Disseminated primary diffuse leptomeningeal gliomatosis: a case report with liquid based and conventional smear cytology
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Published on: November 11, 2021
Masha Bilic1, Cynthia T Welsh, Zoran Rumboldt
1Department of Pathology & Laboratory Medicine, Medical University of South Carolina, Charleston, SC, USA. huseinm@musc.edu
Background:
Primary diffuse leptomeningeal gliomatosis is a rare neoplasm confined to the meninges without evidence of primary tumor in the brain or spinal cord parenchyma. Cerebrospinal fluid diversion via ventriculoperitoneal shunt may be used as a therapeutic modality. Herein, we describe the first report of cytologic findings of a case of this neoplasm with shunt-related peritoneal metastasis.
Case Presentation:
A 19-year-old male presented with a 6-month history of severe headaches. He had bilateral papilledema on physical exam. Cerebrospinal fluid examination was negative. Four months later a ventriculoperitoneal shunt was placed. Shortly thereafter, he was diagnosed with primary diffuse leptomeningeal gliomatosis based on the biopsy of an intradural extramedullary lesion adjacent to the lumbar spinal cord at a referral cancer center. The histology featured an infiltrating growth pattern of pleomorphic astrocytes with diffuse positivity for glial fibrillary acidic protein. A couple of months later he presented at our institution with ascites and an anterior peritoneal mass. Repeat cerebrospinal fluid cytology and fine needle aspiration of the mass confirmed disseminated gliomatosis. Cytologic characteristics included clusters of anaplastic cells of variable size, high nuclear to cytoplasm ratio and scant to moderate cytoplasm. Occasional single bizarre multinucleated cells were seen with eccentric "partial wreath-like" nuclei, clumped chromatin and prominent nucleoli. Patient expired 13 months after initial presentation.
Conclusion:
Disseminated primary diffuse leptomeningeal gliomatosis should be considered in the differential diagnosis of chronic aseptic meningitis and in the presence of a peritoneal tumor in patients with ventriculoperitoneal shunts. Immunocytochemistry may be of diagnostic value.
Insights
Primary diffuse leptomeningeal gliomatosis, a rare meningeal neoplasm, can metastasize via cerebrospinal fluid shunts. This case highlights cytologic findings of peritoneal metastasis in a patient with a ventriculoperitoneal shunt.
Area of Science:
- Neuropathology
- Oncology
- Cytopathology
Background:
- Primary diffuse leptomeningeal gliomatosis is a rare meningeal neoplasm.
- Ventriculoperitoneal shunts are sometimes used for cerebrospinal fluid diversion in these cases.
- This report details the first cytologic findings of peritoneal metastasis from this condition.
Purpose of the Study:
- To describe the cytologic findings of primary diffuse leptomeningeal gliomatosis with shunt-related peritoneal metastasis.
- To raise awareness of this rare complication.
Main Methods:
- Case presentation of a 19-year-old male with headaches.
- Diagnosis of primary diffuse leptomeningeal gliomatosis via spinal cord biopsy.
- Confirmation of disseminated gliomatosis via cerebrospinal fluid cytology and peritoneal mass fine needle aspiration.
Main Results:
- Histology showed infiltrating pleomorphic astrocytes positive for glial fibrillary acidic protein.
- Cytology revealed anaplastic cells with high nuclear-to-cytoplasmic ratio and bizarre multinucleated cells.
- Peritoneal metastasis was confirmed in a patient with a ventriculoperitoneal shunt.
Conclusions:
- Disseminated primary diffuse leptomeningeal gliomatosis should be considered in patients with unexplained aseptic meningitis or peritoneal tumors and shunts.
- Immunocytochemistry is valuable for diagnosis.
