5 Cavernous Malformations as Dynamic Lesions: De Novo Formation, Radiologic Changes, and Radiation-Induced Forms

Author(s):  
Gildas Patet ◽  
Andrea Bartoli ◽  
Torstein R. Meling

AbstractRadiation-induced cavernous malformations (RICMs) are delayed complications of brain irradiation during childhood. Its natural history is largely unknown and its incidence may be underestimated as RCIMS tend to develop several years following radiation. No clear consensus exists regarding the long-term follow-up or treatment. A systematic review of Embase, Cochrane Library, PubMed, Google Scholar, and Web of Science databases, following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was performed. Based on our inclusion/exclusion criteria, 12 articles were included, totaling 113 children with RICMs, 86 were treated conservatively, and 27 with microsurgery. We were unable to precisely define the incidence and natural history from this data. The mean age at radiation treatment was 7.3 years, with a slight male predominance (54%) and an average dose of 50.0 Gy. The mean time to detection of RICM was 9.2 years after radiation. RICM often developed at distance from the primary lesion, more specifically frontal (35%) and temporal lobe (34%). On average, 2.6 RICMs were discovered per child. Sixty-seven percent were asymptomatic. Twenty-one percent presented signs of hemorrhage. Clinical outcome was favorable in all children except in 2. Follow-up data were lacking in most of the studies. RICM is most often asymptomatic but probably an underestimated complication of cerebral irradiation in the pediatric population. Based on the radiological development of RICMs, many authors suggest a follow-up of at least 15 years. Studies suggest observation for asymptomatic lesions, while surgery is reserved for symptomatic growth, hemorrhage, or focal neurological deficits.


Neurosurgery ◽  
2004 ◽  
Vol 55 (6) ◽  
pp. E1435-E1439 ◽  
Author(s):  
Pascal Jabbour ◽  
Judith Gault ◽  
Steven E. Murk ◽  
Issam A. Awad

Abstract OBJECTIVE AND IMPORTANCE: This is the first reported case of histologically proven multiple spinal cavernous malformations (CMs) associated with previous irradiation. There are only two cases reported in the literature of solitary spinal CM after irradiation. In addition, the lesions in our patient had an atypical magnetic resonance imaging appearance mimicking intraspinal drop metastasis. CLINICAL PRESENTATION: A 33-year-old man had an incidental finding of multiple enhancing intraspinal lesions as revealed by magnetic resonance imaging during staging tests for hepatocellular carcinoma. He had a history of Wilms' tumor at a young age with irradiation to the abdomen and pelvis. His family history included a paternal cousin with multiple cerebral CMs. The diagnosis of spinal drop metastasis was made, and further intervention was undertaken for confirmation. INTERVENTION: The patient underwent a lumbar laminectomy with durotomy and excision of two of the lesions. Macroscopic analysis revealed mulberry-like appearance with nerve root involvement, and pathological analysis confirmed the diagnosis of CM. Genetic testing of the patient and his affected cousin was negative for the CCM1 gene. CONCLUSION: The occurrence of multiple spinal lesions in the context of known neoplasia indicates a diagnosis of metastasis. Spinal CMs were not suspected preoperatively because of the atypical appearance revealed by magnetic resonance imaging scans, with uniform contrast enhancement and absence of hemosiderin rim. This case report is discussed relative to previous literature regarding radiation-induced CMs and other known causes of the disease.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 11024-11024
Author(s):  
Loic Lebellec ◽  
Francois Bertucci ◽  
Emmanuelle Tresch-Bruneel ◽  
Isabelle Laure Ray-Coquard ◽  
Axel Le Cesne ◽  
...  

11024 Background: WP is an active regimen for treatment of AS pts (Ray-Coquard JCO 2015). We report here the correlative analysis conducted during a phase 2 trial assessing WP +/- B. Methods: Circulating pro/anti-angiogenic factors (FGF, PlGF, SCF, Selectin, thrombospondin, VEGF, VEGF-C) were collected at D1 and D8. Prognostic value for PFS was assessed using Cox model (biomarkers as continuous variables). We attempt to identify subgroups of pts benefiting from adding B using interaction tests (predictive factors). Results: Among the 51 pts enrolled in this trial, 45 were analyzable: 20 in Arm A (WP without B) and 25 in Arm B (with B). Median PFS was 5.5 and 6.1 months, respectively (p = 0.84). Samples were collected in 45 pts at D1 and 42 pts at D1 and 8. Baseline biomarkers were similar in both arms (excluding Selectin, significantly lower in arm A: median of 25 vs. 35 ng/mL, p = 0.03). In arm A, there was no significant difference between values at D1 and D8. In arm B, there were a significant decrease in VEGF (from a median of 0.49 to 0.08 ng/mL; p < 0.01) and selectin (from a median of 35.3 to 31.7 ng/mL; p < 0.01), and a significant increase in PlGF (from a median of 16.1 to 30.0 pg/mL; p < 0.01). In univariate analysis, factors associated with PFS were: de novo vs. radiation-induced AS (HR = 2.39 (p < 0.01), visceral vs. superficial AS (HR = 2.04; p < 0.03), VEGF-C at D1 (HR = 0.77; p < 0.03), FGF at D8 (HR = 1.17; p < 0.01), difference in FGF D8-D1 (HR = 1.24; p < 0.01), and PlGF value at D1 (HR = 1.02; p < 0.05). In multivariate analysis, factors associated with PFS were: de novo AS (HR = 2.39; p = 0.03), VEGF-C at D1 (HR = 0.73; p < 0.02) and FGF difference between D8 and D1 (HR = 1.16; p < 0.02). None of these factors were associated with benefit of adding B. Conclusions: Baseline VEGF-C levels and change in FGF were independent prognostic factors in pts with or without B. Addition of B significantly decreased the level of circulating VEGF and selectin and increased the level of circulating PlGF in AS patients. We did not identify subgroup of pts benefiting from adding of B to WP. Clinical trial information: NCT01303497.


