Clinical Neurology and Neurosurgery 126 (2014) 99–102
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Review
Epidural capillary hemangioma: A review of the literature Pinar Gencpinar a , Sabri Cem Ac¸ıkbas¸ b , Banu Güzel Nur c , Kamil Karaali d , Murat Arslan b , Elif Inanc Gurer e , Ozgur Duman a , Senay Haspolat a,∗ a
Department of Pediatric Neurology, Akdeniz University Medical Faculty, Antalya, Turkey Department of Neurosurgery, Akdeniz University Medical Faculty, Antalya, Turkey Department of Pediatric Genetics, Akdeniz University Medical Faculty, Antalya, Turkey d Department of Radiology, Akdeniz University Medical Faculty, Antalya, Turkey e Department of Pathology, Akdeniz University Medical Faculty, Antalya, Turkey b c
a r t i c l e
i n f o
Article history: Received 25 April 2014 Received in revised form 15 August 2014 Accepted 23 August 2014 Available online 30 August 2014 Keywords: Epidural hemangioma Capillary hemangioma Childhood
a b s t r a c t Hemangiomas in the spinal epidural area are very rare lesions, and most of these lesions are of the cavernous type. Only seven cases of capillary hemangiomas have been reported in the English literature, and all of these cases occurred in adulthood. Here, we report on a 17-month-old girl who presented with an inability to walk. MRI revealed an epidural mass, which was diagnosed as an epidural capillary hemangioma in the thoracic region. To our best knowledge, this case is the first epidural capillary hemangioma case to occur in childhood that has been reported. © 2014 Elsevier B.V. All rights reserved.
Contents 1. 2. 3. 4.
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 Case . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102
1. Introduction Capillary hemangiomas are ubiquitous hamartomatous malformations that result from proliferations of vascular endothelial cells [1]. Capillary hemangiomas are present in as many as 1 of every 200 births. These hemangiomas are most commonly found in the skin, soft tissues, and bone, and they typically regress without surgical treatment. In contrast, histologically similar capillary hemangiomas in the spinal epidural area are rare lesions, and most of the reported cases have been of the cavernous type. The occurrence of epidural capillary hemangiomas is exceedingly rare. Only seven cases have been reported in the English literature, and all of these cases have occurred in adulthood [2–8].
∗ Corresponding author at: Department of Pediatric Neurology, Faculty of Medicine, Akdeniz University, Pınarbas¸ı Mh. Akdeniz Ünv. Hst., 07070 Konyaaltı, Antalya, Turkey. Tel.: +90 242 249 65 20; fax: +90 242 227 43 43. E-mail address: [email protected] (S. Haspolat). http://dx.doi.org/10.1016/j.clineuro.2014.08.026 0303-8467/© 2014 Elsevier B.V. All rights reserved.
Here, we report on a 17-month-old girl who presented with the inability to walk and was diagnosed with an epidural capillary hemangioma in the thoracic region. Our case is the first case of diagnosed epidural capillary hemangioma in childhood. 2. Case A previously well, 17-month-old girl presented with the inability to walk and restlessness for 1 day. She had no prodromal features such as fever. There were no histories of trauma, vaccination or infection, and her immunization schedules were complete and included poliomyelitis. Physical examination revealed weakness in the lower extremities that caused the walking difficulties. Stiffness in the neck was also present. Neither Brudzinski’s nor Kernig’s sign were present. Upon admission, she was afebrile, and her strength in the upper extremities was graded as 4/5 MRC. She was able to lift her legs but was afraid to step. Her deep tendon reflexes were normal in all four extremities. There were no pathological reflexes. A sensory examination could not be completed due to a
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P. Gencpinar et al. / Clinical Neurology and Neurosurgery 126 (2014) 99–102
Fig. 1. T2-weighted sagittal MR image showing an epidural mass extending from the T3 to T7 vertebrae and causing spinal cord compression. Flow-void tubular structures (arrows) can be seen within the mass.
