Neurosurg Rev (2003) 26:292–296 DOI 10.1007/s10143-003-0275-8
CASE REPORT
Giancarlo D’Andrea · Orlando Epimenio Ramundo · Giuseppe Trill · Raffaelino Roperto · Alessandra Isidori · Luigi Ferrante
Dorsal foramenal extraosseous epidural cavernous hemangioma Received: 24 January 2003 / Accepted: 21 March 2003 / Published online: 14 June 2003 Springer-Verlag 2003
Abstract Cavernous hemangiomas are vascular hamartomatous malformations that affect the central nervous system. This pathology is frequently encountered in the cerebral hemispheres, cerebellum, and brainstem. Cavernous hemangiomas infrequently occur at the spinal level or relative to an intramedullary localization; extramedullary epidural sites are also sometimes affected by this pathology. We report an extradural, extramedullary, cavernous hemangioma with foramenal extension of the dorsal section and discuss the differential diagnosis with dorsal foramenal neurinoma. A 52-year-old woman was admitted with irritation and deficit symptoms radiating into the left D3 spinal root space. The first radiological diagnosis was of a foramenal neurinoma beginning at the D3 root. The presence of a heterogeneous MR signal in both T1 and T2 images led us to consider the differential diagnosis of a cavernous hemangioma lesion. The patient underwent microsurgical treatment with a far lateral extraforamenal approach. Symptoms quickly improved: pain and dysesthesia disappeared after surgery and only light hypoesthesia was found. We want to stress the importance of MR imaging in formulating a correct differential diagnosis with foramenal neurinoma and underline that microsurgical treatment with a far lateral extraforamenal approach allowed us to remove the lesion completely without affecting Denis’s posterior column, the lamina, and the articular facet.
Introduction Cavernous hemangiomas are vascular hamartomatous malformations that affect the central nervous system. This pathology is frequently encountered in the cerebral hemispheres, cerebellum, and brainstem. Cavernous hemangiomas are rarely found at the spinal level and relative to an intramedullary localization; extramedullary epidural sites are also sometimes affected by this pathology. We report a case of an extradural, extramedullary, cavernous hemangioma with foramenal extension of the dorsal section and discuss the differential diagnosis with dorsal foramenal neurinoma.
Case report A 52-year-old woman was admitted with irritation and deficit symptoms radiating into the left D3 spinal root space. She suffered from mainly nocturnal pain radiating into the D3 root dermatome and from urine dysesthesia in the same area. She had been suffering from cervical tense muscle pain for 6–8 months and presented hypoesthesia in the same dermatome. An MR exam of the cervical-
Keywords Differential diagnosis · Far lateral extraforamenal approach · Foramenal cavernous hemangioma · Foramenal neurinoma G. D’Andrea · O. E. Ramundo · G. Trill · R. Roperto · A. Isidori · L. Ferrante Department of Neurological Sciences, “La Sapienza” University, Rome, Italy G. D’Andrea ()) V. L. Mantegazza 8, 00152 Rome, Italy e-mail:
[email protected] Tel.: +39-333-2489054
Fig. 1 T1-weighted MRI signal showing a foramenal lesion quite similar to neurinoma
293 Fig. 2 Contrast-enhanced T1 MRI reveals the spinal root, the mass compressing the root itself, and a mass displacing in the left D3-4 intervertebral foramen Fig. 3 Postoperative MRI control showing the minimally invasive extraforamenal approach to the dorsal foramenal cavernous hemangioma
Fig. 4 Photomicrograph of the cavernous hemangioma showing the thin-walled vascular spaces with no necrosis, hemorrhage, or degenerative phenomena dorsal section was performed. Sagittal T1- and T2-weighted images showed a displacing mass in the left D3-4 intervertebral foramen. T1-weighted axial images (Fig. 1) with contrast medium confirmed a hypoisointense lesion with significant enhancement (Fig. 2); long repetition time axial images revealed a heterogeneous hyperintense foramenal lesion. The first radiological diagnosis was of a foramenal neurinoma beginning at the D3 root. However, the heterogeneous MR signal in both T1 and T2 images led us to consider the differential diagnosis of a cavernous hemangioma lesion. This was later confirmed by two factors: the presence of a purely foramenal lesion and the appearance in contrast-enhanced T1- and T2-weighted images of the spinal root being straightened inside the foramen by the compression from an extradural mass. The lesion’s reduced dimensions, purely foramenal location, and extradural characteristics allowed us to employ a noninvasive extraforamenal approach. The patient underwent microsurgical treatment with a far lateral extraforamenal approach and minimal bone reduction (Fig. 3). The pathologic finding was striking because of the presence of a cavernous hemangioma (Fig. 4, Fig. 5). A dark reddish mass, completely separated from the root, was found extending to the foramen and compressing the root itself. The lesion measured approximately 75 mm and bled moderately when touched.
