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Desmoplastic cerebral astrocytoma of infancy: a case report
1Department of Tumor Pathology, Chair of Oncology, School of Medicine, Lódź.
Insights
This case study details a rare desmoplastic cerebral astrocytoma of infancy (DCAI) in a 7-month-old. The findings confirm DCAI
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Developmental Neurobiology
Background:
- Desmoplastic cerebral astrocytoma of infancy (DCAI) is a rare central nervous system (CNS) tumor.
- DCAI occurs in infants and is characterized by dense fibrous desmoplasia.
- It belongs to a group of CNS tumors with a generally favorable prognosis.
Observation:
- A massive, partially cystic tumor was observed in the left cerebral hemisphere of a 7-month-old boy.
- CT scans revealed ventricular system enlargement.
- Histological examination showed a tumor with cells arranged in fascicles and whorls, forming a storiform pattern.
Findings:
- Immunohistochemical staining confirmed glial histogenesis.
- Negative staining for neuronal markers excluded desmoplastic infantile ganglioglioma (DIG).
- The case presented aligns with previously reported DCAI characteristics.
Implications:
- This report is the first documented case of DCAI in Poland.
- It contributes to the understanding of rare pediatric CNS tumors.
- Accurate diagnosis through comprehensive data is crucial for patient management.
Abstract:
We report a case of desmoplastic cerebral astrocytoma of infancy (DCAI), in a 7-month-old boy. DCAI belongs to a group of recently described central nervous system (CNS) tumors, which also includes desmoplastic infantile ganglioglioma (DIG), pleomorphic xanthoastrocytoma (PXA) and dysembryoplastic neuroepithelial tumor (DNT), all characterized by relatively favorable prognosis and occurring mostly in children and young adults. DCAI is a rare neoplasm arising in the cerebral hemispheres within the first two years of life, and histologically is characterized by dense fibrous desmoplasia. In our case, CT scan presents a massive partially cystic tumor of the left cerebral hemisphere with an enlargement of the ventricular system. Histologically, the tumor was composed of cells arranged in fascicles and whorls forming storiform pattern. Immunohistochemical stainings for glial fibrillary acidic protein proved glial histogenesis of this tumor, while no cells were unequivocally immunopositive for neuron specific enolase, neurofilament proteins and synaptophysin what excludes a diagnosis of DIG--a similar entity but containing also a neuronal elements. Our studies, comprising a complete clinical, radiological, histopathological and immunohistochemical data, correspond to a cases of DCAI published before and it is the first one described in Poland.