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Infantile gliosarcoma: a case and a review of the literature
1Department of Neurosurgery, Ushiku Aiwa General Hospital, Ibaraki, Japan.
Insights
Pediatric gliosarcoma, a rare brain tumor, presents aggressive growth and poor prognosis. This case highlights the challenges in diagnosis and treatment of this malignant biphasic neoplasm in a young child.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
Background:
- Gliosarcoma is an exceptionally rare brain tumor, particularly in infants.
- This study focuses on a rare case of pediatric gliosarcoma.
Observation:
- A 23-month-old boy presented with severe headache and vomiting.
- MRI revealed a large, enhancing frontal lobe lesion.
- Despite complete surgical resection, rapid tumor recurrence led to the patient's death within two months.
Findings:
- Histopathological analysis confirmed a biphasic tumor composed of malignant glial (glioblastoma) and mesenchymal (fibrosarcoma) elements.
- Both components exhibited high-grade malignancy, characterized by increased cellularity, mitotic activity, and atypical cellular features.
- The mesenchymal component showed fibrosarcomatous differentiation with reticulin-staining spindle cells.
Implications:
- This case underscores the aggressive nature and poor prognosis associated with pediatric gliosarcoma.
- It highlights the importance of accurate histopathological diagnosis for understanding the behavior of these rare tumors.
- Further research into the pathogenesis and targeted therapies for gliosarcoma is warranted.
Introduction:
Gliosarcoma in infant is a very rare entity.
Case Report:
The authors present a case of gliosarcoma in a 23-month-old boy. The patient was admitted to our hospital with persistent headache and frequent vomiting. MR imaging demonstrated a large frontal well-circumscribed lesion with a heterogeneous gadolinium enhancement. Although macroscopically the tumor was totally extirpated, the boy died of rapid tumor regrowth 2 months after surgery. The surgical specimens obtained from the tumor showed an admixture of two distinctive neoplastic tissues. One was a malignant mesenchymal feature. Its fibrosarcomatous nature was characterized by spindle-shaped cells with fine fibers that were deeply stained in silver preparations for reticulin. The other was gliomatous tissue forming islands surrounded by the sarcomatous tissues. Its glioblastomatous nature was obvious, as it was characterized by endothelial proliferation and perinecrotic pseudopalisading. Both tissues were histologically malignant, as evidenced by mitotic figure, high cellularity, atypical features, and variability.
Discussion:
The literature concerning gliosarcomatous tumors is reviewed, and pathological and clinical features of the tumor are briefly discussed.