Dysplastic ganglioneurocytoma with increased glucose metabolism: a heterotopia with unique histopathology
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Published on: January 14, 2014
1Department of Neurosurgery, Gunma University Graduate School of Medicine, Gunma, Japan.
Insights
A rare intracranial mass in a young boy was identified as a dysplastic ganglioneurocytoma. This unusual brain lesion lacked typical neoplastic features, suggesting a developmental origin.
Area of Science:
- Neuropathology
- Neuroradiology
- Pediatric Neurosurgery
Background:
- Intracranial mass lesions in pediatric patients require accurate diagnosis.
- Differentiating neoplastic from non-neoplastic lesions is critical for appropriate management.
Observation:
- A 1.7-year-old boy presented with an incidentally discovered frontal base intracranial mass (45x54x47 mm).
- Imaging revealed calcification, small cysts, extension to the sphenoid sinus and pharynx, and specific MRI signal characteristics without gadolinium enhancement.
- Positron-emission tomography showed increased 18F-fluorodeoxyglucose uptake, prompting surgical resection due to suspected neoplasm.
Findings:
- Histological examination showed anomalous neurons and glial cells with high cellularity (MIB-1 index 2%) but no cellular atypia.
- The lesion lacked normal cortical architecture and did not fit established diagnostic criteria for known entities.
- The findings supported a diagnosis of dysplasia rather than a true neoplasm.
Implications:
- This case introduces 'dysplastic ganglioneurocytoma' as a potential descriptor for this unique brain lesion.
- Recognizing this entity is important for accurate diagnosis and avoiding misclassification as a malignant tumor.
- Further research may clarify the pathogenesis and long-term behavior of dysplastic ganglioneurocytomas.
Abstract:
A 1 year and 7 month old boy was incidentally found to have an intracranial mass lesion at the frontal base. The mass was 45 x 54 x 47 mm in size, contained a calcification, a few small cysts, and extended downward to the sphenoid sinus and upper pharynx. The signal intensity of the lesion on magnetic resonance imaging was iso-high on T1-weighted images, and slightly high on T2-weighted images, and it did not enhance with gadolinium injection. Although there was no obvious mass effect, 18F-fluorode-oxyglucose positron-emission tomography demonstrated increased uptake, and a surgical resection was performed suspecting a neoplastic lesion. Histologically, the lesion consisted of small to large anomalous neurons and glial cells but lacked normal cortical architecture. Cellularity was high in some portion with MIB-1 labeling index of 2%, but there was no cellular atypia suggestive of neoplasm. Therefore, this lesion was considered to be a dysplasia that does not fit into the previously described entity. We suggest this lesion would be better described as dysplastic ganglioneurocytoma.