2017 ◽  
Vol 23 (5) ◽  
pp. 510-515 ◽  
Author(s):  
Katsuhiro Mizutani ◽  
Tomoru Miwa ◽  
Takenori Akiyama ◽  
Tokunori Kanazawa ◽  
Hideaki Nagashima ◽  
...  

Dural sinus malformation (DSM) is a rare paediatric vascular malformation characterised by abnormal dilation of the posterior dural sinus. Owing to its rarity, the pathophysiology of DSM has not been fully elucidated. We report a case of prenatally diagnosed DSM with an unusual clinical course. We detected DSM in a male foetus in the 26th week of gestation by using foetal ultrasonography. Although the DSM regressed during the foetal stage and the arteriovenous shunt was insignificant in the neonate, the shunt rapidly developed four months after birth. The neonate also had postnatal de novo brainstem cavernous malformation (CM), which also developed rapidly, supposedly due to the aggravated venous hypertension resulting from the DSM. We successfully treated the aggravated shunts by endovascular transarterial and transvenous embolisation six times over two years and, subsequently, the clinical condition and the size of the brainstem CM became stable. The DSM and CM seemed to have a metameric origin. Such aberrant cases could help to further the understanding of DSM.


2012 ◽  
Vol 47 (3) ◽  
pp. 475-480 ◽  
Author(s):  
Lorena Mosca ◽  
Silvana Pileggi ◽  
Francesca Avemaria ◽  
Claudia Tarlarini ◽  
Maria Sole Cigoli ◽  
...  

2020 ◽  
Vol 10 (3) ◽  
Author(s):  
Myrto Palkopoulou ◽  
Eleni Bakola ◽  
Marina Foliadi ◽  
Petros Stefanidis ◽  
P. Teresa Acquaviva

The cerebral cavernous malformations are benign vascular hamartomas, with thin and dilated vascular walls and therefore constantly susceptible to hemorrhage. Clinically, they present with recurrent headaches, acute intracranial hemorrhage and focal neurological deficits. They are considered as angiographically occult vascular malformations and the imaging technique of choice for their diagnosis is magnetic resonance tomography. We present the case of a female patient with acute-onset symptomatology, congruent with a lesion in the basal pons. Her medical history included an intracranial hemorrhage due to a cavernoma, which was surgically removed. The magnetic resonance imaging of the brain revealed two new cavernomas, which were not identified in the imaging conducted in the past. In literature, the cases of de novo appearance of cavernomas are considered highly rare, especially in patients with no consistent family history or medical history of radiation therapy. Resultantly, they should be considered as dynamic lesions, regarding their number, size and behavior.


2016 ◽  
Vol 154 (5) ◽  
pp. 880-887 ◽  
Author(s):  
Gerald Ci-An Tay ◽  
N. Gopalakrishna Iyer ◽  
Whee-Sze Ong ◽  
David Tai ◽  
Mei-Kim Ang ◽  
...  

2008 ◽  
Vol 41 (2) ◽  
pp. 329-339 ◽  
Author(s):  
Jonathan P. Wright ◽  
Céline Besnard ◽  
Irene Margiolaki ◽  
Sebastian Basso ◽  
Fabrice Camus ◽  
...  

The preparation of single crystals suitable for X-ray analysis is frequently the most difficult step in structural studies of proteins. With the aid of two examples, it is shown thatde novosolution of the crystallographic phase problem can be achieved at low resolution using microcrystalline powder samplesviathe single isomorphous replacement method. With synchrotron radiation and optimized instrumentation, high-quality powder patterns have been recorded, from which it was possible to generate phase information for structure factors up to 6 Å resolution. pH- and radiation-induced anisotropic lattice changes were exploited to reduce the problem of overlapping reflections, which is a major challenge in protein powder diffraction. The resulting data were of sufficient quality to compute molecular envelopes of the protein molecule and to map out the solvent channels in the crystals. The results show that protein powder diffraction can yield low-resolution data that are potentially useful for the characterization of microcrystalline proteins as novel micro- and mesoporous materials as well as for structural studies of biologically important macromolecules.


2016 ◽  
Vol 29 (6) ◽  
pp. 458-464 ◽  
Author(s):  
Hariprakash Chakravarthy ◽  
Tzu-Kang Lin ◽  
Yao-Liang Chen ◽  
Yi-Ming Wu ◽  
Chin-Hua Yeh ◽  
...  

This is a case report of de novo development of two cerebral cavernous malformations adjacent to existing developmental venous anomalies. The development of cavernomas was noted over a follow-up period of 10 years. These developments happened during the course of staged endovascular management of a complex dural arterio-venous fistula along the right sphenoid wing. The patient presented with a proptosis secondary to lympho-haemangiomatous lesion of the fronto-orbital region and a high-flow right sphenoid wing dural arterio-venous fistula. During the initial period of conservative management of the dural arterio-venous fistula, he developed de novo cavernous malformations in the left mesial temporal lobe adjacent to a developmental venous anomaly in the temporal lobe, and along with this there was engorgement of deep veins related to another existing developmental venous anomaly in the brainstem. Later during the course of endovascular treatment of the dural arterio-venous fistula, a large brainstem cavernoma developed adjacent to the brainstem developmental venous anomaly. This case report discusses the cause-effect relationship of venous pressure changes related to management of dural arterio-venous fistula and de novo formation of cerebral cavernous malformations adjacent to existing developmental venous anomalies.


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