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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.
Indian Journal of Pathology & Microbiology
|June 21, 2017
Summary
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.
