Primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death

Abstract

Primary diffuse leptomeningeal oligodendrogliomatosis is a rare malignancy of central nervous system without evidence of a primary intraparenchymal focus. We present a 25-year-old woman with the postmortem diagnosis of primary diffuse leptomeningeal oligodendrogliomatosis. She was paraplegic and had sensory loss at the level of thoracal 8-9 for nearly 15 months. There was no symptom due to increased intracranial pressure. Unexpectedly she died just before biopsy. Autopsy revealed a nodular lesion at the level of thoracal 10 vertebra and diffuse oligodendroglioma throughout the leptomeninges of the brain and spine without intraaxial focus. To our knowledge, this is the first case of primary diffuse leptomeningeal oligodendrogliomatosis causing sudden death in the literature.

Diffuse anaplastic leptomeningeal oligodendrogliomatosis mimicking neurosarcoidosis

“…5 Although calcification is characteristic of intracerebral oligodendrogliomas, it is infrequently reported in diffuse leptomeningeal oligodendrogliomatosis. 2,6 Elevated CSF protein is common among reported cases, but CSF cytology is almost uniformly negative, as it was in this case. 1,2 This suggests that tumor cells spread centrifugally along the meninges rather than via dissemination in the CSF.…”

“…2,6 Elevated CSF protein is common among reported cases, but CSF cytology is almost uniformly negative, as it was in this case. 1,2 This suggests that tumor cells spread centrifugally along the meninges rather than via dissemination in the CSF. 7 Patients with leptomeningeal oligodendrogliomatosis may experience periods of stability or slow progression.…”

“…On MRI, leptomeningeal malignancies cause nodular leptomeningeal enhancement with or without hydrocephalus-nonspecific findings that can be seen in a variety of infectious, inflammatory, and other neoplastic conditions. [2][3][4] In this case, when extensive laboratory investigations and 2 separate biopsies of the lumbar leptomeninges and cauda equina failed to yield a specific diagnosis, the patient was initially treated with high-dose steroids for presumed neurosarcoidosis. The yield of biopsy in diffuse leptomeningeal oligodendrogliomatosis is low due to regional variations in the density of neoplastic cells and prominent leptomeningeal fibrosis, as was noted in this case despite the presence of extensive imaging abnormalities.…”

Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report

“…Low-grade neoplasms diffusely and primarily involving the leptomeninges have been reported sporadically throughout the literature [3][4][5][6][7][8][9][10], with recent case series examining genetic, histopathologic, radiographic, and clinical characteristics [1,2]. Although they occur predominately in pediatric populations, similar neoplasms have been reported in adults [11,12].…”

Primary leptomeningeal anaplastic oligodendroglioma with a 1p36–19q13 deletion: Report of a unique case successfully treated with Temozolomide

“…Less than 50 cases have been reported [2], mostly astrocytomas and glioblastomas [3]. A literature review revealed only 9 cases of a primary leptomeningeal oligodendroglioma [3][4][5][6][7][8][9][10][11] ( Table 1). Diagnosis was based on the biopsy of contrast-enhancing areas, but sometimes definite diagnosis was established only at autopsy [9].…”