Brain Tumor Res Treat.  2015 Apr;3(1):34-38. 10.14791/btrt.2015.3.1.34.

Primary Diffuse Leptomeningeal Gliosarcomatosis

• Moon JH
• Kim SH
• Kim EH
• Kang SG
• Chang JH

• KMID: 1882008
• DOI: http://doi.org/10.14791/btrt.2015.3.1.34

Abstract

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare condition with a fatal outcome, characterized by diffuse infiltration of the leptomeninges by neoplastic glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. In particular, PDLG histologically diagnosed as gliosarcoma is extremely rare, with only 2 cases reported to date. We report a case of primary diffuse leptomeningeal gliosarcomatosis. A 68-year-old man presented with fever, chilling, headache, and a brief episode of mental deterioration. Initial T1-weighted post-contrast brain magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement without a definite intraparenchymal lesion. Based on clinical and imaging findings, antiviral treatment was initiated. Despite the treatment, the patient's neurologic symptoms and mental status progressively deteriorated and follow-up MRI showed rapid progression of the disease. A meningeal biopsy revealed gliosarcoma and was conclusive for the diagnosis of primary diffuse leptomeningeal gliosarcomatosis. We suggest the inclusion of PDLG in the potential differential diagnosis of patients who present with nonspecific neurologic symptoms in the presence of leptomeningeal involvement on MRI.

Keyword

Glioma, gliosarcoma; Leptomeningeal carcinomatosis; Meningoencephalitis

MeSH Terms

Aged
Biopsy
Brain
Diagnosis
Diagnosis, Differential
Fatal Outcome
Fever
Follow-Up Studies
Gliosarcoma
Headache
Humans
Magnetic Resonance Imaging
Meningeal Carcinomatosis
Meningoencephalitis
Neuroglia
Neurologic Manifestations
Spinal Cord

Figure

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  Fig. 1 Axial (A) and sagittal (B) T1-weighted post-contrast magnetic resonance imaging showed diffuse leptomeningeal enhancement prominently along the right frontal, right operculum, right temporal, left cerebellum, left ambient cistern, interpeducluar cistern, bilateral sylvian fissure, and cervicomedullary junction without any massive intraaxial involvement.

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  Fig. 2 Axial image (A) of the whole-body positron emission tomography-computed tomography (PET-CT) showed intense fluorodeoxyglucose (FDG) uptake in the leptomeningeal enhancing lesions on magnetic resonance imaging (arrows). Coronal image (B) of the PET-CT showed linear increased FDG uptake throughout the cervical and upper thoracic spinal cords (arrows). These findings were suggestive of leptomeningeal involvement of malignancy.

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  Fig. 3 Axial (A) and sagittal (B) T1-weighted post-contrast magnetic resonance imaging showed rapid disease progression with expansion of leptomeningeal enhancement throughout the brain, multiple cranial nerve infiltration of lesions, and exacerbation of parenchymal edema adjacent to the leptomeningeal enhancing lesions.

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  Fig. 4 Photomicrographs. A: Hematoxylin and eosin (H&E) stain of the astrocytic component of the tumor (×400) is shown. B: A portion of the tumor demonstrated sarcomatous, spindle morphology (H&E stain, ×400). C: Microvascular proliferation (arrows) was observed throughout the glial component (H&E stain, ×400). D: Presence of areas of pseudopalisading necrosis (arrows) in the glial component (H&E stain, ×100). E and F: Focal glial fibrillary acidic protein (GFAP) and oligodendrocyte lineage transcription factor 2 (OLIG2) staining (×100) is evident in the astrocytic portion of the gliosarcoma (arrows). By contrast, the sarcomatous portion is negative for GFAP and OLIG2. G and H: The sarcomatous component is rich in trichrome (G) and reticulin (H) (×200).

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