2019 Research Forum

Department of Internal Medicine, Kern Medical , Bakersfield, CA David Aguirre MD, Nosheen Hasan MD R2, Sarah El-Halees MS IV A Case of Gliomatosis Cerebri

Imaging

Introduction

Continued Case Presentation

Post radiation treatment MRI brain was repeated but was notable for worsening bilateral frontotemporal hyperintensities, and an enlarging left temporal lesion with three new left frontal enhancing lesions. Oncology referred the patient to neurosurgery for consideration of resection of the astrocytoma, however were unfortunately notified that patient had already deceased. Gliomatosis cerebri is a rare infiltrating glioma involving multiple contiguous lobes of the brain that affects all age groups, more commonly in males with a median age of 46- 53. Common presenting symptoms may include focal weakness, sensory deficits, seizure, altered mental status, memory deficits with “dementia—like” features, and progressive headaches. Diagnosis is made primarily with MRI and histopathologic confirmation of an astrocytic process. Brain MRI shows a T1 weight intensity, and T2 weighted or FLAIR hyperintensity in the involved areas of the brain. Prognosis is poor despite aggressive treatment with 26-52% surviving less than one year. Surgical resection is generally not feasible due to multiple lobe involvement, radiation has an unclear benefit, and chemotherapy has no proven efficacy. Major obstacles toward establishing standardized treatments include rarity of the disease, lack of in depth understanding of the tumor biology, variation across histopathologic grading, variability in patient outcomes, and lack of response to therapies. References 1. Herrlinger U, Jones DT, Glas M, Hattingen E, Gramatzki D, Stuplich M, et al. Gliomatosis cerebri: no evidence for a separate brain tumor entity. Acta neuropathol (2015) 131(2):309-19.10.1007/s00401-015- 1495-Z 2. Ranjan S, Warren K. Gliomatosis cerebri: current understanding and controversies. Front oncol. 2017: 7:165. 3. Shin YM, Chang KH, han MH, Myung NH, Chi JG, Cha SH, et al. Gliomatosis cerebri comparison of MR and CT features. AHNR Am H Roentgenol (1993) 161(4):859-62.10.2214/ajr.161.4.8372774 4. Kandler RH, Smoth CM, Broome JC, Davies-Jones GA. Gliomatosis cerebri: a clinical, radiological and pathological report of four cases. Br J Neurosurg (1991) 5(2):187-93.10.3109/02688699108998466 5. Chen S, Tanaka S, Giannini C, Morris J, Yan ES, Buckner J, et al. Gliomatosis cerebri: clinical characteristics, management, and outcomes. J Neurooncol (2013):267-75.10.1007/s11060-013-1058-x Conclusion

The patient is a 49 year-old male with a history of IV crystal meth abuse, ETOH abuse and seizure disorder on Keppra 750mg BID presented to the emergency department for increasing frequency of seizures. Patient reported having at least 5 episodes daily of seizures that involved upper extremity clenching with subsequent loss of consciousness and urinary incontinence. Seizures were typically followed by 4-5 minute post ictal period. Per patient sister, he was also having daily visual hallucinations for the past 2-3 weeks in which she described as the patient talking, yelling, and fighting with nonexistent people in an empty room. Medical records were obtained from previous neurological evaluation 4 months prior which indicated an unremarkable CT and MRI brain, no epileptiform activity on EEG monitoring, and negative CSF fluid analysis for acute infections. All labs including urine toxicology, RPR, and HIV were unremarkable on presentation. Overnight EEG monitoring indicated no epileptic activity. MRI brain was performed and was notable for multiple areas of subtle white and gray matter T2 and Flair abnormalities in the bilateral frontotemporal lobes, edema appearing in the bilateral frontotemporal areas and basal ganglion, with one discrete small 7 mm enhancing ring lesions in the left temporal area, highly suspicious gliomatosis cerebri type 2. Neurosurgery was consulted for brain biopsy and pathology of the right temporal lobe was positive for diffuse astrocytoma (WHO grade II/IV). Patient was started on Depakote for seizures as well as chemotherapy and radiation for four months. Gliomatosis cerebri is an extremely rare and aggressive brain tumor with resistance to therapy. Its incidence is reported to be less than 100 cases each year in the United States. The purpose of this case is to discuss the diagnosis, evaluation, and management of gliomatosis cerebri in a patient presenting in the acute setting with increasing frequency of seizures and new onset hallucinations.

Case Presentation

Image 1.A: T2 Weighted frontal; B: T1 Ring enhancing lesion; C: T1 7mm Ring enhancing lesion of left temporal area; D: Flair abnormality in the bilateral frontotemporal lobes.

Above Image: Diffuse infiltrate of astrocytes at 200x magnification

The inferior images of Figure 2 and 3 were obtained at initial presentation. While, the images at the top were obtained 4 months s/p chemotherapy and radiation.

Above image: IDH1 immunohistological stain at 200x magnification

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