- Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Ryusuke Hatae, Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
DOI:10.25259/SNI_422_2022Copyright: © 2022 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, transform, 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: Ryusuke Hatae1, Masahiro Mizoguchi1, Koichi Arimura1, Daisuke Kiyozawa2, Takafumi Shimogawa1, Yuhei Sangatsuda1, Ataru Nishimura1, Kotaro Ono1, Yoshinao Oda2, Koji Yoshimoto1. Giant cranial angiolipoma with arteriovenous fistula: A case report. 22-Jul-2022;13:314
How to cite this URL: Ryusuke Hatae1, Masahiro Mizoguchi1, Koichi Arimura1, Daisuke Kiyozawa2, Takafumi Shimogawa1, Yuhei Sangatsuda1, Ataru Nishimura1, Kotaro Ono1, Yoshinao Oda2, Koji Yoshimoto1. Giant cranial angiolipoma with arteriovenous fistula: A case report. 22-Jul-2022;13:314. Available from: https://surgicalneurologyint.com/surgicalint-articles/11736/
Background: Angiolipomas are benign mesenchymal tumors comprising mature adipocytes and abnormal blood vessels, commonly found in the subcutaneous tissue of the trunk and rarely in the skull. Furthermore, sporadic cases of angiolipoma with arteriovenous fistula (AVF) have been reported.
Case Description: We reported the case of a 72-year-old woman who presented with head swelling, seizures, and cognitive dysfunction. Computed tomography and magnetic resonance imaging revealed a right frontal bone tumor exceeding a sagittal suture of up to 10.7 cm. Angiography revealed AVF and varices formation. Endovascular embolization was performed to treat the AVF and reduce blood loss during surgical resection. Two days after the embolization, a craniotomy was performed; however, uncontrollable bleeding was observed at the time of tumor resection. Postoperatively, the patient was symptom-free and has been stable for 2 years without recurrence.
Conclusion: Despite careful preoperative evaluation and treatment planning, the patient in this case report was difficult to treat. Such cases require adequate preparation.
Keywords: Angiolipomas, Arteriovenous fistula, Epilepsy, Hemorrhage, Skull tumor
Angiolipoma is a slow-growing benign tumor that commonly occurs in subcutaneous tissues.[
A 72-year-old woman had a mass in the right frontal region for 3 years. Due to the appearance of unsteadiness when walking, a magnetic resonance imaging (MRI) was performed 6 months previously, and she was diagnosed with frontal cranial tumor. Two months earlier, the patient experienced left hemiconvulsive seizures with impaired consciousness and was started on anticonvulsant medication. Subsequently, she was referred to our hospital for treatment. She had no neurological symptoms on examination but had mild cognitive dysfunction [
Computed tomography (CT) scans on admission. (a and b) CT scan with bone window demonstrates a large lesion in the right frontoparietal cranium expanding into the diploic space and exerting a mass effect on the right frontal lobe. (c and d) Perfusion CT shows that cerebral blood flow (c) and cerebral blood volume (d) are increased in the right frontal mass. (e) The venous phase of four-dimensional CT angiography displays occluded superior sagittal sinus.
Magnetic resonance imaging (MRI) on admission. (a and b) Both T1- (a) and T2-weighted MRI (b) display high-intensity mass with flow void. The right frontal lobe is compressed by the mass, causing a midline shift. (c) T1-weighted fat-suppressed MRI with contrast shows a mass with heterogeneous enhancement. Fat suppression reveals low signal in most of the mass, suggesting the presence of adipose tissue in the mass. (d–f) T1-weighted fat-suppressed MRI with contrast displays large cranial tumor with superior sagittal sinus (SSS) obstruction.
Cerebral angiogram on admission and after preoperative embolization. (a) The arterial phase of the right external carotid angiography shows a marked tumor staining from the right middle meningeal artery. Notably, dilated blood vessels are noted. (b) The venous phase of right external carotid angiography shows varices and congestion of contrast medium. (c) Right external carotid artery angiography after endovascular embolization confirms no residual hypervascular mass and arteriovenous shunt.
Preoperative endovascular embolization was performed with N-butyl-2-cyanoacrylate (NBCA) and particles through feeding arteries. First, we introduced the microcatheter to the anterior branch of the right MMA and DTA and injected the NBCA diluted with contrast medium to 16.7–20.0%. Next, the MMA main trunk was also embolized with Embosphere® and fibered coils. Finally, the tumor stain was dramatically reduced after embolization [
Postoperative computed tomography (CT) scan. CT scans before (a and b) and after (c and d) placement of the titanium plate are provided. (a and b) A large craniotomy is shown. Compression on the brain is greatly improved. (c and d) The skull defect is reconstructed with a vast mesh plate, and there is no brain compression.
Histological examination revealed that the intracranial tumor was composed of mature adipocytes with various-sized dilated vessels [
Hematoxylin and eosin (H&E) staining of the specimens with different magnifications. (a) Loupe image shows that tumors comprising various-sized blood vessels and fat are enlarged under the cortical bone. (b) A high-power image displays that multiple blood vessels are interspersed in mature adipose tissue (H&E, original magnification, ×20). (c) Pathology photograph of aneurysm-forming vessels is provided. The elastic plate and smooth muscle of the tunica media are not clear, consistent with varices. (H&E, original magnification, ×2). (d) A high-power image reveals mild inflammatory cell infiltration in the outer membrane (H&E, original magnification, ×10).
Angiolipoma is a benign tumor that accounts for 5~17% of all lipomas and is found predominantly in the subcutaneous and intramuscular regions of the peripheral extremities.[
Despite successful preoperative embolization, uncontrollable bleeding was observed at the time of tumor resection in this case. There are two possible reasons for this hemorrhage: (1) the diploic vein, originally the main drainer, was cut during craniotomy and (2) the return of blood flow to the venous system of the SSS and dura mater, chronically compressed and rendered fragile by the tumor, leading to bleeding. Ohigashi and Tanabe reported a case of massive bleeding immediately after craniotomy during the resection of a meningioma having abundant diploic veins as the drainers.[
As shown in
Fortunately, we could achieve gross total resection of the large skull tumor without any neurological deterioration, although we experienced severe hemorrhage during tumor resection. In general, intraoperative hemorrhage is considered to be easily controlled with en bloc resection of the tumor. However, preoperative embolization should be considered to avoid severe hemorrhage, especially for patients whose en bloc resection of the tumor is difficult to maintain the surrounding structure. Furthermore, for cases of skull tumors with a significantly high risk of resection, multidrug therapy for epilepsy or radiotherapy may be an alternative.
We reported a case of resection of a giant, extra-median angiolipoma with the onset of seizures. This is the sixth case of angiolipoma of the skull and the first case worldwide presenting with seizure symptoms, accompanied by an AVF, and found beyond the sagittal suture. Cognitive dysfunction and epilepsy improved after the resection of this large skull tumor; therefore, symptomatic skull tumors, such as this case, are expected to improve with surgery. Since we had a tough time with the removal of the tumor, adequate preparation is necessary for similar cases.
The authors certify that they have obtained all appropriate patient consent.
There are no conflicts of interest.
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