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Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma
J Sperner1, J Gottschalk, K Neumann
1Universitäts-Kinderklinik (KAVH), Universitätsklinikum Rudolf Virchow, Freie Universität Berlin, Germany.
Insights
Desmoplastic infantile ganglioglioma, a rare brain tumor, presents unique radiological and histological features. This case study shows a good prognosis despite some malignant characteristics.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
- Neuroradiology
Background:
- Desmoplastic infantile ganglioglioma (DIG) is a rare, congenital brain tumor typically presenting in infancy.
- Early diagnosis and understanding of its radiological and histological features are crucial for patient management.
- This report details a unique case of DIG in a 30-month-old boy.
Observation:
- The patient experienced complex partial seizures starting at 7 months of age.
- Cranial imaging revealed a cystic mass with ring-enhancement in the right temporal fossa.
- Histological examination showed abundant connective tissue, cystic areas, and glial/neuronal cells.
Findings:
- Immunocytochemical analysis confirmed both glial and neuronal cell origins.
- The tumor exhibited features that could be considered malignant.
- Radiological findings were correlated with histological and immunocytochemical results for the first time.
Implications:
- Despite potentially malignant features, DIG can have a favorable prognosis.
- This case highlights the importance of integrating imaging, histology, and immunocytochemistry for accurate diagnosis.
- Further research into DIG pathogenesis and prognostic factors is warranted.
Abstract:
The clinical course and radiological and histological findings in a 30-month-old boy suffering from desmoplastic infantile ganglioglioma are reported. The child's development was normal until a series of complex partial seizures occurred at the age of 7 months. Cranial computed tomography and magnetic resonance imaging revealed a cystic mass with intensive ring-shaped contrast enhancement in the right temporal fossa without shift of intracranial structures. Histologically, the firm, grayish tumor showed an enormous amount of connective tissue, cystic areas, and some mitoses. Glial and neuronal cell lines were identified by immunocytochemical methods. Eighteen months after surgery the boy had developed well without any neurological dysfunction; no radiation or chemotherapy was given. For the first time a synopsis of radiological findings in this rare brain tumor is correlated with the results of multiple histological and immunocytochemical studies. Despite some malignant characteristics, the prognosis of this dysontogenetic brain tumor is good.