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Radiation–induced cavernous angioma in an adult Sir, Radiation–induced cavernoma (RIC) has been suspected since the first description by Ciricillo SF et al., in 1994.[1] Till January 2014 only 86 cases of RIC have been reported.[2] Here is yet another case report. A 31‑year‑old male presented with one episode of generalized tonic‑clonic seizure. He had a previous history of surgery for a suprasellar tumor followed by 60 Gy of radiation therapy at the age of 17. Magnetic resonance imaging (MRI) done at that time revealed a suprasellar mass and involving the optic chiasm [Figure 1]. MRI repeated as part of evaluation of seizure revealed a right medial temporal perisylvian heterointense lesion on T1‑/T2‑weighted images measuring 2 × 2.2 × 2.7 cms with blooming on gradient images suggesting cavernoma [Figure 2]. The MRI also showed post operative changes in the left temporal lobe and suprasellar region done 14 years back. On cerebral angiography the lesion was occult. Total excision of the cavernoma was done by fronto‑temporal craniotomy. Histopathological examination showed features consistent with cavernous angioma [Figure 3]. Repeat MRI done 14 years back showed no corresponding cavernoma. The temporal relation between the radiotherapy and appearance of cavernoma 14 years later suggests possible causal relation between the two.
a
b
c
d
Figure 1: Non contrast coronal (a), sagittal (c) and contrast enhanced coronal (b), sagittal (d) T1 weighted images show a homogenously enhancing mass lesion in the suprachiasmatic region and involving the optic chiasm (arrow)
a
b
c
d
The reported prevalence of cavernomas in general population is 0.5–0.6%.[3] Cavernomas can be sporadic and familial. The familial form accounts for 30% to 50% of cases harboring a cavernous malformation.[4] These lesions are considered to be congenital, but the lesions can rarely be acquired. Rarely these lesions have been observed in areas of normal brain (on prior MRI) after long years of radiation therapy for other brain lesions. Various hypotheses have been proposed to explain the pathogenesis of RIC: (1) radiation therapy induced capillary wall necrosis leads to endothelial swelling, dilatation of the vessel lumen, hyalinization and fibroses predisposing to cavernoma formation; (2) radiographically occult cavernomas present prior to radiotherapy subsequently grow to radiographically demonstrable cavernomas; (3) radiation triggered release of vascular endothelial growth factor may induce neoangiogenesis;[3] and (4) possible radiation induced somatic mutagenesis of Krev interaction trapped protein1 (KR1T1) encoded by the CCM1 gene may result in cavernoma formation.[4]
craniospinal radiation and chemotherapy following surgery. Vinchon M et al., retrospectively studied 552 patients irradiated for brain tumor under 18 years of age since 1970 and identified 60 cavernomas.[5] In an another study, Lew SM et al., identified 26 cavernomas in 59 patients aged 3–21 years irradiated for medulloblastoma.[6] These studies suggest that the reported worldwide prevalence of RIC is a gross under estimate. The reported prevalence in the study by Burn S et al., was more than 6 times the prevalence rate cited in the literature.[7] Cerebral cavernomas have been the most frequent MRI abnormality (57%) in 56 childhood leukemia survivors treated with cranial radiotherapy.[8]
RIC were predominantly reported in children suffering from medulloblastoma who had received
It is important to obtain clinical history pertaining to radiation therapy in radiographically diagnosed patients
572
Figure 2: Axial T2 (a) weighted image shows heterogenous hyperintense lesion in the right temporal region (arrow). Gradient axial image (b) shows blooming suggesting cavernoma (arrow). Contrast enhanced Coronal T1 weighted images (c,d) show negligible enhancement within the lesion (arrow). Previous post operative changes are seen in the suprachiasmatic region (arrow head) and left temporal lobe (open arrow)
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for t journal Letters to Editor
7. Burn S, Gunny R, Phipps K, Gaze M, Hayward R. Incidence of cavernoma development in children after radiotherapy for brain tumors J Neurosurg 2007;106:379‑83. 8. Faraci M, Morana G, Bagnasco F, Barra S, Polo P, Hanau G, et al. Magnetic resonance imaging in childhood leukemia survivors treated with cranial radiotherapy: A cross sectional, single center study. Pediatr Blood Cancer 2011;57:240‑6. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144512
Figure 3: Histopathological examination showing vascular spaces of varying sizes filled with RBCs suggesting cavernous angioma. No evidence of atypia, mitosis or necrosis (H and E, x40)
of cerebral cavernomas. And patients undergoing radiotherapy, regardless of their age, should have regular follow‑up with MRI for any RIC. We support the suggestion by Burn S et al., for mentioning RIC as a possible complication when consent for radiation therapy is obtained so that the patients understand and their compliance for long term follow‑up is better.[7] Once the diagnosis of RIC has been confirmed, it should be treated as any other cavernoma since the natural history remains the same for both categories of cavernomas.