Fig. 3. T1-weighted post-contrast image showing mild septal enhancement of the mass.
an urgent left hemilaminectomy of the third thoracic vertebra was performed, and the lesion was totally removed. During this operation, the epidural mass was observed to extent into the left T4-5 and T5-6 foramina. Histopathological examination revealed a capillary hemangioma (Fig. 4). We did not identify any pathogenic Von Hippel Lindau (VHL) gene mutations in exon 1, exon 2, or exon 3. Upon the final evaluation, we observed that the patient was able to walk without assistance. 3. Discussion Fig. 2. T2-weighted transverse MR image showing the epidural mass and cord compression. The bony structures are normal.
lack of cooperation. The results of laboratory examinations (i.e., complete blood cell count, liver enzymes, kidney function parameters, C-reactive protein, and erythrocyte sedimentation rate) and procoagulant studies were normal. Magnetic resonance imaging (MRI) revealed an extra-osseous, extradural mass without any bone invasion at the T3–T7 vertebral level that was located dorsal to the thecal sac. The spinal cord was compressed dorsally. The mass exhibited mild enhancement (Figs. 1–3). Total laminectomy of the T7 vertebra was initially performed to evaluate the lesion. The lesion appeared as a red-purple colored, well-encapsulated hemorrhagic epidural mass. Laminoplasty was planned to remove the lesion but was not performed due to massive bleeding. Therefore,
Capillary hemangiomas are considered to be hamartomatous proliferations of vascular endothelial cells. These lesions are classified according to the predominant type of vascular channel (i.e., capillary, cavernous, arterio-venous, or venous) that is observed upon histological examination [9]. The characteristic features of capillary hemangioma are thin irregular capillary-sized vessels caught in low attenuating fibroses, lobular architecture and the presence of a lining of a continuous basal lamina. These lesions exhibit low mitotic activity, positive reactions for CD31 and CD34, and negative reactions for S100 and epithelial membrane antigen upon histopathological examination [10]. In the English literature, only seven (three female and four male) cases of purely epidural capillary hemangioma have been reported (Table 1). All of the published cases occurred in adults over the age
Fig. 4. Proliferation of capillary vascular channels. H&E ×50, H&E ×100, H&E ×100, and CD 31 immunoreactivity ×100. (For interpretation of the references to color in the citation to this figure, the reader is referred to the web version of this article.)
P. Gencpinar et al. / Clinical Neurology and Neurosurgery 126 (2014) 99–102
101
Table 1 The features of cases diagnosed spinal capillary hemangiomas in the literature to date. Cases
Age/Gender
Presenting symptoms
Physical examination
Magnetic resonance imaging findings
Computerized Treatment tomography scan images
1
17 months/F
Acute onset of inability to walk, restlessness
Stiffness on the neck, weakness on the lower extremities
N/A
2
58/M
History of increasing low back pain and gait difficulty
3
59/F
Back pain and right intercostal neuralgia
57/M
Persistent dorsal pain after a minor car accident Presented again 2.5 years later with a 5-month history of progressive right leg weakness, low thoracic back pain descending to the right leg, numbness of the left leg and a band of numbness at the level of the umbilicus on the right and complained of occasional urine and stool incontinence Back pain radiating through right anterior thigh and hypoesthesia at medial foot A radiating, band-like, chest wall pain
A severe paraparetic symptomatology without any sphincter involvement Proprioceptive ataxia difficulties with walking, decrease of the motor strength in lower limbs, and brisk osteotendinous reflexes Normal
Extra-osseous, extradural mass, without any bone invasion at the T3–T7 vertebral levels, located dorsal to the thecal sac An extradural mass at the level of T2–T4
4
Second presentation
5
56/F
6
56/M
7
40/F
8
50/M
Progressive pain, muscular contracture and motor deficit of her lower limbs, difficulties with walking and sphincter problems. Backache then progressive myelopathy
Prognosis
Reference
Laminectomy and total resection
Very good neurologic condition
Our case
N/A
Laminectomy and total resection
Very good neurologic condition and no recurrence
Seferi et al. (2014) [2]
Probably epidural mass extending from T5 to T7, with intrathoracic extension
N/A
Laminectomy and total resection
N/A
Vassal et al. (2011) [3]
