Primary leptomeningeal oligodendroglioma with documented progression to anaplasia and t(1;19)(q10;p10) in a child

Excerpt

Oligodendrogliomas are infiltrating gliomas typically arising in the cerebral cortex and white matter. Rare examples present with diffuse leptomeningeal involvement in the absence of a clinically/radiologically detectable intraparenchymal tumor [1‐5, 7, 8, 10, 14, 15]. Although oligodendrogliomas are rare in children, most primary leptomeningeal oligodendrogliomas have occurred in the first decade of life (Table 1). We report the case of a 3-year-old girl, who presented with progressive regression of her ability to walk and an increase of cranial circumference. MRI showed marked hydrocephalus and diffuse leptomeningeal enhancement without supratentorial intraparenchymal lesions (Fig. 1a–c). A C3–C4 mildly enhancing 5 mm nodule was present, which could not be defined with certainty as meningeal or intraparenchymal (Fig. 1c). Cerebrospinal fluid (CSF) was xanthochromic, with high protein (177 mg/dl), normal glucose (63 mg/dl) and increased white blood cells (47 cells/mm³, 50% mononuclear). Bacterial cultures were negative, but polymerase chain reaction showed CSF positivity for M. tuberculosis. A diagnosis of basal meningitis was made and the child was unsuccessfully treated with anti-tuberculosis therapy. When a meningeal biopsy demonstrated a low-grade neuroectodermal tumor consistent with low-grade oligodendroglioma, the patient was started on chemotherapy, according to the SIOP protocol for low-grade gliomas, based on vincristine and carboplatin, changed later to vincristine and cisplatin alternating with cyclophosphamide every 6 weeks due to an allergic reaction to carboplatin. Chemotherapy was suspended at 18 months, after remarkable improvement of her clinical condition. She also received radiotherapy at the level of C2–C4, because of the gradual increase of the cervical nodule (54 Gy in 30 fractions). The child remained stable for the following 3 years, when her tumor progressed to anaplastic oligodendroglioma, as confirmed by a new biopsy of a left frontal paraventricular nodule. At this time, MRIs demonstrated multiple enhancing nodules filling the ventricles, suggesting involvement of the sub-ependymal parenchyma (Fig. 1d–f). The patient received temozolomide, but her clinical condition continued to deteriorate and she expired, 7 years from the original diagnosis.

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