Damodar Rout, Geetha Sharmi K. M., Rajeswaran R.1 Departments of Neurosurgery and 1Radiology, Sri Ramachandra University, Chennai, Tamil Nadu, India E-mail: [email protected]
References 1. Circillo SF, Cogen PH, Edwards MS. Pediatric cryptic vascular malformations: Presentation, diagnosis and treatment. Pediatr Neurosurg 1994;20:137‑47. 2. Ruggeri AG, Donnarumma P, Pichierri A, Delfini R. Two cystic cavernous angiomas after radiotherapy for atypical meningioma in adult woman: Case report and literature review. J Korean Neurosurg Soc 2014;55:40‑2. 3. Walch J, Tettenborn B, Weber J, Hundsberger T. Radiation–induced cavernoma after total body irradiation and haematopoietic stem cell transplantation in an adult patient suffering from acute myeloid leukemia. Case Rep Neurol 2013;5:91‑7. 4. Quinones–Hinjosa A. Schmidek and sweet operative neurosurgical techniques: indications, methods and results. In: Patel AP, Amin‑Hinjani S, Ogilvy CS, editors. Surgical Management of Cavernous Malformations of the Nervous System. 6th ed. Vol: 11. Philadelphia: Elsevier Saunders; 2012. p. 977‑93. 5. Vinchon M, Leblond P, Carson S, Delestret I, Baroncini M, Coche B. Radiation–induced tumors in children irradiated for brain tumor: A longitudinal study. Childs Nerv Syst 2011;27:445‑53. 6. Lew SM, Morgan JN, Psaty E, Lefton DR, Allen JC, Abbott R. Cumulative incidence of radiation–induced cavernomas in long‑term survivors of medulloblastoma. J Neurosurg 2006;104:103‑7. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
Received: 09-10-2014 Review completed: 10-10-2014 Accepted: 21-10-2014
Acute severe backache: Do not forget to look beneath the disc Sir, Schmorl’s nodes or intervertebral disc herniations are commonly seen incidental finding on imaging, often asymptomatic. However, acute Schmorl node, also known as acute cartilaginous node, is a rare and perhaps under‑recognized entity, which can cause intense localized back pain. In this article, we describe one such case. A 35‑year‑old otherwise healthy male presented with acute severe low backache of two days duration. There was no history to suggest radicular pain or claudication. Pain was localized to lower lumbar region. Neurologic examination was essentially normal. Magnetic resonance imaging (MRI) of lumbosacral spine [Figures 1 and 2] revealed intravertebral disk herniation through the superior endplate of L3 and S1 vertebrae with bone marrow edema surrounding the herniated nucleus pulposus. Schmorl’s node classically known as intervertebral disc herniation was first described by Schmorl in 1927.[1] Generally, the Schmorl nodes are considered to be an asymptomatic incidental finding on imaging. Non‑acute asymptomatic Schmorl nodes are common spinal abnormalities and are found in 38%‑75% of the population.[2] A recent study reported that the majority of Schmorl nodes are located in the upper lumbar levels with the highest prevalence in L2/3 level, whereas earlier studies showed the location more common in the T7‑L1 region.[1,3] MRI studies of lumbar spine in patients with low back pain have shown almost double the frequency of Schmorl nodes in the symptomatic group as compared to the control 573
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Radiation–induced cavernous angioma in an adult Sir, Radiation–induced cavernoma (RIC) has been suspected since the first description by Ciricillo SF et al., in 1994.[1] Till January 2014 only 86 cases of RIC have been reported.[2] Here is yet another case report. A 31‑year‑old male presented with one episode of generalized tonic‑clonic seizure. He had a previous history of surgery for a suprasellar tumor followed by 60 Gy of radiation therapy at the age of 17. Magnetic resonance imaging (MRI) done at that time revealed a suprasellar mass and involving the optic chiasm [Figure 1]. MRI repeated as part of evaluation of seizure revealed a right medial temporal perisylvian heterointense lesion on T1‑/T2‑weighted images measuring 2 × 2.2 × 2.7 cms with blooming on gradient images suggesting cavernoma [Figure 2]. The MRI also showed post operative changes in the left temporal lobe and suprasellar region done 14 years back. On cerebral angiography the lesion was occult. Total excision of the cavernoma was done by fronto‑temporal craniotomy. Histopathological examination showed features consistent with cavernous angioma [Figure 3]. Repeat MRI done 14 years back showed no corresponding cavernoma. The temporal relation between the radiotherapy and appearance of cavernoma 14 years later suggests possible causal relation between the two.
