[34] Leptomeningeal Dissemination in Diffuse Intrinsic Pontine Gliomas, a Comparison between Magnetic Resonance Imaging and Autopsy Findings

K Newman, M Karajannis, A Narayana, J Allen, D Zagzag. New York University School of Medicine, New York, NY

Background: Diffuse intrinsic pontine gliomas (DIPG) constitute 60-75 % of tumors found within the pediatric brainstem. These malignant tumors are often rapidly progressive and the median survival is less then 12 months with leptomeningeal dissemination often being the harbinger of disease progression. We report a case series of DIPG and compare the clinical and post-mortem findings.
Design: We reviewed the ante-mortem clinical course and magnetic resonance imaging (MRI) and compared the autopsy results from 4 recent cases of DIPG. The autopsies were restricted to the central nervous system.
Results: Average age ranged from 5 to 11 years with a mean of 7.25 years. The genders were equally represented (2 males and 2 females). All 4 patients presented with cranial nerve and/or cerebellar dysfunction. 2 patients were biopsied and 2 were diagnosed via MRI. All 4 patients were treated with radiation and chemotherapy. Time to progression (clinical worsening with documented MRI progression) ranged from 22 days to 365 days with an average of 210 days. The average time from last imaging and death was 40 days and ranged from 4 days to 75days. The average survival, from initial diagnosis was 315 days, ranging from 120 to 455 days. The average time from progression to death was 90 days. At autopsy, all 4 patients had disease which had spread to the midbrain and medulla and 2 had disease in the cerebellum. The average size of the pontine tumor was 4.4 centimeters (cm) and ranged in size from 3 to 6.5 cm. 3 of 4 patients had hydrocephalus and 2 patients had ventricular shunts. 1 case had bilateral uncal herniation. These findings were consistent with the ante-mortem MRI findings. Microscopically, all cases were diagnosed as glioblastoma, WHO, grade IV. 3 of 4 cases had leptomeningeal dissemination at autopsy. 2 of these patients were not diagnosed prior to death. In both cases of autopsy diagnosed leptomeningeal dissemination MRI imaging was performed ante-mortem and in close proximity to the time of death; the time interval between imaging and post-mortem analysis were 4 and 60 days.
Conclusions: DIPG is a devastating malignant tumor with few post-mortem analyses. MRI is often utilized to monitor and predict disease progression, however, leptomeningeal dissemination is not always diagnosed on ante-mortem imaging studies, as seen in this review, and thus MRI is not always predictive of leptomeningeal dissemination or disease progression.
Category: Autopsy

Wednesday, March 24, 2010 9:30 AM

Poster Session V # 19, Wednesday Morning

 

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