Multifocal spinal glioblastoma and leptomeningeal carcinomatosis in an elderly male with hydrocephalus and myelopathy

Abstract

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.

Figure 1:

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.

Figure 2:

Magnetic resonance imaging thoracic spine T1 sequence with gadolinium contrast, axial (left) and sagittal (right) images at the T6 level. There is an enhancing lesion that appears to be intradural and intramedullary.

Figure 3:

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.