Gliomatosis Cerebri: Imaging Findings on Traditional and Advanced Techniques

Transcription

1 Gliomatosis Cerebri: Imaging Findings on Traditional and Advanced Techniques Poster No.: C-1464 Congress: ECR 2015 Type: Educational Exhibit Authors: L. M. Cruz Hernandez, I. Herrera, A. L. Reyes Ortiz, E. Capilla, R. MORENO DE LA PRESA, B. GUTIERREZ MARTINEZ, R. G G. Gonzalez, J. M. Garcia Benassi ; Toledo/ES, MADRID/ES Keywords: Neuroradiology brain, CT, MR, Education, Neoplasia DOI: /ecr2015/C-1464 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 29

2 Learning objectives The purpose of this exhibit is: 1. To review general GC issues. 2. To show characteristic imaging findings at computed tomography (CT) and magnetic resonance (MR) and its correlation with microscopic pathology features. 3. To explain the utility of MR advanced techniques in the diagnosis. 4. To illustrate differences between GC and its principal differential using sample cases and diagnostic algorithms. Background Gliomatosis cerebri (GC) is a rare diffusely infiltrating usually bilateral glial tumor involving at least 3 lobes (Figure 1). Its etiology is controversial and is classified as neoplasm of unknown histogenesis. The prognosis is generally poor, survival ranges from weeks to years. The imaging appearances of GC may be similar to more common diseases like arteriolosclerosis or herpes encephalitis, but the clinical presentations differ. We performed a literature review of the gliomatosis cerebri as well a review of cases diagnosed and treated in our institution during the period from January 2011 to January With the data obtained we made a review of epidemiology, natural history and clinical presentation of GC, imaging findings and the main differential diagnoses. Images for this section: Page 2 of 29

3 Fig. 1: Gliomatosis cerebri (GC) is a rare diffusely infiltrating usually bilateral glial tumor involving at least 3 lobes. 55 year old man with mental status alteration and episodic agitation. Page 3 of 29

4 Findings and procedure details According to the criteria of the World Health Organization (WHO) classification of tumours of the CNS, gliomatosis cerebri infiltrates but preserves the architecture of the surrounding neural tissues and may infiltrate both cortical gray matter as basal ganglia, and extend along the brainstem and spine. GC is usually a bilateral tumor (Figure 2). Clinical Issues (Figure 3) The range of age at diagnosis going from the first months of life to old age, but most patients are diagnosed beteween fourth and fifth decade. The incidence of GC is higher in males than in women with an index of 1.3:1. The clinical presentation is variable and is not an aid to the diagnosis: symptoms include headache, seizures, dementia, behavioral changes, lethargy and others. GC has a poor prognosis. Good prognostic factors that influence survival include: Karnofsky index #70-80, grade and histologic subtype and age at diagnosis. Classification (Figure 4, 5, 6) Gliomatosis has a biological behavior generally aggressive by the degree of infiltration involved, that is why the WHO classified as a grade III malignancy. Have been described two cell types with defined genetic alterations: TYPE 1: The GC type 1 that shares characteristics with infiltrating astrocytomas and is associated with the TP53 mutation. TYPE 2: Which is less common and where in the predominant cell type are oligodendrocytes. It is associated with a deletion in chromosome 1p. Two gross pathologic gliomatosis cerebri types are recognized and can be applied to image: Page 4 of 29

5 TYPE 1: Neoplastic overgrowth with expansion on the existing structures without circumscribed tumor or mass. TYPE 2: Diffuse lesion with focal neoplastic mass with malignant features, may develop from type 1. Teaching Point " When other types of gliomas have a large infiltrative progression rather than growth of the tumor mass itself it is called secondary GC" Imaging general features (Figure 7, 8) Best diagnostic clue: T2 hyperintense infiltrating mass with enlargement of involved structures. Typically no or minimal enhancement. GC shows too marked elevation of myo-inositol. CT Findings (Figure 9, 10, 11) NECT Poorly defined, asymmetric low density (often subtle). Loss of gray-white differentiation with expansion and mild mass effect. CECT In contrast CT typically the gliomatosis does not enhance. MR Findings at Usual Sequences (Figure 12, 13) T1WI Isointense or hypointense infiltrating mass. Page 5 of 29

6 Typically homogeneous. T2WI Homogeneous hyperintense infiltrating mass. Mass effect with mild diffuse sulcal and ventricular effacement. FLAIR Homogeneous hyperintense infiltrating mass. T1C+ Typically no or minimal enhancement. Patchy enhancement rarely. Enhancement may indicate malignant progression or focus of malignant glioma. Imaging Features in Advanced MR Techniques (Figure 14, 15, 16) Diffusion MRI Usually no restriction. Perfusion MRI Low rcbv : Suggests low grade tumor. High rcbv: Suggests higher grade tumor. MR spectroscopy NAA decreased. Cho elevated with high Cho/Cr ratios. Elevated lactate. Lipid peaks at 1.33 ppm suggests decreased survival. Marked elevation of myoinositol is characteristic. Radiologic-Pathologic Correlation Page 6 of 29