Fig. 5 Second photomicrograph of the same histological exam of the cavernous hemangioma shown in Fig. 4
Results Total removal of the mass was achieved. Histological examination revealed an extraosseous cavernous hemangioma. Magnetic resonance exam and CT scan demonstrated complete removal of the lesion and the integrity of the osseous structure with minimal reduction of the vertebral isthmus. Symptoms quickly improved: the pain and dysesthesia disappeared after surgery, and only light hypoesthesia remained.
Discussion Spinal cavernous hemangiomas are rare lesions and usually localized at the intramedullary level [1, 2, 3, 4, 5, 6]; the greater use of and improvements in MRI diagnostic technology are of great help in detecting this pathology [1, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16]. Extradural localization in the epidural space is very rare [4, 13, 17, 18, 19, 20, 21, 22, 23]; about 65 cases have
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been reported in the literature [16, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54]. Epidural foramenal localization is extremely uncommon: the literature reports only one case localized at the cervical level [24] besides ours. However, cases of epidural cavernous hemangioma with preforamenal and/or intraforamenal extension are described [18, 25, 33, 35, 47, 50, 54]. Cavernous hemangiomas are not vascular neoplasia [45] but can be considered hamartomas caused by malfunctioning of the microcirculation [55, 56]. They constitute 5–12% of spinal vascular lesions [2, 3, 4, 57] and approximately 4% of all spinal epidural tumors [16, 23, 24, 50, 58, 59, 60, 61]. The section involved is thoracic in 54–60% of cases [2, 4, 13, 14, 15, 31, 50], cervical in 30%, and lumbar in 10% [2]. The lesions can measure from a few millimeters to more than a centimeter in size and are usually wellcircumscribed by a reactive gliotic rim. As at the encephalic level, they are pathologically characterized by sinusoidal vascular spaces lined by a thin wall and cysts with a stagnant or particularly slow blood flow. The lesions are usually surrounded by the adjacent nervous tissue infiltration caused by macrophages; hemosiderin deposits and inflammatory cells, related to chronic hemorrhages within the lesion itself, were noted as well. In 70% of cases, this pathology affects women [10, 20, 22, 62, 63]. It is still difficult to define the clinical/ epidemiological features of these lesions [10, 64]. Spinal lesions due to cavernous hemangiomas, such as cerebral ones, seem to have a familial incidence [14, 31, 63, 64, 65, 66, 67, 68, 69]; the mean age of onset of this pathology varies between 30 and 60 years [1, 2], with a particular incidence around 40 [2]. While intramedullary lesions can remain asymptomatic for a long time and thus develop chronically in 78% of cases [2], the clinical features of epidural spinal cavernous hemangiomas are mainly acute [24]. The acute symptomatology is due to the presence of microhemorrhages within the lesion compressing the spinal cord [14, 16, 50, 70, 71]. The onset of hemorrhage can cause an epidural hematoma with spinal cord compression [24]. A lesion with a preforamenal or intraforamenal extension can also appear with classic radiculopathic symptomatology [24, 72], and this must be considered in the differential diagnosis with disc herniation [24] or neurinoma. Magnetic resonance imaging [73] greatly facilitates the diagnosis of this kind of lesion [1, 6, 24, 74, 75, 76]. It shows lesions with a mixed signal, limited by a rim with a typical low-density signal that is noticeable in both T1and T2-weighted images [1, 9, 24] and is due to hemosiderin deposits from previous intralesional bleeding. T1-weighted images show a reticulate, nonhomogeneous, isointense signal, while T2-weighted images reveal a “popcorn”-shaped, nonhomogeneous, hyperintense lesion [24]. Spinal epidural cavernous hemangiomas can arouse questions of differential diagnosis with many other extradural tumoral lesions such as meningioma, lympho-
ma, metastasis, and disc fragments [24, 77, 78]; on the other hand, purely foramenal cavernous hemangioma can awaken significant doubts in the differential diagnosis with neurinoma [24]. In the case presented here, the initial diagnosis was of a foramenal neurinoma of the left D3 dorsal root. Although the MR signal can be similar in both foramenal lesions, T1-weighted imaging and, even more, contrast-enhanced T2 imaging reveal the spinal root and the mass compressing the root itself, the latter being detectable in MRI as well. The MR imaging led us to notice the limited dimensions of the lesion and its purely foramenal and extraforamenal localization. Evaluation of these data persuaded us to doubt the neurinoma diagnosis and to consider the possibility of an epidural cavernous hemangioma. The tumor localization induced us to plan a microsurgical, noninvasive, far lateral approach. The extraforamenal approach allowed us to remove the lesion completely without affecting Denis’s posterior column, the lamina, or the articular facet. The macroscopic operative finding was a round, dark reddish lesion; it measured 75 mm and bled when touched. This suggested an extradural cavernous hemangioma, which was later confirmed by histological studies. We wish to add that this is the second case of an extradural, purely foramenal spinal cavernous hemangioma reported in the literature and the first one described at the dorsal level. We conclude by underlining the importance of MR imaging in formulating a correct differential diagnosis with foramenal neurinoma.
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