Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease

C. Frantzen; R.C. Kruizinga; S.J. Asselt; B.A. Zonnenberg; J.W. Lenders; W.W. de Herder; A.M.E. Walenkamp; R.H. Giles; F.J Hes; W.J. Sluiter; M.G. van Pampus; T.P. Links

doi:10.1212/WNL.0b013e3182661f3c

Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease

C. Frantzen, R.C. Kruizinga, S.J. Asselt, B.A. Zonnenberg, J.W. Lenders, W.W. de Herder, A.M.E. Walenkamp, R.H. Giles, F.J Hes, W.J. Sluiter, M.G. van Pampus, T.P. Links

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

OBJECTIVE: We studied the reciprocal effect of pregnancy and von Hippel-Lindau (VHL) disease by analyzing the influence of pregnancy on VHL disease-related lesions and VHL disease on pregnancy outcome.

METHODS: Medical charts and imaging reports from the VHL disease expertise centers in the Netherlands were used to retrospectively assess lesion progression score before and after pregnancy and to obtain data on pregnancy outcome and VHL disease-related lesions. The Friedman test was used for analysis (p ≤ 0.05). Twenty-nine patients were studied (48 pregnancies, 49 newborns).

RESULTS: The progression score of cerebellar hemangioblastomas significantly changed between the single MRI scan before and the 2 scans after pregnancy (p = 0.049) (n = 12). Fetal mortality rate was 2% (n = 1) caused by maternal pheochromocytoma. Maternal VHL disease-related complications occurred in 17% (n = 8) of all pregnancies. In 4 patients, a life-threatening situation emerged: hydrocephalus due to cerebellar hemangioblastoma (n = 2) and pheochromocytoma (n = 2).

CONCLUSIONS: Pregnancy in patients with VHL disease induces cerebellar hemangioblastoma progression and causes a high VHL disease-related pregnancy complication rate. We recommend intensified surveillance of patients with VHL disease, especially of cerebellar hemangioblastomas during preconception care and pregnancy.

Original language	English
Pages (from-to)	793-796
Number of pages	4
Journal	Neurology
Volume	79
Issue number	8
DOIs	https://doi.org/10.1212/WNL.0b013e3182661f3c
Publication status	Published - 21 Aug 2012

Keywords

Adrenal Gland Neoplasms
Adult
Cerebellar Neoplasms
Disease Progression
Female
Hemangioblastoma
Humans
Hydrocephalus
Magnetic Resonance Imaging
Neuroimaging
Pheochromocytoma
Pregnancy
Pregnancy Complications, Neoplastic
Retrospective Studies
von Hippel-Lindau Disease

Access to Document

10.1212/WNL.0b013e3182661f3c

Cite this

Frantzen, C., Kruizinga, R. C., Asselt, S. J., Zonnenberg, B. A., Lenders, J. W., de Herder, W. W., Walenkamp, A. M. E., Giles, R. H., Hes, F. J., Sluiter, W. J., van Pampus, M. G., & Links, T. P. (2012). Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease. Neurology, 79(8), 793-796. https://doi.org/10.1212/WNL.0b013e3182661f3c

@article{3542f6dda0114c08866b7b218c19bf3e,

title = "Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease",

abstract = "OBJECTIVE: We studied the reciprocal effect of pregnancy and von Hippel-Lindau (VHL) disease by analyzing the influence of pregnancy on VHL disease-related lesions and VHL disease on pregnancy outcome.METHODS: Medical charts and imaging reports from the VHL disease expertise centers in the Netherlands were used to retrospectively assess lesion progression score before and after pregnancy and to obtain data on pregnancy outcome and VHL disease-related lesions. The Friedman test was used for analysis (p ≤ 0.05). Twenty-nine patients were studied (48 pregnancies, 49 newborns).RESULTS: The progression score of cerebellar hemangioblastomas significantly changed between the single MRI scan before and the 2 scans after pregnancy (p = 0.049) (n = 12). Fetal mortality rate was 2% (n = 1) caused by maternal pheochromocytoma. Maternal VHL disease-related complications occurred in 17% (n = 8) of all pregnancies. In 4 patients, a life-threatening situation emerged: hydrocephalus due to cerebellar hemangioblastoma (n = 2) and pheochromocytoma (n = 2).CONCLUSIONS: Pregnancy in patients with VHL disease induces cerebellar hemangioblastoma progression and causes a high VHL disease-related pregnancy complication rate. We recommend intensified surveillance of patients with VHL disease, especially of cerebellar hemangioblastomas during preconception care and pregnancy.",

keywords = "Adrenal Gland Neoplasms, Adult, Cerebellar Neoplasms, Disease Progression, Female, Hemangioblastoma, Humans, Hydrocephalus, Magnetic Resonance Imaging, Neuroimaging, Pheochromocytoma, Pregnancy, Pregnancy Complications, Neoplastic, Retrospective Studies, von Hippel-Lindau Disease",