Epidural lesion at the level of T10–T12 A very slightly increased lesion causing cord compression
N/A
Refused treatment
N/A
Hasan et al. (2011) [4]
Laminectomy
He was mobilizing with a walker, and his urinary symptoms had improved. At 8 months, he was ambulating with a cane and had only residual sensory band-type pain
Right sided L4 dermatomal hypoesthesia
Heterogeneous mass with flow voids
N/A
Hemilaminectomy and total resection
Improved pain and no residual lesion
Tekin et al. (2008) [5]
Unremarkable
Dumbbell-shaped epidural mass
Decompressive laminectomy
N/A
Kang et al. (2006) [6]
Decreased motor strength in her lower limbs
Probably epidural mass extending from T2 to T4
T3–T4 dumbbellshaped epidural mass Widening of the left foramen at T3–T4
Laminectomy and total resection
No residual Tm
Badinand et al. (2006) [7]
Mild paresis in the left lower limb and hyperreflexia in both lower limbs, with bilateral positive Babinski’s sign. Vibration sense was impaired at both medial malleoli and the left tibia1 tuberosity
Enhancing mass
Total resection
No residual Tm and complete recovery in clinical findings
Gupta et al. (1996) [8]
Right leg weakness ranging from 3/5 to 42/5, sensory band around T10 with an altered sensation to light touch and hyperalgia, decreased light touch sensation over the left lower extremity, positive Romberg sign, poor rectal muscle tone
N/A
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of 40 years. Five of these hemangiomas were in the thoracic region [2–4,6–8], and one was in the lumbar region [5]. The lengths of the masses in these cases extended to a maximum of two or three vertebral segments. To the best of our knowledge, ours is the first case to present in childhood. The appearance of the hemangioma in our case was similar to those of the cases that have been described in the literature; however, in our case, the mass extended across five vertebral segments. Central nervous system cavernous hemangiomas can exhibit signs of hemorrhages of various ages. In contrast, epidural hemangiomas present clinically with progressive myelopathy and no signs of acute or chronic bleeding. All reported cases have presented with back pain, radicular pain, or chronic myelopathy due to the compression of the spinal cord by the masses [2–5]. The presenting symptoms of our case were restlessness and the inability to walk. The patient was able to lift her legs but was afraid to step due to the compression of the spinal cord by the mass. Hasan et al. [4] reported a case in a 57-year-old male. This case exhibited a presenting symptom of mild dorsal pain. In the follow-up of this patient, progressive right leg weakness and urinary and fecal incontinence developed after 2.5 years. These authors suggested that even in the absence of neurological symptoms, surgery should be considered due to the risk of cord compression and, at a minimum, these patients should be closely followed up. Capillary hemangiomas of the spinal cord and proximal nerves must be distinguished from hemangioblastomas. Hemangioblastomas contain numerous capillaries but also have additional stromal cells that are reactive for neuron-specific enolase and S100 protein and a rich reticulin network. Hemangioblastomas are most commonly located in the posterior fossa, but up to 5% occur in the spinal cord. Approximately one-third of central/peripheral nervous system hemangioblastomas are associated with VHL syndrome; therefore, the important clinical and histopathologic distinction between hemangioblastomas and capillary hemangiomas should be further examined [6,11]. In our patient, the histopathologic examination revealed a capillary hemangioma, and VHL gene mutation was not detected. The most important diagnostic tool is MRI, which allows for superior morphologic assessment. The radiologic aspects are nonspecific. Well-circumscribed tumors and a dumbbell shape can be observed on MR images but are also commonly found with other benign tumors, such as neuromas and meningiomas. Our case reminds us that hemangioma should also be considered in the differential diagnosis of epidural masses. Draining veins, which
