ABSTRACT
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.
KEYWORDS
Pheochromocytoma - Von Hippel–Lindau - pregnancy - secondary hypertension in pregnancy - renal hypertension
REFERENCES
-
1
Mannelli M.
Management and treatment of pheochromocytomas and paragangliomas.
Ann N Y Acad Sci.
2006;
1073
405-416
-
2
Phupong V, Witoonpanich P, Snabboon T, Tharavej C, Ultchaswadi P.
Bilateral pheochromocytoma during pregnancy.
Arch Gynecol Obstet.
2005;
271
276-279
-
3
Cruz J B, Fernandes L P, Clara S A et al..
Molecular analysis of the Von Hippel-Lindau (VHL) gene in a family with non-syndromic pheochromocytoma: the importance of genetic testing.
Arq Bras Endocrinol Metabol.
2007;
51
1463-1467
-
4
Ahlawat S K, Jain S, Kumari S, Varma S, Sharma B K.
Pheochromocytoma associated with pregnancy: case report and review of the literature.
Obstet Gynecol Surv.
1999;
54
728-737
-
5
Schenker J G, Granat M.
Phaeochromocytoma and pregnancy—an updated appraisal.
Aust N Z J Obstet Gynaecol.
1982;
22
1-10
-
6
Harper M A, Murnaghan G A, Kennedy L, Hadden D R, Atkinson A B.
Phaeochromocytoma in pregnancy. Five cases and a review of the literature.
Br J Obstet Gynaecol.
1989;
96
594-606
-
7
Botchan A, Hauser R, Kupfermine M, Grisaru D, Peyser M R, Lessing J B.
Pheochromocytoma in pregnancy: case report and review of the literature.
Obstet Gynecol Surv.
1995;
50
321-327
-
8
Lyman D J.
Paroxysmal hypertension, pheochromocytoma, and pregnancy.
J Am Board Fam Pract.
2002;
15
153-158
-
9
Manger W M.
The vagaries of pheochromocytomas.
Am J Hypertens.
2005;
18
1266-1270
-
10
Junglee N, Harries S E, Davies N, Scott-Coombes D, Scanlon M F, Rees D A.
Pheochromocytoma in pregnancy: when is operative intervention indicated?.
J Womens Health (Larchmt).
2007;
16
1362-1365
-
11
Kaelin W G.
Von Hippel-Lindau disease.
Annu Rev Pathol.
2007;
2
145-173
-
12
Kamari Y, Sharabi Y, Leiba A, Peleg E, Apter S, Grossman E.
Peripartum hypertension from pheochromocytoma: a rare and challenging entity.
Am J Hypertens.
2005;
18
1306-1312
-
13
Schreinemakers J MJ, Zonnenberg B A, Höppener J WM, Hes F J, Rinkes I H, Lips C JM.
A patient with bilateral pheochromocytoma as part of a Von Hippel-Lindau (VHL) syndrome type 2C.
World J Surg Oncol.
2007;
5
112
-
14
Neumann H PH, Bausch B, McWhinney S R Freiburg-Warsaw-Columbus Pheochromocytoma Study Group et al.
Germ-line mutations in nonsyndromic pheochromocytoma.
N Engl J Med.
2002;
346
1459-1466
-
15
Friedrich C A.
Von Hippel-Lindau syndrome. A pleomorphic condition.
Cancer.
1999;
86(11 suppl)
2478-2482
-
16
Erlic Z, Neumann H P.
Familial pheochromocytoma.
Hormones (Athens).
2009;
8
29-38
-
17
Fudge T L, McKinnon W M, Geary W L.
Current surgical management of pheochromocytoma during pregnancy.
Arch Surg.
1980;
115
1224-1225
-
18
Jessurun C R, Adam K, Moise Jr K J, Wilansky S.
Pheochromocytoma-induced myocardial infarction in pregnancy. A case report and literature review.
Tex Heart Inst J.
1993;
20
120-122
Kjersti M Aagaard-TilleryM.D. Ph.D.
Baylor College of Medicine, 1 Baylor Plaza
Jones 314 Houston, TX, 77030
Email: aagaardt@bcm.tmc.edu
