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Rapid Progression of Gliomatosis Cerebri to Secondary Glioblastoma

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Rapid Progression of Gliomatosis Cerebri to Secondary Glioblastoma Case Report pISSN 1738-2637 / eISSN 2288-2928 J Korean Soc Radiol 2017;76(3):221-228 https://doi.org/10.3348/jksr.2017.76.3.221 Rapid Progression of Gliomatosis Cerebri to Secondary Glioblastoma, Factors That Affect the Progression Rate: A Case Report 대뇌신경아교종증에서 빠른 진행을 보인 이차성 다형성아교모세포종, 진행속도에 영향을 미친 인자에 대한 증례 보고 Hee Kyung Kim, MD1, In Kyu Yu, MD, PhD1*, Seung Min Kim, MD2, Joo Heon Kim, MD3, Seung Hoon Lee, MD2, Seung Yeon Lee, MD3 Departments of 1Radiology, 2Neurosurgery, Eulji University Hospital, Daejeon, Korea 3Department of Pathology, Eulji University School of Medicine, Daejeon, Korea Glioblastomas may develop de novo or through progression from low-grade or ana- plastic astrocytomas. The term ‘primary glioblastoma’ refers to a glioblastoma that Received May 11, 2016 lacks a precursor lesion and has a clinical history of less than three months. On the Revised July 13, 2016 Accepted August 13, 2016 other hand, the term ‘secondary glioblastoma’ indicates that the glioblastoma has *Corresponding author: In Kyu Yu, MD progressed from a low-grade tumor after a long latency period and often manifests Department of Radiology, Eulji University Hospital, 95 Dunsanseo-ro, Seo-gu, Daejeon 35233, Korea. in younger patients. These subtypes of glioblastoma develop via different genetic Tel. 82-42-611-3581 Fax. 82-42-611-3567 pathways, and they differ in prognosis and response to therapy. Thus, differential E-mail: [email protected] diagnosis of these subtypes and prediction of the factors that affect the progression This is an Open Access article distributed under the terms from low-grade diffuse astrocytoma to secondary glioblastoma would be clinically of the Creative Commons Attribution Non-Commercial very important. We present a rare case of secondary glioblastoma, which developed License (http://creativecommons.org/licenses/by-nc/4.0) only three months after the follow up imaging evaluations, with a history of low which permits unrestricted non-commercial use, distri- bution, and reproduction in any medium, provided the grade glioma, and present the factors that cause rapid progression. original work is properly cited. Index terms Glioblastoma Secondary IDH1 Protein, Human Disease Progression INTRODUCTION of secondary glioblastoma (2). Differential diagnosis of these subtypes of glioblastomas is Glioblastomas may develop de novo or through progression important to therapeutic approaches. The ability to predict the from low-grade or anaplastic astrocytomas. Primary glioblasto- pace of progression from low-grade diffuse astrocytoma to sec- mas present as full-blown tumors, with rapid development of ondary glioblastoma would be clinically very important. Previ- clinical symptoms, without clinical, radiological, or histopatho- ous studies reported that the factors that cause shortening of logical evidence of a less-malignant precursor lesion (1). On the the time of progression from low-grade glioma to glioblastoma other hand, secondary glioblastomas develop slowly through were events such as TP53 protein mutation, IDH1 gene muta- progression from low-grade diffuse astrocytoma or anaplastic tion, and hypoxia (3, 4). However, to the best of our knowledge, astrocytoma. The mean time for progression from low-grade as- there are no radiological case reports of correlation with these trocytoma to secondary glioblastoma was 55 months in a study factors that shorten the time of progression to secondary glio- Copyrights © 2017 The Korean Society of Radiology 221 Rapid Progression of Gliomatosis Cerebri to Secondary Glioblastoma, Factors That Affect the Progression Rate blastoma. We present a rare case of secondary glioblastoma which with normal brain tissue, supposedly caused by a decrease in developed only three months after the initial detection of glioma- NAA, reflects replacement of neurons by neoplastic glial cells. It tosis cerebri and discuss the factors that cause rapid progression. is consistent with a neoplastic lesion and characteristic findings of WHO grade II glioma. Thus, we suggested that this lesion CASE REPORT was gliomatosis cerebri or a multicentric low-grade glioma. Other noticeable imaging findings were mild chronic isch- An 80-year-old male presented with syncope and scalp bleed- emic change in the left border zonal frontoparietal white matter ing he had a history of myocardial infarction, which was treated area. On CT angiography, total occlusion of the left proximal 7 years ago with coronary angiography and stent insertion, and portion of the middle cerebral artery was detected (Fig. 2A). he has been taking aspirin for cardiovascular disease. His vital However, collaterals to the distal run-off were seen faintly. On