a
b
c
d
Figure 1: Non contrast coronal (a), sagittal (c) and contrast enhanced coronal (b), sagittal (d) T1 weighted images show a homogenously enhancing mass lesion in the suprachiasmatic region and involving the optic chiasm (arrow)
a
b
c
d
The reported prevalence of cavernomas in general population is 0.5–0.6%.[3] Cavernomas can be sporadic and familial. The familial form accounts for 30% to 50% of cases harboring a cavernous malformation.[4] These lesions are considered to be congenital, but the lesions can rarely be acquired. Rarely these lesions have been observed in areas of normal brain (on prior MRI) after long years of radiation therapy for other brain lesions. Various hypotheses have been proposed to explain the pathogenesis of RIC: (1) radiation therapy induced capillary wall necrosis leads to endothelial swelling, dilatation of the vessel lumen, hyalinization and fibroses predisposing to cavernoma formation; (2) radiographically occult cavernomas present prior to radiotherapy subsequently grow to radiographically demonstrable cavernomas; (3) radiation triggered release of vascular endothelial growth factor may induce neoangiogenesis;[3] and (4) possible radiation induced somatic mutagenesis of Krev interaction trapped protein1 (KR1T1) encoded by the CCM1 gene may result in cavernoma formation.[4]
craniospinal radiation and chemotherapy following surgery. Vinchon M et al., retrospectively studied 552 patients irradiated for brain tumor under 18 years of age since 1970 and identified 60 cavernomas.[5] In an another study, Lew SM et al., identified 26 cavernomas in 59 patients aged 3–21 years irradiated for medulloblastoma.[6] These studies suggest that the reported worldwide prevalence of RIC is a gross under estimate. The reported prevalence in the study by Burn S et al., was more than 6 times the prevalence rate cited in the literature.[7] Cerebral cavernomas have been the most frequent MRI abnormality (57%) in 56 childhood leukemia survivors treated with cranial radiotherapy.[8]
RIC were predominantly reported in children suffering from medulloblastoma who had received
It is important to obtain clinical history pertaining to radiation therapy in radiographically diagnosed patients
572
Figure 2: Axial T2 (a) weighted image shows heterogenous hyperintense lesion in the right temporal region (arrow). Gradient axial image (b) shows blooming suggesting cavernoma (arrow). Contrast enhanced Coronal T1 weighted images (c,d) show negligible enhancement within the lesion (arrow). Previous post operative changes are seen in the suprachiasmatic region (arrow head) and left temporal lobe (open arrow)
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for t journal Letters to Editor
7. Burn S, Gunny R, Phipps K, Gaze M, Hayward R. Incidence of cavernoma development in children after radiotherapy for brain tumors J Neurosurg 2007;106:379‑83. 8. Faraci M, Morana G, Bagnasco F, Barra S, Polo P, Hanau G, et al. Magnetic resonance imaging in childhood leukemia survivors treated with cranial radiotherapy: A cross sectional, single center study. Pediatr Blood Cancer 2011;57:240‑6. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144512
Figure 3: Histopathological examination showing vascular spaces of varying sizes filled with RBCs suggesting cavernous angioma. No evidence of atypia, mitosis or necrosis (H and E, x40)
of cerebral cavernomas. And patients undergoing radiotherapy, regardless of their age, should have regular follow‑up with MRI for any RIC. We support the suggestion by Burn S et al., for mentioning RIC as a possible complication when consent for radiation therapy is obtained so that the patients understand and their compliance for long term follow‑up is better.[7] Once the diagnosis of RIC has been confirmed, it should be treated as any other cavernoma since the natural history remains the same for both categories of cavernomas.