appear as hypointense structures on MRI, should be identified to distinguish hypervascular lesions from other types of lesions in the epidural space. Prior to surgery, angiography is recommended to identify capillary hemangiomas. Angiography was not performed in our case because the patient underwent immediate surgery due to spinal cord compression [6,12]. The treatment of choice for capillary hemangioma is complete surgical removal. Gross total resection should be the goal [4,7]. The prognosis is typically good, and no relapses have been reported. 4. Conclusion Epidural capillary hemangioma is an extremely rare condition. No childhood cases had previously been reported. Our study describes the youngest patient in the literature. References [1] Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. Musculoskeletal angiomatous lesions: radiologic–pathologic correlation. Radiographics 1995;15:893–917. [2] Seferi A, Alimehmeti R, Vyshka G, Bushati T, Petrela M. Case study of a spinal epidural capillary hemangioma: a 4-year postoperative follow-up. Global Spine J 2014;4:55–8. [3] Vassal F, Péoc’h M, Nuti C. Epidural capillary hemangioma of the thoracic spine with proximal nerve root involvement and extraforaminal extension. Acta Neurochir (Wien) 2011;153:2279–81. [4] Hasan A, Guiot MC, Torres C, Marcoux J. A case of spinal epidural capillary hemangioma: case report. Neurosurgery 2011;68:850–3. [5] Tekin T, Bayrakli F, Simsek H, Colak A, Kutlay M, Demircan MN. Lumbar epidural capillary hemangioma presenting as lumbar disc herniation disease. Spine 2008;33:795–7. [6] Kang JS, Lillehei KO, Kleinschmidt-Demasters BK. Proximal nerve root capillary hemangioma presenting as a lung mass with bandlike chest pain: case report and review of literature. Surg Neurol 2006;65:584–9. [7] Badinand B, Morel C, Kopp N, Tran Min VA, Cotton F. Dumbbell-shaped epidural capillary hemangioma. AJNR Am J Neuroradiol 2003;24:190–2. [8] Gupta S, Kumar S, Banerji D, Pandey R, Gujral R. Magnetic resonance imaging features of an epidural spinal haemangioma. Australas Radiol 1996;40: 342–4. [9] Abe M, Misago N, Tanaka S, Masuoka J, Tabuchi K. Capillary hemangioma of the central nervous system: a comparative study with lobular capillary hemangioma of the skin. Acta Neuropathol 2005;109:151–8. [10] Aoyagi N, Kojima K, Kasai H. Review of spinal epidural cavernous hemangioma. Neurol Med Chir (Tokyo) 2003;43:471–6. [11] Escott EJ, Kleinschmidt-DeMasters BK, Brega K, Lillehei KO. Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review. Surg Neurol 2004;61:262–73. [12] Abdullah DC, Raghuram K, Phillips CD, Jane Jr JA, Miller B. Thoracic intradural extramedullary capillary hemangioma. AJNR Am J Neuroradiol 2004;25:1294–6.
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Review
Epidural capillary hemangioma: A review of the literature Pinar Gencpinar a , Sabri Cem Ac¸ıkbas¸ b , Banu Güzel Nur c , Kamil Karaali d , Murat Arslan b , Elif Inanc Gurer e , Ozgur Duman a , Senay Haspolat a,∗ a
Department of Pediatric Neurology, Akdeniz University Medical Faculty, Antalya, Turkey Department of Neurosurgery, Akdeniz University Medical Faculty, Antalya, Turkey Department of Pediatric Genetics, Akdeniz University Medical Faculty, Antalya, Turkey d Department of Radiology, Akdeniz University Medical Faculty, Antalya, Turkey e Department of Pathology, Akdeniz University Medical Faculty, Antalya, Turkey b c
a r t i c l e
i n f o
Article history: Received 25 April 2014 Received in revised form 15 August 2014 Accepted 23 August 2014 Available online 30 August 2014 Keywords: Epidural hemangioma Capillary hemangioma Childhood
a b s t r a c t Hemangiomas in the spinal epidural area are very rare lesions, and most of these lesions are of the cavernous type. Only seven cases of capillary hemangiomas have been reported in the English literature, and all of these cases occurred in adulthood. Here, we report on a 17-month-old girl who presented with an inability to walk. MRI revealed an epidural mass, which was diagnosed as an epidural capillary hemangioma in the thoracic region. To our best knowledge, this case is the first epidural capillary hemangioma case to occur in childhood that has been reported. © 2014 Elsevier B.V. All rights reserved.
Contents 1. 2. 3. 4.
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 Case . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102
1. Introduction Capillary hemangiomas are ubiquitous hamartomatous malformations that result from proliferations of vascular endothelial cells [1]. Capillary hemangiomas are present in as many as 1 of every 200 births. These hemangiomas are most commonly found in the skin, soft tissues, and bone, and they typically regress without surgical treatment. In contrast, histologically similar capillary hemangiomas in the spinal epidural area are rare lesions, and most of the reported cases have been of the cavernous type. The occurrence of epidural capillary hemangiomas is exceedingly rare. Only seven cases have been reported in the English literature, and all of these cases have occurred in adulthood [2–8].
