Presentation
Headache and staring spells. Mass noted on CT. Status post resection of a calcified central neurocytoma as child. MRI for preoperative planning for attempted total resection.
Patient Data
There is a left frontal tumor with expansion and high T2/FLAIR signal in the left frontal lobe. There is a cystic portion of the lesion posteriorly in the left frontal lobe. There is also extension of T2 signal hyperintensity adjacent to the left frontal horn more superiorly. There are enhancing components along the gyrus rectus and orbital frontal gyri, with extension superiorly into the paramedian left frontal white matter.
There is an incidentally noted epidermoid cyst within the right cerebellopontine angle cistern.
Case Discussion
This is a case of a recurrent central neurocytoma located in the left frontal lobe, which was resected at age 6 (>25 years prior to the above study).
The patient underwent debulking via reopening of left craniotomy. Gross pathology of the specimens retrieved showed aggregates of tan-pink soft tissue of variable size. Histopathology showed round/ovoid cells with predominantly nesting to clustering arrangement with interspersed fibrillary neuropil islands along with foci of microcalcifications, hyalinized vessels, hemosiderin deposition, and rare eosinophilic granular bodies and Rosenthal fibers. Shattered tumor cells show mild nuclear enlargement, conspicuous nucleoli, and a subset displaying prominent gangloid appearance and frequent binucleation. The non-gangloid portion of the sample stained positive for synaptophysin and focally for GFAP and negative for EMA and neurofilament. The gangloid portion stained positive for chromogranin and focally weakly positive for NeuN. Tumor cells multifocally stained positive for Olig2. The findings along with previous history of central neurocystoma are compatible with the recurrent/progressive extraventricular/central neurocytoma, WHO Grade II. Additional molecular testing showcased positive chromosome 7 gain, FGFR1::TACC1 RNA fusion, and negative chromosome 10 loss, consistent with a central neurocytoma.
The patient’s post-operative course was complicated by hypoxic respiratory failure with MSSA and Citrobacter pneumonia status post tracheostomy and collar placement, seizures, and encephalopathy. The patient was discharged to a rehabilitation facility with some functional deficits following the resection.
Co-author:
Joshua Goldwag
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