hypertension and a concurrent diagnosis of bilateral pheochromocytomas. A missense mutation in exon 3 of the VHL gene was identified, confirming the diagnosis of VHL type 2C. In the second case, a multigravida with a prior diagnosis of VHL syndrome but sporadic follow-up underwent renal and adrenal imaging surveillance as part of her prenatal care. Although she was normotensive and clinically asymptomatic, such imaging enabled the detection of bilateral pheochromocytomas. In summary, in this report we discuss our management in gravidae with pheochromocytoma and VHL, emphasizing current recommendations pertaining to obstetric management, genetic testing, and long-term follow-up. |
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Authors:
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Nonna Kolomeyevskaya; Maria Blazo; Ignatia Van den Veyver; Stacy Strehlow; Kjersti M Aagaard-Tillery |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2009-09-26 |
Journal Detail:
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Title: American journal of perinatology Volume: 27 ISSN: 1098-8785 ISO Abbreviation: Am J Perinatol Publication Date: 2010 Mar |
Date Detail:
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Created Date: 2010-02-22 Completed Date: 2010-05-20 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8405212 Medline TA: Am J Perinatol Country: United States |
Other Details:
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Languages: eng Pagination: 257-63 Citation Subset: IM |
Copyright Information:
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Thieme Medical Publishers. |
Affiliation:
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Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas 77030, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adrenal Gland Neoplasms
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complications*,
diagnosis,
genetics Adult Female Humans Hypertension, Pregnancy-Induced / diagnosis, genetics Pheochromocytoma / complications*, diagnosis, genetics Pregnancy Pregnancy Complications, Neoplastic / diagnosis*, genetics Prenatal Diagnosis / methods Risk Factors Young Adult von Hippel-Lindau Disease / complications*, diagnosis, genetics |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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