Primary XXXX : an autopsy case and review of the literature.

@entity129 @entity32 @entity1 @entity2003 @entity1313 @entity5 @entity79 @entity288 @entity1174 @entity911 @entity7993 @entity158 @entity690 @entity892 @entity10 @entity3554 @entity4653
We report a case of primary @entity3554 (PDLG) in a 76-year-old male presenting with @entity911 , @entity4653 , @entity892 , @entity158 and @entity10 of recent onset. Neurological examination revealed @entity2003 and bilateral sixth @entity129 . The cerebrospinal fluid showed a marked hyperproteinorachia (4711 mg/L) and mild cytorachia (5-10 leucocytes/mm3) with a few atypical lymphoid cells. On admission, brain CT scan and MRI demonstrated diffuse and nodular leptomeningeal contrast enhancement predominant at the skull base and several @entity690 in the right parietal bone. Extensive serological studies for infectious, autoimmune or @entity5 were negative. The work-up diagnosis was @entity7993 or multiple meningeal and osseous metastases of an unknown primary @entity5 . Surgical biopsy of the right parietal @entity79 showed only fibrous tissue with no evidence of @entity5 or @entity32 . The @entity1 was treated with high dose @entity288 but its neurological status progressively worsened and he died of @entity1174 35 days after admission. Post-mortem examination revealed a PDLG, a rare @entity5 with about 60 cases reported. PDGL is characterized by the diffusion of neoplastic glial cells throughout the leptomeninges without evidence of a primary intra-parenchymal lesion. Recognition of this rare @entity1313 is important as recent reports suggest that radiotherapy and chemotherapy can improve @entity1 survival.
