- University of Costa Rica School of Medicine, Ciudad Universitaria Rodrigo Facio, San Pedro de Montes de Oca, San José, Costa Rica
- Department of Neurological Surgery, Hospital San Juan de Dios, Paseo Colón, San José, Costa Rica
University of Costa Rica School of Medicine, Ciudad Universitaria Rodrigo Facio, San Pedro de Montes de Oca, San José, Costa Rica
Department of Neurological Surgery, Hospital San Juan de Dios, Paseo Colón, San José, Costa Rica
DOI:10.4103/2152-7806.125864Copyright: © 2014 Hernández-Durán S. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Silvia Hernández-Durán, Esteban Sánchez-Jiménez, José Pérez-Berríos. Hemangiopericytoma of the foramen magnum in a pregnant patient: A case report and literature review. Surg Neurol Int 28-Jan-2014;5:13
How to cite this URL: Silvia Hernández-Durán, Esteban Sánchez-Jiménez, José Pérez-Berríos. Hemangiopericytoma of the foramen magnum in a pregnant patient: A case report and literature review. Surg Neurol Int 28-Jan-2014;5:13. Available from: http://sni.wpengine.com/surgicalint_articles/hemangiopericytoma-of-the-foramen-magnum-in-a-pregnant-patient-a-case-report-and-literature-review/
Background:The presentation of intracranial hemangiopericytomas is very rare, and only one case of a hemangiopericytoma during pregnancy has been reported in the literature. The management of these lesions poses a great challenge to the neurosurgeon, since the physiological and hormonal changes of pregnancy can exacerbate the symptoms of this highly vascularized neoplasm and pose different risks to both the mother and the fetus. We report the case of a patient who had sudden onset of intracranial hypertension at the ninth week of gestation due to a hemangiopericytoma of the foramen magnum and review the literature in this regard.
Case Description:A 23-year-old female who presented with signs and symptoms of intracranial hypertension at the ninth week of gestation was initially thought to have hyperemesis gravidarum. Because her symptoms persisted, she was found to have intracranial hypertension due to a tumor in the foramen magnum. She was treated by means of derivative surgery to allow for her pregnancy to progress beyond the first trimester, and at the 22nd week of gestation she underwent a sub-occipital craniotomy with partial tumor removal. Pathology was consistent with hemangiopericytoma. Both the mother and the fetus had positive outcomes.
Conclusions:To our knowledge, this is the second intracranial hemangiopericytoma presenting during pregnancy to be reported in the literature, and it is the first one of its kind to be located in the foramen magnum and causing severe intracranial hypertension.
Keywords: Hemangiopericytoma, hyperemesis gravidarum, intracranial hypertension, intracranial neoplasms, pregnancy complications
The presentation of intracranial tumors during pregnancy is rare, and international data estimate an incidence of approximately 15 in 100,000.[
A 23-year-old female patient, with no known past medical history, was referred to our centre at the 9th week of her first pregnancy because of incoercible vomiting and mild dehydration. She was admitted to the Obstetrics department, where she received symptomatic treatment with antiemetics and prokinetics. She was discharged on the third day with the diagnosis of hyperemesis gravidarum. Nevertheless, 2 weeks later, the patient was readmitted for presenting incoercible vomiting, dizziness, and headaches. On this occasion, vitamin B6 was added to her treatment, and she was discharged on the fifth day.
On the 16th week of gestation, the patient was readmitted because she persisted with the symptoms. At this time, the patient was obtunded and disoriented, and she also complained of an oppressive occipital headache. On physical examination, left VI cranial nerve palsy, papilledema, and right ankle clonus were found. Brain magnetic resonance imaging (MRI) was performed, suspecting a dural sinus thrombosis. However, the study revealed triventricular hydrocephalus with transependymal edema due to a tumor in the posterior fossa [Figures
The patient was assessed by the authors, and she underwent an urgent ventriculoperitoneal shunt with a high pressure valve without any complications. In spite of a favorable course in the immediate postoperatory period, the patient experienced neurological decline with blurry vision, vomiting and headaches 10 days later. These symptoms did not respond to medical treatment with dexamethasone. In view of the latter, an endoscopic third ventriculostomy was performed, and her derivative system was removed. At the same time, we discussed with the Obstetrics department the need to operate on the mass, since the patient showed progressive involvement of the brainstem.
