- Email: [email protected]
Capillary hemangiomas with hemorrhage in cervicothoracic intramedullary, a case report
Interdisciplinary Neurosurgery: Advanced Techniques and Case Management 10 (2017) 78–80
Contents lists available at ScienceDirect
Interdisciplinary Neurosurgery: Advanced Techniques and Case Management journal homepage: www.inat-journal.com
Case Report
Capillary hemangiomas with hemorrhage in cervicothoracic intramedullary, a case report Jian Li a, Renlin Mao a, Jiang Feng a, Li Xiao b, Jiejing Li c, Yu Duan a,⁎ a b c
Department of Neurosurgery, Huadong Hospital, Fudan University, Shanghai, China Department of Pathology, Huadong Hospital, Fudan University, Shanghai, China Department of General Surgery, Huadong Hospital, Fudan University, Shanghai, China
a r t i c l e
i n f o
Article history: Received 31 December 2016 Revised 21 June 2017 Accepted 9 July 2017 Available online xxxx Keywords: Capillary hemangiomas Intramedullary hemorrhage Intramedullary tumor Vascular malformation
a b s t r a c t A 48-year-old male patient had presented with worsening pain at extremities and body. The MRI showed an inhomogeneously enhancing lesion at C5-T1. During the surgical evacuation through a midline myelotomy, a frozen section could not find any tumor cells or vascular malformations. Immunohistochemically, the diagnosis of capillary hemangiomas was confirmed. © 2017 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Capillary hemangiomas (CHs) are benign vascular malformations, histologically characterized by irregular nodules of capillary-sized vessels lined by flattened endothelium without abnormal cells [1], which are very rare in spinal cord. Up to date, just only two cervicothoracic intramedullary CHs patients have been reported. Because of the extreme rarity, CHs were preoperatively misdiagnosed [2]. In this study, we discuss the clinic and surgical features of one patient with hemorrhagic CHs in cervicothoracic intramedullary location.
especially more serious at left side. The patient was still able to perceive light touch, pain and the proprioception was intact. 2.2. Radiological features
2. Case report
On T1-weighted or T2-weighted of Magnetic resonance images (MRI), the mass showed different abnormal signals in the cervicothoracic intramedullary spinal cord (C5 to T1) with slightly higher or slightly lower density shadow (Fig. 1A, B); On contrast-enhanced images, the mass was inhomogeneously enhancing about 53 mm × 11 mm (Fig. 1C). Spinal cord magnetic resonance angiography displayed the surface of C4-C6 spinal cord did show some abnormal vessels signals (Fig. 1D).
2.1. Clinical summary
2.3. Surgical procedure and pathologic findings
A 48-year-old man presented with a one month history of progressive numbness and pain. On examination, the muscular force or the reflex at extremities was normal throughout. Sensation was subjectively abnormal below the T3 to T4 dermatome,
Excepting some thicker veins on the surface of spinal cord, there was no obvious lesion after the C5-C7 and partial T1 laminectomies. The midline myelotomy at C5 was carefully performed, however, only the blood blot could be found, even at C6. During the surgery, the intraoperative frozen section likewise showed just a blood clot (Fig. 2). Considering not to cause more severe complications, the surgery was terminated after evacuation of most of the blood clot. Histologically, the mass was composed of capillary-sized blood vessels lined by simple endothelium, and the necrotic cells and inflammatory cells were also found (Fig. 3).
⁎ Corresponding author at: Huadong Hospital Affiliated to Fudan University, 221#, Yan'an Western Road, Jin'an District, Shanghai 200040, China. E-mail address: [email protected] (Y. Duan).
