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[Intracranial primitive neuroectodermal tumor in an infant: a case report]
K Yoshizato1, S Yoshioka, T Tamai
1Department of Neurosurgery, Oita Prefectural Hospital, Japan.
Insights
A supratentorial primitive neuroectodermal tumor (PNET) in an infant was successfully removed and treated with chemotherapy. The child remains disease-free after 12 months, suggesting effective treatment for this rare brain tumor.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Molecular pathology
Background:
- Primitive neuroectodermal tumors (PNETs) are rare and aggressive central nervous system neoplasms.
- Distinguishing between central and peripheral PNETs is crucial for prognosis and treatment, but can be challenging based solely on location.
Observation:
- A 2-month-old infant presented with a supratentorial PNET extending into the skull.
- The tumor exhibited histological features of poorly differentiated neuroectodermal cells.
- Immunohistochemical analysis revealed reactivity for GFAP, synaptophysin, neuron-specific enolase, and vimentin.
Findings:
- A significant finding was moderate immunoreactivity for the HBA71 antigen (p30/32M1C2), a marker typically associated with peripheral PNETs.
- Despite its intracranial location, the tumor's immunophenotype suggests a classification as a peripheral PNET.
- The patient underwent complete tumor resection followed by chemotherapy, achieving a 12-month recurrence-free survival.
Implications:
- This case highlights the potential for intracranial tumors to exhibit characteristics of peripheral PNETs.
- The HBA71 antigen may serve as a valuable diagnostic marker in differentiating PNET subtypes, even in unusual locations.
- Successful management with surgery and chemotherapy offers hope for similar pediatric cases.
Abstract:
A 2-month-old girl with a supratentorial primitive neuroectodermal tumor (PNET), which extended into the skull, is herein presented. The patient underwent total removal of the tumor and also received a course of postoperative chemotherapy. After a follow-up period of 12 months, the infant is alive without recurrence. Histologically, the tumor was composed of poorly differentiated neuroectodermal cells, and these neoplastic cells showed a mild immunohistochemical reaction for GFAP and synaptophysin, and a moderate reactivity for neuron specific enolase and vimentin. In addition, a moderate level of immunoreactivity for HBA71 antigen (p30/32M1C2), which is the product of the M1C2 gene and is found in peripheral PNETs but not in central PNETs, was noted in many neoplastic cells. Although this tumor was located intracranially, it may be classified as a peripheral PNET.