author = "C. Frantzen and R.C. Kruizinga and S.J. Asselt and B.A. Zonnenberg and J.W. Lenders and {de Herder}, W.W. and A.M.E. Walenkamp and R.H. Giles and F.J Hes and W.J. Sluiter and {van Pampus}, M.G. and T.P. Links",

year = "2012",

month = aug,

day = "21",

doi = "10.1212/WNL.0b013e3182661f3c",

language = "English",

volume = "79",

pages = "793--796",

journal = "Neurology",

issn = "0028-3878",

publisher = "Lippincott Williams & Wilkins",

number = "8",

}

TY - JOUR

T1 - Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease

AU - Frantzen, C.

AU - Kruizinga, R.C.

AU - Asselt, S.J.

AU - Zonnenberg, B.A.

AU - Lenders, J.W.

AU - de Herder, W.W.

AU - Walenkamp, A.M.E.

AU - Giles, R.H.

AU - Hes, F.J

AU - Sluiter, W.J.

AU - van Pampus, M.G.

AU - Links, T.P.

PY - 2012/8/21

Y1 - 2012/8/21

N2 - OBJECTIVE: We studied the reciprocal effect of pregnancy and von Hippel-Lindau (VHL) disease by analyzing the influence of pregnancy on VHL disease-related lesions and VHL disease on pregnancy outcome.METHODS: Medical charts and imaging reports from the VHL disease expertise centers in the Netherlands were used to retrospectively assess lesion progression score before and after pregnancy and to obtain data on pregnancy outcome and VHL disease-related lesions. The Friedman test was used for analysis (p ≤ 0.05). Twenty-nine patients were studied (48 pregnancies, 49 newborns).RESULTS: The progression score of cerebellar hemangioblastomas significantly changed between the single MRI scan before and the 2 scans after pregnancy (p = 0.049) (n = 12). Fetal mortality rate was 2% (n = 1) caused by maternal pheochromocytoma. Maternal VHL disease-related complications occurred in 17% (n = 8) of all pregnancies. In 4 patients, a life-threatening situation emerged: hydrocephalus due to cerebellar hemangioblastoma (n = 2) and pheochromocytoma (n = 2).CONCLUSIONS: Pregnancy in patients with VHL disease induces cerebellar hemangioblastoma progression and causes a high VHL disease-related pregnancy complication rate. We recommend intensified surveillance of patients with VHL disease, especially of cerebellar hemangioblastomas during preconception care and pregnancy.

AB - OBJECTIVE: We studied the reciprocal effect of pregnancy and von Hippel-Lindau (VHL) disease by analyzing the influence of pregnancy on VHL disease-related lesions and VHL disease on pregnancy outcome.METHODS: Medical charts and imaging reports from the VHL disease expertise centers in the Netherlands were used to retrospectively assess lesion progression score before and after pregnancy and to obtain data on pregnancy outcome and VHL disease-related lesions. The Friedman test was used for analysis (p ≤ 0.05). Twenty-nine patients were studied (48 pregnancies, 49 newborns).RESULTS: The progression score of cerebellar hemangioblastomas significantly changed between the single MRI scan before and the 2 scans after pregnancy (p = 0.049) (n = 12). Fetal mortality rate was 2% (n = 1) caused by maternal pheochromocytoma. Maternal VHL disease-related complications occurred in 17% (n = 8) of all pregnancies. In 4 patients, a life-threatening situation emerged: hydrocephalus due to cerebellar hemangioblastoma (n = 2) and pheochromocytoma (n = 2).CONCLUSIONS: Pregnancy in patients with VHL disease induces cerebellar hemangioblastoma progression and causes a high VHL disease-related pregnancy complication rate. We recommend intensified surveillance of patients with VHL disease, especially of cerebellar hemangioblastomas during preconception care and pregnancy.

KW - Adrenal Gland Neoplasms

KW - Adult

KW - Cerebellar Neoplasms

KW - Disease Progression

KW - Female

KW - Hemangioblastoma

KW - Humans

KW - Hydrocephalus

KW - Magnetic Resonance Imaging

KW - Neuroimaging

KW - Pheochromocytoma

KW - Pregnancy

KW - Pregnancy Complications, Neoplastic

KW - Retrospective Studies

KW - von Hippel-Lindau Disease

U2 - 10.1212/WNL.0b013e3182661f3c

DO - 10.1212/WNL.0b013e3182661f3c

M3 - Article

C2 - 22875085

SN - 0028-3878

VL - 79

SP - 793

EP - 796

JO - Neurology

JF - Neurology

IS - 8

ER -

Pregnancy-related hemangioblastoma progression and complications in von Hippel-Lindau disease

Abstract

Keywords

Access to Document

Fingerprint

Cite this