http://dx.doi.org/10.1016/j.inat.2017.07.007 2214-7519/© 2017 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
J. Li et al. / Interdisciplinary Neurosurgery: Advanced Techniques and Case Management 10 (2017) 78–80
79
Fig. 1. Preoperative MRI images. No syringomyelia. T1-weighted images (A); T2-weighted image (B); T1-weighted image with contrast enhancement (C); Spinal cord magnetic resonance angiography (D). Red arrows indicated the lesion. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Immunohistochemistry showed positive reaction for CD34 and CD31, while no positive staining for GFAP and Olig-2. The Ki 67 was 2%. The histologic diagnosis of capillary hemangiomas was confirmed. 3. Discussion Capillary hemangiomas involving the spinal cord are extremely rare, most in the extramedullary location [3]. To our knowledge, in the last three decades, only nineteen CHs patients involved in intramedullary location, most locating thoracic or lumbar spine
cord, have been reported. The only two previous cervicothoracic intramedullary patients received better results, presumably because of typical lobular tumor shapes and distinct tumor boundaries [2,4]. Central neuropathic pain could be the only initial symptom, but it has been reported to progress very quickly and seriously, if the dorsal horn is implicated [5]. In our study, postoperatively, the muscular strength and sensation were not impaired, but the pain invaded extremely severe at first few days. After taking oxycodone and eperisone, a muscle relaxant, the patient felt effectively relief without recurrence until discharge. The postoperative pain
80
J. Li et al. / Interdisciplinary Neurosurgery: Advanced Techniques and Case Management 10 (2017) 78–80
4. Conclusion Capillary hemangiomas are very rare and easily misdiagnosed in cervicothoracic intramedullary junction and may cause severe complications. If well demarcated, surgical evacuation of the lesion should be offered, even in this location, with potential of good outcome. Follow -up MRI should be applied in any instance, even if no pathology was found intraoperatively and on pathology. One must always assume an underlying vascular lesion that has been overlooked. Conflict of interest statement
Fig. 2. The hematoma cavity of the spinal cord. The blood blot with some loose bodies could be found.
The authors declare that they have no competing financial interests and personal relationships with other people or organizations that can inappropriately influence our work.
Fig. 3. Photomicrographs showing histopathological and immunohistochemical analysis. A Histopathological photomicrograph of the mass (H&E straining, ×200). The capillary vessels were lined by endothelial cells. Blood clots were found. B–C The cells were positive for CD 31 and CD34 respectively (×100).
management could be also very important for intramedullary CHs patient. The typical MRI images of CHs usually show isointense on T1weighted, hyperintense on T2-weigted and homogeneous enhancement after contrast injection [6]. The rich vascular signals could be found by MRI spinal cord angiography [7]. But if the hematoma arose in the lesion cavity, the tumor images may display heterogeneous density on MRI images, as this case. In previous studies, the typical CHs were well-demarcated, nodular lesions at the time of surgery [2], and could be diagnosed as “vascular tumor” by frozen quick section [6]. In this CHs, only old blood clots without other lesion were found on surgical vision, even frozen quick section could not give the accurate result. We lost faith to remove the rest of mass at C7 and T1, fearing to cause more severe unnecessary complications.
References [1] D.A. Nowak, D.C. Widenka, Spinal intradural capillary haemangioma: a review, Eur. Spine J. 10 (2001) 464–472. [2] L. Wu, X. Deng, C. Yang, Y. Xu, Intramedullary spinal capillary hemangiomas: clinical features and surgical outcomes: clinical article, J. Neurosurg. Spine 19 (2013) 477–484. [3] S.H. McEvoy, M. Farrell, F. Brett, S. Looby, Haemangioma, an uncommon cause of an extradural or intradural extramedullary mass: case series with radiological pathological correlation, Insights into imaging 7 (2016) 87–98. [4] K. Hida, M. Tada, Y. Iwasaki, H. Abe, Intramedullary disseminated capillary hemangioma with localized spinal cord swelling: case report, Neurosurgery 33 (1993) 1099–1101. [5] R. Babu, T.R. Owens, I.O. Karikari, J. Moreno, T.J. Cummings, O.N. Gottfried, et al., Spinal cavernous and capillary hemangiomas in adults, Spine 38 (2013) E423–E430. [6] R. Gonzalez, J. Spears, A. Bharatha, D.G. Munoz, Spinal lobular capillary hemangioma with an intramedullary component, Clin. Neuropathol. 33 (2014) 38–41. [7] M. Abe, K. Tabuchi, S. Tanaka, A. Hodozuka, K. Kunishio, N. Kubo, et al., Capillary hemangioma of the central nervous system, J. Neurosurg. 101 (2004) 73–81.