∗ Corresponding author at: Department of Pediatric Neurology, Faculty of Medicine, Akdeniz University, Pınarbas¸ı Mh. Akdeniz Ünv. Hst., 07070 Konyaaltı, Antalya, Turkey. Tel.: +90 242 249 65 20; fax: +90 242 227 43 43. E-mail address: [email protected] (S. Haspolat). http://dx.doi.org/10.1016/j.clineuro.2014.08.026 0303-8467/© 2014 Elsevier B.V. All rights reserved.
Here, we report on a 17-month-old girl who presented with the inability to walk and was diagnosed with an epidural capillary hemangioma in the thoracic region. Our case is the first case of diagnosed epidural capillary hemangioma in childhood. 2. Case A previously well, 17-month-old girl presented with the inability to walk and restlessness for 1 day. She had no prodromal features such as fever. There were no histories of trauma, vaccination or infection, and her immunization schedules were complete and included poliomyelitis. Physical examination revealed weakness in the lower extremities that caused the walking difficulties. Stiffness in the neck was also present. Neither Brudzinski’s nor Kernig’s sign were present. Upon admission, she was afebrile, and her strength in the upper extremities was graded as 4/5 MRC. She was able to lift her legs but was afraid to step. Her deep tendon reflexes were normal in all four extremities. There were no pathological reflexes. A sensory examination could not be completed due to a
100
P. Gencpinar et al. / Clinical Neurology and Neurosurgery 126 (2014) 99–102
Fig. 1. T2-weighted sagittal MR image showing an epidural mass extending from the T3 to T7 vertebrae and causing spinal cord compression. Flow-void tubular structures (arrows) can be seen within the mass.
Fig. 3. T1-weighted post-contrast image showing mild septal enhancement of the mass.
an urgent left hemilaminectomy of the third thoracic vertebra was performed, and the lesion was totally removed. During this operation, the epidural mass was observed to extent into the left T4-5 and T5-6 foramina. Histopathological examination revealed a capillary hemangioma (Fig. 4). We did not identify any pathogenic Von Hippel Lindau (VHL) gene mutations in exon 1, exon 2, or exon 3. Upon the final evaluation, we observed that the patient was able to walk without assistance. 3. Discussion Fig. 2. T2-weighted transverse MR image showing the epidural mass and cord compression. The bony structures are normal.
lack of cooperation. The results of laboratory examinations (i.e., complete blood cell count, liver enzymes, kidney function parameters, C-reactive protein, and erythrocyte sedimentation rate) and procoagulant studies were normal. Magnetic resonance imaging (MRI) revealed an extra-osseous, extradural mass without any bone invasion at the T3–T7 vertebral level that was located dorsal to the thecal sac. The spinal cord was compressed dorsally. The mass exhibited mild enhancement (Figs. 1–3). Total laminectomy of the T7 vertebra was initially performed to evaluate the lesion. The lesion appeared as a red-purple colored, well-encapsulated hemorrhagic epidural mass. Laminoplasty was planned to remove the lesion but was not performed due to massive bleeding. Therefore,
Capillary hemangiomas are considered to be hamartomatous proliferations of vascular endothelial cells. These lesions are classified according to the predominant type of vascular channel (i.e., capillary, cavernous, arterio-venous, or venous) that is observed upon histological examination [9]. The characteristic features of capillary hemangioma are thin irregular capillary-sized vessels caught in low attenuating fibroses, lobular architecture and the presence of a lining of a continuous basal lamina. These lesions exhibit low mitotic activity, positive reactions for CD31 and CD34, and negative reactions for S100 and epithelial membrane antigen upon histopathological examination [10]. In the English literature, only seven (three female and four male) cases of purely epidural capillary hemangioma have been reported (Table 1). All of the published cases occurred in adults over the age
Fig. 4. Proliferation of capillary vascular channels. H&E ×50, H&E ×100, H&E ×100, and CD 31 immunoreactivity ×100. (For interpretation of the references to color in the citation to this figure, the reader is referred to the web version of this article.)