7 The pathologic diagnosis of gliomatosis cerebri requires radiologic-pathologic correlation; otherwise, the entity may be underreported, because the widely infiltrative nature of the lesion will not be evident. Groos Pathologic Findings (Figure 17) Diffuse neoplastic overgrowth. Blurring of gray-white junction borders. Underlying brain architecture preserved. Teaching Point " In GC the extensive tumor infiltration is disproportionate to histologic features " Microscopic Features (Figure 18, 19) Elongated glial cells with hyperchromatic nuclei and variable mitoses. Neoplastic cells often arranged in parallel rows. Diffuse infiltration along and between myelinated nerve fibers. Necrosis and neovascularity typically absent. Diffuse invasion of parenchyma with tumor cells. Differential Diagnosis (Figure 20) Arteriolosclerosis Aging brain with microvascular disease. No mass effect. Often associated volume loss. Some cases may be indistinguishable without biopsy. Page 7 of 29

8 Vasculitis Often multifocal areas of ischemia. DWI positive acutely. Patchy or multifocal enhancement may be seen. May be indistinguishable without biopsy. Anaplastic astrocytoma May appear discrete or infiltrating, often less diffuse. Variable enhancement. Viral encephalitis Meningeal involvement. Demyelination. Usually multiple lesions in typical locations. Typically lack significant mass effect. Often enhances, incomplete ring, open at cortex. Progressive multifocal leukoencephalopathy White matter t2 hyperintensity. Nonenhancing. Involves subcortical V-fibers and corpus callosum. Lymphoma Periventricular deep gm enhancing mass in primary cns lymphoma. Corpus callosum involvement. Inherited/acquired metabolic disorders Metachromatic leukodystrophy. Alexander disease: frontal lobe WM hyperintensity and enhancement. Page 8 of 29

9 Images for this section: Fig. 2: GC is usually a bilateral tumor. 77 year old woman with gait disturbances and episodic disorientation of one month evolution. Page 9 of 29

10 Fig. 3: Gliomatosis Cerebri clinical issues. Page 10 of 29

11 Fig. 4: In GC have been described two cell types with defined genetic alterations. Page 11 of 29

12 Fig. 5: Two gross pathologic gliomatosis cerebri types are recognized and can be applied to image. Page 12 of 29

13 Fig. 6: Secondary Gliomatosis in 73 year old man with progressive dementia of 6 months evolution. Page 13 of 29

14 Fig. 7: GC imaging general features. Page 14 of 29

15 Fig. 8: GC imaging general features. Page 15 of 29

16 Fig. 9: GC CT findings. 49 years old man with disorientation and decreased level of consciousness. Page 16 of 29

17 Fig. 10: GC CT findings. 49 years old man with disorientation and decreased level of consciousness. Page 17 of 29

18 Fig. 11: GC CT findings. 55 year old man withmental status alterationand episodic agitation. Page 18 of 29

19 Fig. 12: GC MR findings at usual sequences. Page 19 of 29

20 Fig. 13: GC MR findings at usual sequences. Page 20 of 29

21 Fig. 14: GC imaging features in advanced MR techniques. Page 21 of 29

22 Fig. 15: GC imaging features in advanced MR techniques. Page 22 of 29

23 Fig. 16: GC imaging features in advanced MR techniques. Page 23 of 29

24 Fig. 17: Radiologic-Pathologic correlation. Groos pathologic findings. Page 24 of 29

25 Fig. 18: Radiologic-Pathologic correlation. Microscopic features. Page 25 of 29

26 Fig. 19: Radiologic-Pathologic correlation. Microscopic features. Page 26 of 29

27 Fig. 20: GC differential diagnosis. Page 27 of 29

28 Conclusion Gliomatosis cerebri have characteristic features on conventional imaging which are well described, however, distinguish a lesion as GC on this imaging basis alone is often difficult. The increasing role of supportive imaging is important and may eventually preclude the need for histological diagnosis. Therefore it is important the knowledge of the imaging modalities and findings that are key to making the diagnosis. Personal information Lina Marcela Cruz Hernandez Radiology Resident Department of Medical Imaging Virgen de la Salud Hospital Toledo - Spain lina_cruzhernandez@hotmail.com Isabel Herrera Herrera Consultant Radiologist Department of Medical Imaging Virgen de la Salud Hospital Toledo - Spain isabel.herherrera@gmail.com References Chun-Pu Z, Hua-Qing L, Wei-Tao Z, et al. Clinical Manifestations and Imaging Characteristics of Gliomatosis Cerebri with Pathological Confirmation. Asian Pac J Cancer Prev 2014; 15 (11): Osborne A, et al. Diagnostic Imaging. Brain. 2nd ed Herrlinger U, Felsberg J, Küker W, et al. Gliomatosis cerebri: molecular pathology and clinical course. Ann Neurol 2002; 52: Page 28 of 29

29 Felsberg G, Silver S, Brown M, et al. Radiologic-Pathologic Correlation Gliomatosis Cerebri. AJNR 1994; 15: Bruna J and Velasco R. Gliomatosis cerebri. Neurología 2010; 25(3): Bendszus M, Warmuth-Metz M, Klein R, et al. MR Spectroscopy in GliomatosisCerebri. AJNR 2002; 21: Yang S, Wetzel S, Cha S. Dynamic Contrast-Enhanced T2*-Weighted MR Imaging of Gliomatosis Cerebri. AJNR 2002; 23: Park S, Suh Y, Nam D, et al. Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. Clinical Neuropathology 2009;28: Page 29 of 29