Case Reports in Orthopedics
Volume 2021 (2021), Article ID 6651160, 5 pages
https://doi.org/10.1155/2021/6651160

Case Report

Angiomatous Meningioma in the Craniocervical Junction

Shunsuke Ito,¹ Yoichi Iizuka,¹ Masanori Aihara,² Hiromi Koshi,³ Tokue Mieda,¹ Eiji Takasawa,¹ Sho Ishiwata,¹ Yusuke Tomomatsu,¹ Akira Honda,¹ Kazuhiro Inomata,¹ and Hirotaka Chikuda¹

¹Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma 371-8511, Japan
²Department of Neurosurgery, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma 371-8511, Japan
³Clinical Department of Pathology, Gunma University Hospital, 3-39-22, Showa, Maebashi, Gunma 371-8511, Japan

Correspondence should be addressed to Shunsuke Ito; shunsuke-ito@gunma-u.ac.jp

Received 18 December 2020; Revised 28 March 2021; Accepted 1 April 2021; Published 12 April 2021

Academic Editor: Da sen Li

Copyright © 2021 Shunsuke Ito et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract.

Introduction. Spinal angiomatous meningioma arising in the craniocervical junction has not been reported. Case Presentation. A 68-year-old man presented to our hospital with pain in the back and left leg. He showed slight motor weakness in his upper extremities. Magnetic resonance imaging revealed a mass with marked enhancement in the craniocervical junction. Computed tomography angiography showed feeding vessels arising from the right vertebral artery. Preoperative embolization of the feeding vessels was performed to reduce intraoperative bleeding. Gross total resection of the tumor was achieved by debulking and piecemeal resection. The tumor attachment to the dura mater was also resected (Simpson grade 1 resection). A histopathological examination confirmed the diagnosis of an angiomatous meningioma. The patient’s symptoms improved shortly after surgery. Conclusions. We achieved gross total resection of spinal angiomatous meningioma arising in the craniocervical junction. A preoperative evaluation and embolization of the feeding arteries may help prevent massive intraoperative bleeding.

1. Introduction

Angiomatous meningioma, characterized by an abundant vascular component, is a rare subtype of meningioma, accounting for 2.1% of meningiomas [1]. Angiomatous meningiomas arising from the spinal cord is even rarer. We herein report a case of angiomatous meningioma in the craniocervical junction.

2. Case Presentation

A 68-year-old man without notable comorbidity presented to our hospital with a 15-month history of pain in the back and left leg. A neurological examination revealed motor weakness (manual muscle testing 4 out of 5) in the upper extremities. Magnetic resonance imaging (MRI) demonstrated an intradural mass lesion at the craniocervical junction, which has low intensity on both T1- and T2-weighted imaging. Enhanced MRI with gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA) showed homogeneous enhancement without a dural tail sign (Figure 1). Computed tomography angiography using iodinated contrast agent revealed two feeding vessels arising from the right vertebral artery.

(a)

(b)

(c)

Figure 1: (a) T2-weighted sagittal MRI of the cervical spine showed a tumor in the craniocervical junction. (b, c) MRI followed by the intravenous administration of gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA) showed a homogeneously enhanced intradural tumor without obvious dural tail sign.

Given the aggravating symptoms, the patient decided to undergo surgery. To reduce the risk of massive bleeding, we embolized the feeding arteries using soft coils prior to surgery. An angiogram after embolization showed a decreased tumor stain (Figure 2). Subsequently, resection of the tumor was performed the same day. Following durotomy, a tumor mass was identified on the ventrolateral side of the spinal cord. Resection was carefully performed via central debulking with piecemeal resection to avoid neurological damage under spinal monitoring (Figure 3). We observed substantial but manageable bleeding from inside of the tumor. The tumor attachment to the dura mater was also resected (Simpson grade 1 resection). The dural defect was repaired with a Gore-Tex patch and further covered with a bioabsorbable polyglycolic acid sheet and fibrin glue. The intraoperative blood loss, mainly due to bleeding from the tumor itself, was 567 ml. The drain was removed two days after surgery without noticeable cerebrospinal fluid leakage.

(a)

(b)

Figure 2: (a) Right vertebral angiogram demonstrating tumor staining, supplied by the right C2 (arrow) and C3 (arrowhead) segmental arteries. (b) A right vertebral angiogram after embolization using a coil showing slight tumor staining.

(a)

(b)

Figure 3: (a) A mass was identified after durotomy (arrowhead). (b) The tumor was resected in a piecemeal fashion.

