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Primary leptomeningeal primitive neuroectodermal tumor: A difficult entity to diagnose
Jyothy Sehgal1, Murthy Murali Krishna Jagarlapudi2, Murthy V R K Tenneti3
1Department of Neurology, Medanta - The Medicity Hospital, Gurgaon, Haryana, India.
Abstract:
A 25-year-old male presented with difficulty in walking, loss of vision, and seizures. A clinical possibility of craniospinal meningeal pathology was considered. On computed tomography (CT) scan and magnetic resonance imaging, there was an enhancement of leptomeninges with few ring-enhancing lesions in both frontal lobes and right frontoparietal region. He was evaluated for low backache and occipital headache 2½ years earlier and was found to have communicating hydrocephalus on CT scan. He underwent ventriculoperitoneal shunt and was followed up with CT scans. Meningeal biopsy was done in the present admission, and there was a diffusely infiltrating small round cell tumor. Immunohistochemistry was done, and the tumor cells were found to be negative for glial, mesenchymal, melanotic, and lymphoid markers. The cells were positive for neuron-specific enolase, chromogranin, and vimentin. A diagnosis of primitive neuroectodermal tumor involving the meninges was made. A possibility of primary leptomeningeal tumor extending to parenchyma was considered based on the clinical progression. Patient was treated with chemotherapy and radiotherapy. He improved partially and was stable at 3-year follow-up.
Insights
A rare primitive neuroectodermal tumor (PNET) of the meninges was diagnosed in a young male presenting with neurological deficits. Treatment with chemotherapy and radiotherapy led to partial improvement and stability.
Area of Science:
- Neuro-oncology
- Neuropathology
Background:
- A 25-year-old male presented with progressive neurological symptoms including difficulty walking, vision loss, and seizures.
- Initial evaluation revealed communicating hydrocephalus, treated with a ventriculoperitoneal shunt 2.5 years prior.
Observation:
- Imaging showed leptomeningeal enhancement and ring-enhancing lesions in the frontal and frontoparietal regions.
- Meningeal biopsy revealed a diffusely infiltrating small round cell tumor.
Findings:
- Immunohistochemistry was negative for glial, mesenchymal, melanotic, and lymphoid markers.
- Tumor cells were positive for neuron-specific enolase, chromogranin, and vimentin, leading to a diagnosis of primitive neuroectodermal tumor (PNET) involving the meninges.
- The findings suggested a primary leptomeningeal tumor with possible parenchymal extension.
Implications:
- This case highlights a rare instance of meningeal PNET with unusual presentation and progression.
- The patient showed partial improvement and clinical stability after chemotherapy and radiotherapy.
- Further research into primary leptomeningeal tumors and their management is warranted.