Damodar Rout, Geetha Sharmi K. M., Rajeswaran R.1 Departments of Neurosurgery and 1Radiology, Sri Ramachandra University, Chennai, Tamil Nadu, India E-mail: [email protected]
References 1. Circillo SF, Cogen PH, Edwards MS. Pediatric cryptic vascular malformations: Presentation, diagnosis and treatment. Pediatr Neurosurg 1994;20:137‑47. 2. Ruggeri AG, Donnarumma P, Pichierri A, Delfini R. Two cystic cavernous angiomas after radiotherapy for atypical meningioma in adult woman: Case report and literature review. J Korean Neurosurg Soc 2014;55:40‑2. 3. Walch J, Tettenborn B, Weber J, Hundsberger T. Radiation–induced cavernoma after total body irradiation and haematopoietic stem cell transplantation in an adult patient suffering from acute myeloid leukemia. Case Rep Neurol 2013;5:91‑7. 4. Quinones–Hinjosa A. Schmidek and sweet operative neurosurgical techniques: indications, methods and results. In: Patel AP, Amin‑Hinjani S, Ogilvy CS, editors. Surgical Management of Cavernous Malformations of the Nervous System. 6th ed. Vol: 11. Philadelphia: Elsevier Saunders; 2012. p. 977‑93. 5. Vinchon M, Leblond P, Carson S, Delestret I, Baroncini M, Coche B. Radiation–induced tumors in children irradiated for brain tumor: A longitudinal study. Childs Nerv Syst 2011;27:445‑53. 6. Lew SM, Morgan JN, Psaty E, Lefton DR, Allen JC, Abbott R. Cumulative incidence of radiation–induced cavernomas in long‑term survivors of medulloblastoma. J Neurosurg 2006;104:103‑7. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
Received: 09-10-2014 Review completed: 10-10-2014 Accepted: 21-10-2014
Acute severe backache: Do not forget to look beneath the disc Sir, Schmorl’s nodes or intervertebral disc herniations are commonly seen incidental finding on imaging, often asymptomatic. However, acute Schmorl node, also known as acute cartilaginous node, is a rare and perhaps under‑recognized entity, which can cause intense localized back pain. In this article, we describe one such case. A 35‑year‑old otherwise healthy male presented with acute severe low backache of two days duration. There was no history to suggest radicular pain or claudication. Pain was localized to lower lumbar region. Neurologic examination was essentially normal. Magnetic resonance imaging (MRI) of lumbosacral spine [Figures 1 and 2] revealed intravertebral disk herniation through the superior endplate of L3 and S1 vertebrae with bone marrow edema surrounding the herniated nucleus pulposus. Schmorl’s node classically known as intervertebral disc herniation was first described by Schmorl in 1927.[1] Generally, the Schmorl nodes are considered to be an asymptomatic incidental finding on imaging. Non‑acute asymptomatic Schmorl nodes are common spinal abnormalities and are found in 38%‑75% of the population.[2] A recent study reported that the majority of Schmorl nodes are located in the upper lumbar levels with the highest prevalence in L2/3 level, whereas earlier studies showed the location more common in the T7‑L1 region.[1,3] MRI studies of lumbar spine in patients with low back pain have shown almost double the frequency of Schmorl nodes in the symptomatic group as compared to the control 573
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
