Journal article
Malignant transformation and leptomeningeal spread of recurrent ganglioglioma: case report and review of literature
Clinical imaging, Vol.48, pp.7-11
03/2018
DOI: 10.1016/j.clinimag.2017.09.011
PMID: 28950177
Abstract
Gangliogliomas are rare, typically benign low grade tumors which usually occur in the supratentorial compartment, more commonly in the pediatric age group. Malignant transformation of a low grade ganglioglioma in the absence of prior radiation therapy is exceptionally rare with only 17 such cases previously reported. The author present an additional case of malignant transformation of recurrent ganglioglioma. Unlike the prior cases however, the recurrent tumor in our case showed prominent intratumoral hemorrhage and widespread leptomeningeal spread. The rapidly fatal course in our case was likely secondary to tumor dedifferentiation which is supported by serial histopathological specimens.
•Gangliogliomas are rare, typically benign, supratentorial brain neoplasms. The vast majority of gangliogliomas are benign WHO grade I tumors (90–94%). The remaining 6% either present as grade III anaplastic ganglioglioma or rarely as grade I lesions which undergo malignant conversion.•Including the present case, there have been 18 cases of malignant transformation of grade I ganglioglioma unassociated with radiation reported in the English literature. Exceptional features in our case included leptomeningeal involvement, intratumoral hemorrhage and rapid histological dedifferentiation.•Leptomeningeal spread in similar such cases has been reported only twice, including the present case. Leptomeningeal spread in our case could be secondary to direct tumor extension in to the subarachnoid space, disruption of the normal blood-brain barrier in the surgical bed or tumor dedifferentiation.•Intratumoral hemorrhage has never been previously described in patients who underwent malignant conversion. The intratumoral hemorrhage in our case may reflect prominent vascular recruitment and neoangiogenesis, again secondary to tumor dedifferentiation.•Although a BRAF V600E and a p53mutations were present in our case, a detailed mutational analysis was not carried out to assess for additional mutations which could fully explain the malignant behavior of the tumor.
Details
- Title: Subtitle
- Malignant transformation and leptomeningeal spread of recurrent ganglioglioma: case report and review of literature
- Creators
- Grant Riesberg - Carver College of Medicine, University of Iowa, IA, USAGirish Bathla - Department of Radiology, University of Iowa hospitals and clinics, IA, USASarika Gupta - Department of Pathology, University of Iowa hospitals and clinics, IA, USAPankaj Watal - Department of Radiology, University of Iowa hospitals and clinics, IA, USAToshio Moritani - Department of Radiology, University of Iowa hospitals and clinics, IA, USA
- Resource Type
- Journal article
- Publication Details
- Clinical imaging, Vol.48, pp.7-11
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.clinimag.2017.09.011
- PMID
- 28950177
- ISSN
- 0899-7071
- eISSN
- 1873-4499
- Language
- English
- Date published
- 03/2018
- Academic Unit
- Radiology; Pathology
- Record Identifier
- 9984051778202771
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