At 22 weeks of gestation, the patient underwent a right sub-occipital craniotomy with partial resection of a highly vascularized mass in the posterior margin of the foramen magnum. Its intraoperative biopsy reported a low-grade stromal tumor, suggestive of hemangioblastoma. The surgery was performed without any obstetric or neurologic complications. In the postoperatory period, the patient only presented mild right hemiparesis, which remitted within a few days. The definitive biopsy showed the presence of a vascular neoplasm with irregular “stag-horn” cells, and positive immunohistochemistry for CD34 and vimentin. All of the latter was compatible with hemangiopericytoma. The patient continued to be followed at the Neurosurgery and Obstetric outpatient clinics after the 26th week of gestation. She underwent a cesarean section at the 36th week because of intrauterine growth restriction, and it was performed without any complications. Currently, both the mother and the baby are in good health.
Intracranial tumors are a relatively rare complication in the setting of pregnancy. Glial neoplasms are the most common, followed by meningiomas and acoustic neuromas, all of which have similar frequencies to nonpregnant controls.[
One of the challenges faced by physicians when diagnosing intracranial tumors during pregnancy is that the symptoms of intracranial hypertension, such as nausea and vomiting, can be mistaken for pregnancy-related issues, such as hyperemesis gravidarum.[
To date, only one case of intracranial hemangiopericytoma during pregnancy has been reported in the literature.[
Unlike the previous case, our patient presented symptoms at an earlier gestational age, and she suffered progressive neurological decline with intracranial hypertension and involvement of the brainstem. The latter was, to a large extent, due to the location of her tumor in the foramen magnum. Therefore, she required a more aggressive management, including a sub-occipital craniotomy at the 22nd week of gestation. Currently, there are no guidelines for the management of intracranial tumors in pregnant patients, because the available evidence is limited to case reports or small patient series.[
In 2009, Ng and Kitchen created an algorithm to guide the management of pregnant patients with intracranial tumors.[
In our case, two surgical interventions, namely the ventriculoperitoneal shunt and the third ventriculostomy, were performed in order to treat her intracranial hypertension and allow for her pregnancy to advance. The goal was to avoid exposing the mother and the fetus to the anesthetic and surgical risks of a craniotomy. These measures allowed for the pregnancy to progress until the second trimester, which is when the mass was surgically treated, without further complications. Thus, greater neurological decline of the mother was prevented, and the pregnancy progressed until the birth of a healthy preterm infant. This course coincides with the one observed by Terry et al.[
Much has been discussed about the correlation between hormonal factors and the development of intracranial tumors. Specifically, it has been noted that the incidence of meningiomas is twice as high in women as in men, while gliomas are 1.5 times more frequent in men.[
Hemangiopericytomas have not been studied as comprehensively, for they are a rare histological type of intracranial tumors. However, molecular studies have shown that these neoplasms strongly express receptors for the placenta-induced growth factor (PIGF) and the vascular endothelial growth factor (VEGF) 1.[
In conclusion, our case evinces the complexity of managing intracranial tumors during pregnancy. Protean changes in the hormonal and physiological milieus inherent to the pregnant state can favor the manifestation of these neoplasms, be it through tumor growth itself or through the hemodynamic changes of pregnancy. Therefore, it is very important to provide early care. Since evidence is controversial, and since there are no validated guidelines, it is extremely important for an interdisciplinary team of neurosurgeons and obstetricians to approach each case individually, in order to achieve the best results for the mother and the child.
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