Multifocal spinal glioblastoma and leptomeningeal carcinomatosis in an elderly male with hydrocephalus and myelopathy
- Department of Neurosurgery, Upstate University Hospital, Syracuse, New York, United States .
- Department of Internal Medicine, New York Institute of Technology College of Osteopathic Medicine, Glen Head, New York, United States .
- Department of Pathology, Upstate University Hospital, Syracuse, New York, United States .
George W. Koutsouras, Department of Neurosurgery, Upstate University Hospital, Syracuse, New York, United States.
DOI:10.25259/SNI_985_2021Copyright: © 2021 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: George W. Koutsouras1, Annelle Amsellem2, Timothy Richardson3, Harish Babu1. Multifocal spinal glioblastoma and leptomeningeal carcinomatosis in an elderly male with hydrocephalus and myelopathy. 08-Dec-2021;12:595
How to cite this URL: George W. Koutsouras1, Annelle Amsellem2, Timothy Richardson3, Harish Babu1. Multifocal spinal glioblastoma and leptomeningeal carcinomatosis in an elderly male with hydrocephalus and myelopathy. 08-Dec-2021;12:595. Available from: https://surgicalneurologyint.com/surgicalint-articles/11278/
Background: Primary spinal glioblastoma multiforme with multifocal leptomeningeal enhancement is rarely diagnosed or documented. We describe a rare case of multifocal spinal isocitrate dehydrogenase (IDH) wild type glioblastoma with leptomeningeal carcinomatosis in an elderly male presenting with a chronic subdural hematoma, progressive myelopathy, and communicating hydrocephalus.
Case Description: A 77-year-old male with a medical history of an acoustic schwannoma, anterior cranial fossa meningioma, and immune thrombocytopenic purpura presented with right-sided weakness after repeated falls. Magnetic resonance imaging of the brain and spine demonstrated a left-sided subdural hematoma, leptomeningeal enhancement of the brain and skull base, ventricles, and the cranial nerves, and along with florid enhancement of the leptomeninges from the cervicomedullary junction to the cauda equina. Most pertinent was focal thickening of the leptomeninges at T1 and T6 with mass effect on the spinal cord. A T6 laminectomy with excisional biopsy of the lesion was planned and completed. Findings were significant for glioblastoma the World Health Organization Grade IV IDH 1 wild type of the thoracic spinal cord. Subsequently, his mental status declined, and he developed progressive hydrocephalus which required cerebrospinal fluid diversion. Unfortunately, the patient had minimal improvement in his neurological exam and unfortunately died 2 months later.
Conclusion: In a review of the limited literature describing similar cases of primary spinal glioblastoma, the prognosis of this aggressive tumor remains unfavorable, despite aggressive treatment options. The purpose of this report is to increase awareness of this rare condition as a potential differential diagnosis in patients presenting with multifocal invasive spinal lesions.
Keywords: Glioblastoma, Leptomeningeal carcinomatosis, Thoracic myelopathy
Glioblastoma multiforme (GBM) is a World Health Organization (WHO) Grade IV histological malignancy in the central nervous system.[
We describe a rare case of multifocal spinal isocitrate dehydrogenase (IDH) wild type glioblastoma with leptomeningeal carcinomatosis in an elderly male presenting with a chronic subdural hematoma, progressive myelopathy, and communicating hydrocephalus.
A 77-year-old male with a medical history significant for acoustic schwannoma, anterior cranial fossa meningioma, and immune thrombocytopenic purpura presented with the right-sided weakness after repeated falls. Non-contrast head computed tomography (CT) scan demonstrated a left-sided subdural hematoma. On examination, he was found awake, but disoriented. He had weakness of the left arm and leg, along with long tract signs that included sustained clonus and hyperreflexia of the bilateral lower extremities. Magnetic resonance imaging (MRI) of the brain demonstrated leptomeningeal enhancement of the brain and skull base, ventricles, and the cranial nerves [
Magnetic resonance imaging T1 sequences with gadolinium contrast- parasagittal views. (a) Brain - Suprasellar enhancement, leptomeningeal enhancement seen along the ventral brainstem and pineal region. (b) Cervical spine- Diffuse leptomeningeal enhancement seen in the ventral/dorsal spinal cord. (c) Thoracic spine - Dorsally compressive thickened lesions at T1 (single asterick) and T6 (double asterick). (d) Lumbar spine - Diffuse enhancement of the conus medullaris and the cauda equina.
For once having platelets within normal range a month prior, he developed thrombocytopenia with platelets of 40,000 uL. He was initially treated for this thrombocytopenia with intravenous Ig and platelet transfusion with minimal improvement. Once his thrombocytopenia was improved a biopsy was offered to the patient’s family and they wished to proceed.
Subsequently, a T6 laminectomy was planned and completed. Intraoperatively, we noted a firm infiltrative lesion of the spinal cord. There was no clear plane separating the tumor and the spinal cord. A subtotal resection was achieved secondary to the infiltrative nature of the lesion. No intraoperative neuromonitoring changes were observed. The pathology report of the biopsied specimens demonstrated hypercellularity with atypical mitotic nuclei and astrocytic features. In addition, there were foci of microvascular proliferation [
Histopathological stains of T6 lesion. (a) Tumor cells with hypercellularity, atypical mitotic nuclei and astrocytic features. There are foci of microvascular proliferation. Hematoxylin and eosin ×200 (b) Positive stain for Glial Fibrillary acidic protein ×50. (c) Positive for ATRX gene ×100. (d) Tumor cell negative for mutant IDH1 ×100.
Postoperatively, he was fully oriented and following commands in all extremities with more diminished strength and sensation on the right lower extremity compared to the left lower extremity. Over the course of a few days, he developed worsening encephalopathy. A lumbar puncture was completed as part of encephalopathy workup. Elevated opening pressures with an elevated protein level were observed. On placement of a lumbar drain, we identified a meaningful clinical improvement. A ventriculoperitoneal shunt was placed for cerebrospinal fluid diversion. Given the diagnosis with extensive leptomeningeal spread and overall poor neurological function, chemotherapy and radiotherapy were deferred by the oncologists and the family. The patient continued to decline and subsequently passed away 2 months after his surgical diagnosis.
Primary spinal glioblastoma with leptomeningeal disease is a rare diagnosis.[
Spinal GBM most commonly presents in younger patients, as demonstrated by a systematic review where the median age of presentation was 35.5–41 years.[
Our patient demonstrated diffuse cranial and spinal leptomeningeal enhancement with at least one infiltrative tumor at T6. Features commonly associated with spinal GBM include hydrocephalus, increased intracranial pressure, early leptomeningeal infiltration, and malformation of the spinal cord.[
Historically, spinal GBM has been treated with a combination of radiation therapy and temozolomide.[
While glioblastomas of the brain are the most common aggressive primary tumor in adults, primary GBM of the spine are exceptionally rare. Spinal glioblastomas are notorious for their nonspecific presentation, aggressive nature, and grim prognosis. Despite their rarity, primary spinal GBM should be considered in the differential diagnosis of scattered spinal lesions.
Patient’s consent not required as patients identity is not disclosed or compromised.
There are no conflicts of interest.
Thank you to Annette Absellem and Harish Babu, MD for aiding in the completion of this manuscript and Timothy Richardson, MD for his pathology data.
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