- Department of Neurosurgery, All India Institute of Medical Science, Raipur, Chhattisgarh, India.
DOI:10.25259/SNI_203_2020Copyright: © 2020 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Nitish Nayak, Anil Kumar. Cerebellar hemangioblastoma during pregnancy: Management options and review of literature. 23-May-2020;11:123
How to cite this URL: Nitish Nayak, Anil Kumar. Cerebellar hemangioblastoma during pregnancy: Management options and review of literature. 23-May-2020;11:123. Available from: https://surgicalneurologyint.com/surgicalint-articles/10041/
Background: Symptomatic cerebellar hemangioblastomas are extremely rare in pregnant women and the ideal management is not well established. In the present article, we aimed to report a case of large cerebellar hemangioblastoma complicated by pregnancy and managed successfully by surgical resection. In addition, we also discuss management options and review of the current literature pertaining to this pathology.
Case Description: A 22-year-old female presented with a history of headache and vomiting for 4 weeks. She was carrying 28 weeks of pregnancy. She had left cerebellar signs, gait ataxia, and bilateral six nerve paresis. Fundus examination revealed bilateral papilledema. She was diagnosed to have large cerebellar hemangioblastoma with mass effect and obstructive hydrocephalus. She underwent suboccipital craniotomy and excision of lesion in lateral position. She recovered well postoperatively and delivered a healthy baby in the full term. Imaging at10- month follow-up demonstrates no residual lesion or another hemangioblastoma.
Conclusion: Early diagnosis and direct surgery for excision of hemangioblastoma is a good option during pregnancy while avoiding CSF diversion procedures. The symptomatic hemangioblastoma during pregnancy can be safely operated during early pregnancy.
Keywords: Brain tumor, Cerebellar, Hemangioblastoma, Pregnancy
Cerebellar hemangioblastoma is a benign, relatively rare vascular lesion and can be sporadic or associated with von Hippel-Lindau disease. Symptomatic cerebellar hemangioblastomas are extremely rare in pregnant women and the ideal management is not well established.[
A 22-year-old female presented with a history of headache and vomiting for 4 weeks. She was carrying 28 weeks of pregnancy (gravida 2, para 1). Her medical history was unremarkable. Neurologically, she was conscious and alert. She had left cerebellar signs, ataxic gait, and bilateral six nerve paresis. Fundus examination revealed bilateral Frisen Grade 4 papilledema and no retinal angioma was observed. The rest of the neurological examination was essentially within normal limits. Per abdominal examination showed a uterine size consistent with 28 weeks of gestation, normal fetal heart rate. Biochemical and hematological parameters were within standard limits. Magnetic resonance imaging revealed an intra-axial mass lesion of 5.4 × 4.5 × 3 cm dimension in the left cerebellar hemisphere, which was hypointense on T1-weighted image and hyperintense in T2-weighted image causing marked distortion of cerebellar hemisphere, with brain stem compression, pressure over the fourth ventricle with enlargement of both lateral ventricles, ballooning of third ventricles, and periventricular lucency [
(a) Contrast-enhancing magnetic resonance imaging revealed an intra-axial mass lesion of 5.4 × 4.5 × 3 cm dimension in the left cerebellar hemisphere, enhancing nodule with marked distortion of cerebellar hemisphere, with brain stem compression. (b) T2 axial image showing hyperintense lesion with perifocal edema around the tumor. (c) T1-weighted image showed enlargement of both lateral ventricles and ballooning of the third ventricles with periventricular lucency suggestive of hydrocephalus.
After obtaining the patient’s consent, a multidisciplinary team involving obstetricians, anesthesiologists, and neurosurgeons decided to perform surgical excision in view of raised intracranial pressure symptoms. She was considered for emergency surgery and underwent suboccipital craniotomy and resection of lesion in lateral position. Surgery revealed a tense dura and significant herniation of the left cerebellar hemisphere. Exploration of the cystic cavity revealed a vascular cherry red mural nodule that was resected from its insertion into the wall of the cystic capsule. At the end of the surgery, cerebellum was lax, pulsatile and the procedure was uneventful. The histopathological examination of the specimen confirmed hemangioblastoma. She recovered well postoperatively and had marked improvement in neurological status. The ultrasound examination of the fetus on the 10th postoperative day normal viable fetus. She delivered a healthy baby in the full term. She was doing well, at last follow-up at 10 months following surgery. A cranial contrast-enhanced MRI scan, at the last follow-up, revealed no residual lesion or another hemangioblastoma [
Postoperative magnetic resonance imaging (MRI) at 10 months follow-up. (a) T1-weighted contrast MRI showed postoperative changes with no enhancing nodule and no other hemangioblastoma. (b) T2 axial images showing postoperative changes and disappearance of edema. (c) T1 axial image showed resolution of hydrocephalus.
During pregnancy, cerebellar hemangioblastoma is rare and a patient may need urgent surgical intervention due to a rapid increase in size.[
Mechanism of increase in the size of the lesion during pregnancy
The effect of pregnancy on the natural history of hemangioblastomas remains unclear, though various theories have been proposed.[
Laviv et al. suggested the involvement of placental growth factor (PlGF) and its receptor vascular endothelial growth factor receptor 1 (VEGFR-1) can lead to the growth of peritumoral edema and cysts, which are the principal causes for the development of any symptoms in hemangioblastoma.[
Various management options have been described in the pertinent literature for cerebellar hemangioblastomas encountered during pregnancy which include conservative management with close observation, CSF diversion, and direct surgery. As these symptoms may get worse during pregnancy, so conservative management with close monitoring is not a feasible option in symptomatic patients. Some authors have recommended direct open surgery for a symptomatic hemangioblastoma during pregnancy, however, in high-risk pregnancies, particularly those at risk for preterm labor, symptomatic hemangioblastomas can be treated with more conservative approach-like CSF diversion.[
Naidoo et al. performed definitive surgery in two patients at 21 weeks and 33 weeks of gestation with good outcome.[
The severity of maternal symptoms and gestational age should be taken into account while deciding appropriate treatment. CSF diversion is an option, but the patient can deteriorate after shunt surgery.[
In view of overall management options, direct surgery is a better option if patients present in early pregnancy. It can avoid repeated exposure of general anesthesia and shunt- related complications (i.e., bleeding during placement of the catheter, infection, shunt malfunction, shunt exposure, and rare bowel perforation).[
This case highlights the significance of performing a detailed neurological examination in all cases of persisting headache and/or vomiting during pregnancy. As in the present case, the presence of lateral rectus palsies in any patient with a headache necessitates urgent imaging to rule out a space- occupying lesion. The case reported here is important because it suggests a direct surgical approach to the management of symptomatic cerebellar hemangioblastomas in pregnant women.
Cerebellar hemangioblastomas are extremely rare during pregnancy and should be included in differential diagnosis for those patients presented with persistent nausea and vomiting associated with focal neurological deficits. Early diagnosis and direct surgery for excision of hemangioblastoma is a good option during pregnancy while avoiding CSF diversion procedures. The symptomatic hemangioblastoma during pregnancy can be safely operated during early pregnancy. Further, case reports and series are required to support this recommendation.
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