signs were stable and there were no abnormal laboratory find- transfemoral cerebral angiography (TFCA), total occlusion of ings. He complained of symptoms of memory loss at the time the middle cerebral artery at the proximal level was seen and col- of syncope. To identify any traumatic injury or brain lesions, lateral flow via the posterior communicating artery through the contrast-enhanced multi-detector computed tomography im- vertebral artery was detected. Also, there were asymmetrically ages were obtained with a 32 channel dual source CT scanner prominent deep medullary veins in the left hemisphere, sugges- (SOMATOM Definition; Siemens Medical Solutions, Forch- tive of increased oxygen extraction on susceptibility weighted heim, Germany) and magnetic resonance images (MRI) were images (Fig. 2B). On perfusion images, mean transit time (MTT) obtained with 3T; MRI scanner (MAGNETOM Skyra 3T, Sie- parameter maps were significantly asymmetric with a large area mens Medical Systems, Erlangen, Germany). of increased intensity which indicates prolonged MTT in the left On the CT scan, wide regional, patchy and decreased attenua- border zone of the frontal, parietal, and temporal periventricular tion lesions involving the left insula, the left subinsula, the left white matter areas in the left middle cerebral artery territory, hippocampus, the left anterior temporal lobe, left parahippo- suggesting the presence of a large penumbra or oligemia (Fig. campus, and some of the left frontal and high parietal cortices 2C, D). and white matter were detected along with mild gyral efface- We decided to perform conservative treatment without biopsy, ment. On MRI, these wide, regional and patchy lesions showed and during the follow-up, the patient did not present any other high signal intensity (SI) on T2-weighted images (WI) and fluid symptoms and there was no change in imaging evaluations. attenuation inversion recovery (FLAIR) images (Fig. 1A, B) and After three months, our patient visited the emergency depart- slightly increased SI on diffusion weighted imaging and appar- ment with a complaint of right hemiparesis and fine motor dis- ent diffusion coefficient maps. On contrast-enhanced T1WI turbance which had started five days ago. On laboratory exami- images, these lesions showed decreased SI without an enhanc- nations, there were no abnormal findings. There were no interval ing lesion (Fig. 1C, D). On perfusion MRI, the cerebral blood changes in the left anterior temporal, insular, and subinsular ar- volume (CBV) parameter map did not show increased signal at eas, which showed a diffuse regional hypodense lesion without the level of high SI detected on conventional images (Fig. 1E, F). enhancement on contrast-enhanced CT and high SI on T2WI. The spectroscopic findings showed an increased choline peak However, a focal, lobulated, unevenly thickened, enhancing le- and a markedly decreased N-acetylaspartate (NAA) peak in the sion with newly developed central necrosis was observed in the diffuse ill-demarcated patchy lesion involving the left anterior left high parietal white matter and cortical areas, previously parahippocampal temporal area (Fig. 1G). Diffuse hypoattenu- seen as non-enhancing hyperintense signal on T2WI (Fig. 3A). ating lesion on CT and bilateral poorly defined areas of high Peritumoral edema was prominently visible on the T2WI and T2SI represent tumor spread and lack of enhancement indi- FLAIR images (Fig. 3B). There was diffusion restriction in the cates a preserved blood-brain barrier. This lesion involved three thickened wall of the mass, but not in the central necrotic por- lobes of the brain with preservation of the underlying struc- tion (Fig. 3C, D). On perfusion MRI, a markedly increased tures. Increased choline/NAA peak in the tumor as compared CBV in the newly developed interval enhancing peripheral wall 222 J Korean Soc Radiol 2017;76(3):221-228 jksronline.org Hee Kyung Kim, et al A B C D E F G Fig. 1. Initial contrast-enhanced conventional, perfusion MRI, and MR spectroscopy in an 80-year-old male, suggesting gliomatosis cerebri. A-D. Fluid attenuation inversion recovery (FLAIR) images show diffuse swelling with increased signal intensity and mild gyral effacement involv- ing the left anterior temporal, the left parahippocampal, some of the left frontal cortical and white matter areas (A) as well as the left high pari- etal white matter and some cortical areas (B). Gadobenate dimeglumine-enhanced T1-weighted MRI shows slightly decreased signal without an enhancing lesion in the corresponding sites (C, D). E, F. Cerebral blood volume parameter map shows an increased signal at the level of high signal intensity detected on conventional MRI. G. On MR spectroscopy, an increased choline peak and a markedly decreased N-acetylaspartate peak are seen in the diffuse ill-demarcated patchy high
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