Document Detail

Pheochromocytoma and Von Hippel-Lindau in pregnancy.
MedLine Citation:
PMID:  19784914     Owner:  NLM     Status:  MEDLINE    
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.
Nonna Kolomeyevskaya; Maria Blazo; Ignatia Van den Veyver; Stacy Strehlow; Kjersti M Aagaard-Tillery
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-09-26
Journal Detail:
Title:  American journal of perinatology     Volume:  27     ISSN:  1098-8785     ISO Abbreviation:  Am J Perinatol     Publication Date:  2010 Mar 
Date Detail:
Created Date:  2010-02-22     Completed Date:  2010-05-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8405212     Medline TA:  Am J Perinatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  257-63     Citation Subset:  IM    
Copyright Information:
Thieme Medical Publishers.
Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas 77030, USA.
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MeSH Terms
Adrenal Gland Neoplasms / complications*,  diagnosis,  genetics
Hypertension, Pregnancy-Induced / diagnosis,  genetics
Pheochromocytoma / complications*,  diagnosis,  genetics
Pregnancy Complications, Neoplastic / diagnosis*,  genetics
Prenatal Diagnosis / methods
Risk Factors
Young Adult
von Hippel-Lindau Disease / complications*,  diagnosis,  genetics

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