Contents lists available at ScienceDirect
Interdisciplinary Neurosurgery: Advanced Techniques and Case Management journal homepage: www.inat-journal.com
Case Report
Capillary hemangiomas with hemorrhage in cervicothoracic intramedullary, a case report Jian Li a, Renlin Mao a, Jiang Feng a, Li Xiao b, Jiejing Li c, Yu Duan a,⁎ a b c
Department of Neurosurgery, Huadong Hospital, Fudan University, Shanghai, China Department of Pathology, Huadong Hospital, Fudan University, Shanghai, China Department of General Surgery, Huadong Hospital, Fudan University, Shanghai, China
a r t i c l e
i n f o
Article history: Received 31 December 2016 Revised 21 June 2017 Accepted 9 July 2017 Available online xxxx Keywords: Capillary hemangiomas Intramedullary hemorrhage Intramedullary tumor Vascular malformation
a b s t r a c t A 48-year-old male patient had presented with worsening pain at extremities and body. The MRI showed an inhomogeneously enhancing lesion at C5-T1. During the surgical evacuation through a midline myelotomy, a frozen section could not find any tumor cells or vascular malformations. Immunohistochemically, the diagnosis of capillary hemangiomas was confirmed. © 2017 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Capillary hemangiomas (CHs) are benign vascular malformations, histologically characterized by irregular nodules of capillary-sized vessels lined by flattened endothelium without abnormal cells [1], which are very rare in spinal cord. Up to date, just only two cervicothoracic intramedullary CHs patients have been reported. Because of the extreme rarity, CHs were preoperatively misdiagnosed [2]. In this study, we discuss the clinic and surgical features of one patient with hemorrhagic CHs in cervicothoracic intramedullary location.
especially more serious at left side. The patient was still able to perceive light touch, pain and the proprioception was intact. 2.2. Radiological features
2. Case report
On T1-weighted or T2-weighted of Magnetic resonance images (MRI), the mass showed different abnormal signals in the cervicothoracic intramedullary spinal cord (C5 to T1) with slightly higher or slightly lower density shadow (Fig. 1A, B); On contrast-enhanced images, the mass was inhomogeneously enhancing about 53 mm × 11 mm (Fig. 1C). Spinal cord magnetic resonance angiography displayed the surface of C4-C6 spinal cord did show some abnormal vessels signals (Fig. 1D).
2.1. Clinical summary
2.3. Surgical procedure and pathologic findings
A 48-year-old man presented with a one month history of progressive numbness and pain. On examination, the muscular force or the reflex at extremities was normal throughout. Sensation was subjectively abnormal below the T3 to T4 dermatome,
Excepting some thicker veins on the surface of spinal cord, there was no obvious lesion after the C5-C7 and partial T1 laminectomies. The midline myelotomy at C5 was carefully performed, however, only the blood blot could be found, even at C6. During the surgery, the intraoperative frozen section likewise showed just a blood clot (Fig. 2). Considering not to cause more severe complications, the surgery was terminated after evacuation of most of the blood clot. Histologically, the mass was composed of capillary-sized blood vessels lined by simple endothelium, and the necrotic cells and inflammatory cells were also found (Fig. 3).
⁎ Corresponding author at: Huadong Hospital Affiliated to Fudan University, 221#, Yan'an Western Road, Jin'an District, Shanghai 200040, China. E-mail address: [email protected] (Y. Duan).
