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CorrespondenCe

Introduction

Cavernous angioma (CA) is a type of vascular malformation affecting the central nervous system. It is estimated to occur in 0.4–0.5 % of the population, and comprising 5–13 % of all cerebrovascular malformations [1–3]. CA arising from the cranial dura mater, cranial dural cavernous angioma (CdCA), is an infrequent but distinct entity [4–38]. They have been reported mainly as case documentations or small series of less than five patients [1, 18, 20, 23]. In the clini-cal setting, CdCAs have frequently been misdiagnosed as meningiomas on presurgical neuroimaging examination [5, 8, 9, 11, 12, 14–16, 19–27, 29, 31, 32, 34–36, 38].

Case Report

A 43-year-old man, with a medical history of glaucoma, suf-fered persistent headache and was referred to our department for brain check-up. At presentation, neurological examina-tion found no abnormalities. Cranial computed tomography (CT) revealed a mostly isodense mass located superficially in the right cerebellar hemisphere. neither bony change in the adjacent skull nor intralesional calcification was noted (Fig. 1). Magnetic resonance imaging (MrI) demonstrated

a well-demarcated, dural-based extraaxial tumor, measur-ing 16 × 15 × 11 mm in dimension. The lesion appeared as hypointense on T1-weighted and as mixed intensity on

Clin neuroradioldoI 10.1007/s00062-013-0210-5

Cranial Dural Cavernous Angioma

S. Tsutsumi · Y. Yasumoto · H. Saeki · M. Ito

s. Tsutsumi, Md () · Y. Yasumoto, Md · M. Ito, Mddepartment of neurological surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba 279-0021, Japane-mail: shotaro@juntendo-urayasu.jp

H. saeki, Mddepartment of pathology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba 279-0021, Japan

received: 4 october 2012 / Accepted: 29 January 2013© springer-Verlag Berlin Heidelberg 2013

Fig. 1 Axial computed tomography scans without contrast medium at presentation showing an isodense mass located superficially in the right cerebellar hemisphere (a, arrows), with intact adjacent skull (b, arrowhead)

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T2-weighted Mr images with homogeneous enhancement after gadolinium infusion. perilesional hemosiderin depo-sition or concurrent vascular malformation was not found (Fig. 2). The patient underwent tumor resection, at his request, instead of periodical observation by neuroimaging examination. The lesion was reddish and had a mulberry-like appearance. Also, it was elastic hard and tightly adhered to the dura mater of the cerebellar convexity, while less vas-cular with minimal bleeding. The lesion was resected en bloc with the basal dura mater (Fig. 3). Histological exami-nation revealed that the tumor consisted of a collection of abnormally ectatic vessels arranged in a back-to-back man-ner. The vascular lumens were lined with a single layer of endothelium and filled with varying degrees of recent and remote intraluminal thrombi, with little intervening connec-tive tissue. The collections of ectatic vessels were poorly demarcated from the dura mater underneath, instead of appearing continuous with it. no atypia was seen (Fig. 4). Immunostaining for CD34 was confined to the endothelial layer, which was surrounded by an alpha smooth muscle actin (α-SMA) layer of varying thickness. These findings were consistent with the diagnosis of CdCA. The postop-erative course was uneventful.

Discussion

CdCAs can occur in any age [5–17, 19–22, 24–38], even in the pediatric population less than 15 years old [6, 11, 15, 24, 32, 34]. Clinical symptoms of CDCAs are nonspecific and can cause, depending on their locations in the cranial cavity, cranial nerve paresis [8, 10, 19, 20, 28, 36], headache [12, 15, 17, 29, 30, 37], seizure [16, 26, 31, 38], signs of increased intracranial pressure [5, 11, 32], cerebellar ataxia [9, 35], hemisensory disturbance [33], consciousness dis-turbance [13], confusion [22], syncope [21], and enlarging subcutaneous mass [27]. Exceptional cases were identified incidentally [7, 14], or as congenital tumors [24, 34]. A patient sustained nontraumatic acute subdural hemorrhage from a CdCA arose in the frontal dura mater [37].