P. Gencpinar et al. / Clinical Neurology and Neurosurgery 126 (2014) 99–102
101
Table 1 The features of cases diagnosed spinal capillary hemangiomas in the literature to date. Cases
Age/Gender
Presenting symptoms
Physical examination
Magnetic resonance imaging findings
Computerized Treatment tomography scan images
1
17 months/F
Acute onset of inability to walk, restlessness
Stiffness on the neck, weakness on the lower extremities
N/A
2
58/M
History of increasing low back pain and gait difficulty
3
59/F
Back pain and right intercostal neuralgia
57/M
Persistent dorsal pain after a minor car accident Presented again 2.5 years later with a 5-month history of progressive right leg weakness, low thoracic back pain descending to the right leg, numbness of the left leg and a band of numbness at the level of the umbilicus on the right and complained of occasional urine and stool incontinence Back pain radiating through right anterior thigh and hypoesthesia at medial foot A radiating, band-like, chest wall pain
A severe paraparetic symptomatology without any sphincter involvement Proprioceptive ataxia difficulties with walking, decrease of the motor strength in lower limbs, and brisk osteotendinous reflexes Normal
Extra-osseous, extradural mass, without any bone invasion at the T3–T7 vertebral levels, located dorsal to the thecal sac An extradural mass at the level of T2–T4
4
Second presentation
5
56/F
6
56/M
7
40/F
8
50/M
Progressive pain, muscular contracture and motor deficit of her lower limbs, difficulties with walking and sphincter problems. Backache then progressive myelopathy
Prognosis
Reference
Laminectomy and total resection
Very good neurologic condition
Our case
N/A
Laminectomy and total resection
Very good neurologic condition and no recurrence
Seferi et al. (2014) [2]
Probably epidural mass extending from T5 to T7, with intrathoracic extension
N/A
Laminectomy and total resection
N/A
Vassal et al. (2011) [3]
Epidural lesion at the level of T10–T12 A very slightly increased lesion causing cord compression
N/A
Refused treatment
N/A
Hasan et al. (2011) [4]
Laminectomy
He was mobilizing with a walker, and his urinary symptoms had improved. At 8 months, he was ambulating with a cane and had only residual sensory band-type pain
Right sided L4 dermatomal hypoesthesia
Heterogeneous mass with flow voids
N/A
Hemilaminectomy and total resection
Improved pain and no residual lesion
Tekin et al. (2008) [5]
Unremarkable
Dumbbell-shaped epidural mass
Decompressive laminectomy
N/A
Kang et al. (2006) [6]
Decreased motor strength in her lower limbs
Probably epidural mass extending from T2 to T4
T3–T4 dumbbellshaped epidural mass Widening of the left foramen at T3–T4
Laminectomy and total resection
No residual Tm
Badinand et al. (2006) [7]
Mild paresis in the left lower limb and hyperreflexia in both lower limbs, with bilateral positive Babinski’s sign. Vibration sense was impaired at both medial malleoli and the left tibia1 tuberosity
Enhancing mass
Total resection
No residual Tm and complete recovery in clinical findings
Gupta et al. (1996) [8]
Right leg weakness ranging from 3/5 to 42/5, sensory band around T10 with an altered sensation to light touch and hyperalgia, decreased light touch sensation over the left lower extremity, positive Romberg sign, poor rectal muscle tone
N/A
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of 40 years. Five of these hemangiomas were in the thoracic region [2–4,6–8], and one was in the lumbar region [5]. The lengths of the masses in these cases extended to a maximum of two or three vertebral segments. To the best of our knowledge, ours is the first case to present in childhood. The appearance of the hemangioma in our case was similar to those of the cases that have been described in the literature; however, in our case, the mass extended across five vertebral segments. Central nervous system cavernous hemangiomas can exhibit signs of hemorrhages of various ages. In contrast, epidural hemangiomas present clinically with progressive myelopathy and no signs of acute or chronic bleeding. All reported cases have presented with back pain, radicular pain, or chronic myelopathy due to the compression of the spinal cord by the masses [2–5]. The presenting symptoms of our case were restlessness and the inability to walk. The patient was able to lift her legs but was afraid to step due to the compression of the spinal cord by the mass. Hasan