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Primary diffuse leptomeningeal gliosarcomatosis.

Ju Hyung Moon1, Se Hoon Kim2, Eui Hyun Kim3

  • 1Department of Neurosurgery, Yonsei University Health System, Seoul, Korea. ; Brain Tumor Center, Yonsei University Health System, Seoul, Korea.

Brain Tumor Research and Treatment
|May 16, 2015
PubMed
Summary

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare, fatal condition. This case highlights gliosarcoma as an extremely rare PDLG subtype, emphasizing its inclusion in differential diagnoses for leptomeningeal enhancement.

Keywords:
Glioma, gliosarcomaLeptomeningeal carcinomatosisMeningoencephalitis

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Area of Science:

  • Neurology
  • Oncology
  • Pathology

Background:

  • Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare, fatal neoplastic condition.
  • PDLG involves diffuse leptomeningeal infiltration by glial cells without a primary intraparenchymal tumor.
  • PDLG histologically diagnosed as gliosarcoma is exceptionally rare, with only two prior reports.

Purpose of the Study:

  • To report a rare case of primary diffuse leptomeningeal gliosarcomatosis.
  • To emphasize the diagnostic challenges and clinical presentation of this rare condition.
  • To suggest PDLG as a differential diagnosis for unexplained leptomeningeal enhancement.

Main Methods:

  • Case report of a 68-year-old male patient.
  • Clinical presentation including fever, headache, and mental deterioration.
  • Brain MRI demonstrating diffuse leptomeningeal enhancement.
  • Meningeal biopsy confirming gliosarcoma.

Main Results:

  • The patient presented with nonspecific neurological symptoms and leptomeningeal enhancement on MRI.
  • Initial antiviral treatment was ineffective, with rapid disease progression.
  • Meningeal biopsy confirmed gliosarcoma, establishing the diagnosis of primary diffuse leptomeningeal gliosarcomatosis.

Conclusions:

  • Primary diffuse leptomeningeal gliosarcomatosis is an extremely rare entity.
  • Leptomeningeal enhancement on MRI, even without a clear primary tumor, warrants consideration of PDLG.
  • Early consideration of PDLG in the differential diagnosis may aid in timely management.