The location of CdCAs is diverse. They arose from the parietal [18, 20, 26, 36, 38], frontal [12, 16, 25, 27, 37], frontoparietal [13, 33, 34], occipital [5, 15, 30], and tempo-ral convexity [18], anterior fossa [10], middle fossa [4, 8, 17, 21, 23, 28, 29, 31], sphenoid ridge [18], tentorial ridge [18], upper surface of the tentorium cerebelli [19], and falx cere-bri [4, 7]. CdCAs can also occur in the posterior fossa dura. They originated from the cerebellar convexity [11, 32, 35], falx cerebelli [14, 22], and lower surface of the tentorium cerebelli [24].

Fig. 2 preoperative magnetic resonance images demon-strating a well-demarcated, dural-based lesion (a–d, arrows), located extraaxially, measuring 16 × 15 × 11 mm, and appearing as hypointense on T1-weighted (a) and mixed intensity on T2-weighted (b) images with homogeneous enhancement after gadolinium infusion (c, d). no perilesional hemosiderin deposition or concurrent vascular malforma-tion is seen

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CdCAs can show diverse appearance on MrI presenting hypointense [4, 10, 16–18, 20, 21, 31, 33, 35], isointense [12, 14, 19, 22, 29, 30, 36], mixed intensity [15, 27, 38], and hyperintense [13, 28] on T1-weighted images, and hyperin-tense [4, 10, 12, 14, 16–22, 27–31, 36, 38], isointense [35], mixed intensity [15], and hypointense [13] on T2-weighted images. While intense/homogeneous enhancement with con-trast medium was frequently noted [4, 7, 10, 12, 14, 15, 17–19, 21, 22, 25, 27, 28, 35, 36], heterogeneous enhancement was also found [5, 13, 16, 29, 31, 33]. dural tail sign was fre-quently noted [7, 14, 18–22, 27, 36], while hypointense rim on T2-weighted imaging was rarely reported [38]. CT showed CdCAs as isodense or hyperdense with varying degrees of intralesional calcification [13, 16, 24, 25, 30, 32, 37]. notably, not a few patients were diagnosed as meningioma on presurgical MrI [5, 12, 14–16, 19–22, 25, 27, 29, 31, 35, 36].

surgical resection is the mainstay of treatment. Intraoper-ative CdCAs appeared reddish [9–11, 14, 21, 22, 27, 34, 37] or vascular [4, 11, 16, 19, 24, 26, 27, 31], with varying degrees of feeding vessels from the basal dura mater. Most CdCAs were totally removed with comfortable outcome. A patient underwent emergent hematoma evacuation for acute subdural hemorrhage from CdCA [37].

Angioleiomyoma, a benign tumor also known as vascular leiomyoma or angiomyoma, is commonly present as cutane-ous and soft tissue lesions in middle-aged women, and must be included in the differential diagnosis of extraaxial tumor mimicking CdCAs and meningiomas [39]. Also, metastatic foci, fibrosarcoma, neurinoma, neurofibroma, lymphoma, hemangiopericytoma, sarcoidosis, and aspergilloma should be assumed as presurgical diagnoses. Angioleiomyoma has

Fig. 3 Macroscopic appearance of the resected specimen showing the mulberry-like appearance of the lesion (L) tightly adhered to the dura mater

Fig. 4 a photomicrograph of the specimen demonstrating a collection of ectatic vascular lumens arranged in a back-to-back manner, lined with a single layer of endothelium and filled with varying degrees of recent and remote intraluminal thrombi, with little intervening con-nective tissue (arrows) and poorly demarcated from the dura mater underneath. no atypia is seen. Hematoxylin and eosin stain, original magnification × 20. b, c Immunostaining for CD34 is confined to the endothelial layer (b, arrows), which is surrounded by an alpha smooth muscle actin (α-sMA) layer of varying thickness (c, arrows). original magnification × 40

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a nodular, mulberry-like appearance, and consists of endo-thelium-lined vessels of varying sizes. Therefore, angioleio-myoma arising from the dura mater is hard to discriminate from CdCA. Histologically, angioleiomyomas include con-centric whorls of sMA+/Cd34− cells, which are not found in CAs [39].

Conclusion

CdCA should be included in the differential diagnosis of dural-based, extraaxial tumors located in the cranial cavity. surgical resection is the optimum treatment with satisfac-tory outcome.

Conflict of Interest The authors declare no conflict of interest con-cerning the materials or methods used in this study or the findings specified in this paper.

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