et al. [4] reported a case in a 57-year-old male. This case exhibited a presenting symptom of mild dorsal pain. In the follow-up of this patient, progressive right leg weakness and urinary and fecal incontinence developed after 2.5 years. These authors suggested that even in the absence of neurological symptoms, surgery should be considered due to the risk of cord compression and, at a minimum, these patients should be closely followed up. Capillary hemangiomas of the spinal cord and proximal nerves must be distinguished from hemangioblastomas. Hemangioblastomas contain numerous capillaries but also have additional stromal cells that are reactive for neuron-specific enolase and S100 protein and a rich reticulin network. Hemangioblastomas are most commonly located in the posterior fossa, but up to 5% occur in the spinal cord. Approximately one-third of central/peripheral nervous system hemangioblastomas are associated with VHL syndrome; therefore, the important clinical and histopathologic distinction between hemangioblastomas and capillary hemangiomas should be further examined [6,11]. In our patient, the histopathologic examination revealed a capillary hemangioma, and VHL gene mutation was not detected. The most important diagnostic tool is MRI, which allows for superior morphologic assessment. The radiologic aspects are nonspecific. Well-circumscribed tumors and a dumbbell shape can be observed on MR images but are also commonly found with other benign tumors, such as neuromas and meningiomas. Our case reminds us that hemangioma should also be considered in the differential diagnosis of epidural masses. Draining veins, which
appear as hypointense structures on MRI, should be identified to distinguish hypervascular lesions from other types of lesions in the epidural space. Prior to surgery, angiography is recommended to identify capillary hemangiomas. Angiography was not performed in our case because the patient underwent immediate surgery due to spinal cord compression [6,12]. The treatment of choice for capillary hemangioma is complete surgical removal. Gross total resection should be the goal [4,7]. The prognosis is typically good, and no relapses have been reported. 4. Conclusion Epidural capillary hemangioma is an extremely rare condition. No childhood cases had previously been reported. Our study describes the youngest patient in the literature. References [1] Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. Musculoskeletal angiomatous lesions: radiologic–pathologic correlation. Radiographics 1995;15:893–917. [2] Seferi A, Alimehmeti R, Vyshka G, Bushati T, Petrela M. Case study of a spinal epidural capillary hemangioma: a 4-year postoperative follow-up. Global Spine J 2014;4:55–8. [3] Vassal F, Péoc’h M, Nuti C. Epidural capillary hemangioma of the thoracic spine with proximal nerve root involvement and extraforaminal extension. Acta Neurochir (Wien) 2011;153:2279–81. [4] Hasan A, Guiot MC, Torres C, Marcoux J. A case of spinal epidural capillary hemangioma: case report. Neurosurgery 2011;68:850–3. [5] Tekin T, Bayrakli F, Simsek H, Colak A, Kutlay M, Demircan MN. Lumbar epidural capillary hemangioma presenting as lumbar disc herniation disease. Spine 2008;33:795–7. [6] Kang JS, Lillehei KO, Kleinschmidt-Demasters BK. Proximal nerve root capillary hemangioma presenting as a lung mass with bandlike chest pain: case report and review of literature. Surg Neurol 2006;65:584–9. [7] Badinand B, Morel C, Kopp N, Tran Min VA, Cotton F. Dumbbell-shaped epidural capillary hemangioma. AJNR Am J Neuroradiol 2003;24:190–2. [8] Gupta S, Kumar S, Banerji D, Pandey R, Gujral R. Magnetic resonance imaging features of an epidural spinal haemangioma. Australas Radiol 1996;40: 342–4. [9] Abe M, Misago N, Tanaka S, Masuoka J, Tabuchi K. Capillary hemangioma of the central nervous system: a comparative study with lobular capillary hemangioma of the skin. Acta Neuropathol 2005;109:151–8. [10] Aoyagi N, Kojima K, Kasai H. Review of spinal epidural cavernous hemangioma. Neurol Med Chir (Tokyo) 2003;43:471–6. [11] Escott EJ, Kleinschmidt-DeMasters BK, Brega K, Lillehei KO. Proximal nerve root spinal hemangioblastomas: presentation of three cases, MR appearance, and literature review. Surg Neurol 2004;61:262–73. [12] Abdullah DC, Raghuram K, Phillips CD, Jane Jr JA, Miller B. Thoracic intradural extramedullary capillary hemangioma. AJNR Am J Neuroradiol 2004;25:1294–6.