http://dx.doi.org/10.1016/j.inat.2017.07.007 2214-7519/© 2017 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
J. Li et al. / Interdisciplinary Neurosurgery: Advanced Techniques and Case Management 10 (2017) 78–80
79
Fig. 1. Preoperative MRI images. No syringomyelia. T1-weighted images (A); T2-weighted image (B); T1-weighted image with contrast enhancement (C); Spinal cord magnetic resonance angiography (D). Red arrows indicated the lesion. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Immunohistochemistry showed positive reaction for CD34 and CD31, while no positive staining for GFAP and Olig-2. The Ki 67 was 2%. The histologic diagnosis of capillary hemangiomas was confirmed. 3. Discussion Capillary hemangiomas involving the spinal cord are extremely rare, most in the extramedullary location [3]. To our knowledge, in the last three decades, only nineteen CHs patients involved in intramedullary location, most locating thoracic or lumbar spine
cord, have been reported. The only two previous cervicothoracic intramedullary patients received better results, presumably because of typical lobular tumor shapes and distinct tumor boundaries [2,4]. Central neuropathic pain could be the only initial symptom, but it has been reported to progress very quickly and seriously, if the dorsal horn is implicated [5]. In our study, postoperatively, the muscular strength and sensation were not impaired, but the pain invaded extremely severe at first few days. After taking oxycodone and eperisone, a muscle relaxant, the patient felt effectively relief without recurrence until discharge. The postoperative pain
80
J. Li et al. / Interdisciplinary Neurosurgery: Advanced Techniques and Case Management 10 (2017) 78–80
4. Conclusion Capillary hemangiomas are very rare and easily misdiagnosed in cervicothoracic intramedullary junction and may cause severe complications. If well demarcated, surgical evacuation of the lesion should be offered, even in this location, with potential of good outcome. Follow -up MRI should be applied in any instance, even if no pathology was found intraoperatively and on pathology. One must always assume an underlying vascular lesion that has been overlooked. Conflict of interest statement
Fig. 2. The hematoma cavity of the spinal cord. The blood blot with some loose bodies could be found.
The authors declare that they have no competing financial interests and personal relationships with other people or organizations that can inappropriately influence our work.
Fig. 3. Photomicrographs showing histopathological and immunohistochemical analysis. A Histopathological photomicrograph of the mass (H&E straining, ×200). The capillary vessels were lined by endothelial cells. Blood clots were found. B–C The cells were positive for CD 31 and CD34 respectively (×100).
management could be also very important for intramedullary CHs patient. The typical MRI images of CHs usually show isointense on T1weighted, hyperintense on T2-weigted and homogeneous enhancement after contrast injection [6]. The rich vascular signals could be found by MRI spinal cord angiography [7]. But if the hematoma arose in the lesion cavity, the tumor images may display heterogeneous density on MRI images, as this case. In previous studies, the typical CHs were well-demarcated, nodular lesions at the time of surgery [2], and could be diagnosed as “vascular tumor” by frozen quick section [6]. In this CHs, only old blood clots without other lesion were found on surgical vision, even frozen quick section could not give the accurate result. We lost faith to remove the rest of mass at C7 and T1, fearing to cause more severe unnecessary complications.
References [1] D.A. Nowak, D.C. Widenka, Spinal intradural capillary haemangioma: a review, Eur. Spine J. 10 (2001) 464–472. [2] L. Wu, X. Deng, C. Yang, Y. Xu, Intramedullary spinal capillary hemangiomas: clinical features and surgical outcomes: clinical article, J. Neurosurg. Spine 19 (2013) 477–484. [3] S.H. McEvoy, M. Farrell, F. Brett, S. Looby, Haemangioma, an uncommon cause of an extradural or intradural extramedullary mass: case series with radiological pathological correlation, Insights into imaging 7 (2016) 87–98. [4] K. Hida, M. Tada, Y. Iwasaki, H. Abe, Intramedullary disseminated capillary hemangioma with localized spinal cord swelling: case report, Neurosurgery 33 (1993) 1099–1101. [5] R. Babu, T.R. Owens, I.O. Karikari, J. Moreno, T.J. Cummings, O.N. Gottfried, et al., Spinal cavernous and capillary hemangiomas in adults, Spine 38 (2013) E423–E430. [6] R. Gonzalez, J. Spears, A. Bharatha, D.G. Munoz, Spinal lobular capillary hemangioma with an intramedullary component, Clin. Neuropathol. 33 (2014) 38–41. [7] M. Abe, K. Tabuchi, S. Tanaka, A. Hodozuka, K. Kunishio, N. Kubo, et al., Capillary hemangioma of the central nervous system, J. Neurosurg. 101 (2004) 73–81.