A histopathological examination showed numerous blood vessels making up most of the mass and intervening tumor cells. The blood vessels were variably hyalinized and thick-walled. The tumor cells had round nuclei with occasional intranuclear pseudoinclusions. Some whorls and psammoma bodies were present (Figure 4). Immunohistochemically, the tumor cells were positive for epithelial membrane antigen, and the MIB-1 index was <1%. The tumor was diagnosed as an angiomatous meningioma.

Figure 4: The resected tumor was composed of numerous blood vessels and intervening tumor cells. The blood vessels were variably hyalinized and thick-walled.

The patient’s symptoms, including back pain and leg pain, improved shortly after surgery. MRI obtained one year after surgery revealed decompression of the spinal cord and no sign of recurrence (Figure 5). The patient remained neurologically intact until he died of an unrelated cause 16 months after surgery.

(a)

(b)

Figure 5: (a, b) T2-weighted sagittal and axial MRI at one year after surgery showed no obvious recurrence.

3. Discussion

Spinal angiomatous meningioma is extremely rare. To our knowledge, a total of 16 cases have been reported (Table 1) [2–6]. Angiomatous meningiomas are classified as WHO grade 1 meningioma without aggressive nature, while motor and/or sensory deficits and bladder and rectal disturbance can occur as the disease progresses. Spinal angiomatous meningiomas arise most commonly in the thoracic spine followed by the cervical spine [2–6]. The present case is the first report of angiomatous meningioma in the craniocervical junction.

Table 1: Summary of 17 cases of angiomatous meningioma of the spinal cord and the present case.

Authors
Age
Sex
Location
Treatment
Dural attachment
Blood loss
Follow-up
Outcome

Gul et al. 2008 [2]
25
F
T9-L2
GTR
NA
NA
NA
NA

Vij et al. 2012 [3]
16
M
C2-C5
GTR; piecemeal resection
NA
NA
3 d
Dead

Wu et al. 2015 [4]
28
M
C2-C3
Simpson grade II; piecemeal resection
Ventral
400
152 m
No recurrence

56
F
C1-C3
GTR; en bloc resection
Nerve root
100
131 m
No recurrence

54
F
T10-T11
Simpson grade II; piecemeal resection
Ventral
500
120 m
No recurrence

49
M
T1-T3
Simpson grade I; en bloc resection
Dorsal
200
108 m
No recurrence

43
F
T1
GTR; en bloc resection
Nerve root
100
99 m
No recurrence

28
F
C2-C4
GTR; en bloc resection
Nerve root
100
86 m
No recurrence

67
F
C7-T3
Simpson grade II; en bloc resection
Lateral
300
72 m
No recurrence

76
F
T4
Simpson grade II; en bloc resection
Lateral
100
61 m
No recurrence

41
M
T11-T12
Simpson grade II; en bloc resection
Lateral
100
53 m
No recurrence

54
M
C1-C2
Simpson grade II; piecemeal resection
Ventral
600
32 m
No recurrence

57
M
T12-L1
Simpson grade II; piecemeal resection
Lateral
400
18 m
No recurrence

42
M
C2-C3
Simpson grade II; en bloc resection
Lateral
200
11 m
No recurrence

Yang et al. 2016 [5]
55
F
T6-T8
Simpson grade II; en bloc resection
Dorsolateral
NA
6 m
No recurrence

Missori et al. 2017 [6]
40
M
T3-T4
GTR
Nerve root
NA
14 m
No recurrence

Present case
68
M
O-C1
Simpson grade I; piecemeal resection
Ventrolateral
567
11 m
No recurrence

M: male; F: female; C: cervical; T: thoracic; L: lumbar; GTR: gross total resection; RT: radiotherapy; CT: chemotherapy; NA: not available; d: day; m: month.

The preferable treatment for angiomatous meningioma is Simpson grade 1 resection (total resection including dural attachment and abnormal bone) although some researchers suggested that Simpson grade 2 resection (total resection and dural coagulation) is sufficient [4, 7–10]. In the present case, we decided to perform Simpson grade 1 resection to reduce risk of potential recurrence. As angiomatous meningiomas are rich in small blood vessels, preoperative embolization to reduce tumor blood supply may be useful [11]. Contrarily, Wu et al. reported good control of intraoperative bleeding without using preoperative embolization. The authors found that en bloc resection was associated with a significant reduction of intraoperative blood loss compared with piece-by-piece resection [4]. In the present case, even though we successfully embolized the feeding arteritis just prior to the surgery, we still observed substantial intraregional bleeding.

When spinal angiomatous meningioma is suspected, we recommend performing a preoperative evaluation of the feeder vessels using an angiogram. Subsequent embolization of the feeder vessels should be discussed, especially when the tumor is located ventrally, making en bloc resection difficult.

Data Availability

There are no available data supporting the results of this study as it is a case report.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

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