Gangliogliomas (GGs) are rare, usually low-grade
tumors that account for 1-2% of all central nervous system (
CNS) neoplasms. Spinal GGs are exceedingly rare (1% of all spinal
tumors) and the presentation of anaplastic features in them is even rarer. According to the last World Health Organization (WHO) classification of
CNS neoplasms, anaplastic GG (AGG) is classified as a
malignant neoplasm (grade III). We performed a scoping review of the literature to elucidate the epidemiology, clinical features, histopathology, treatment, and outcome of primary spinal AGGs, which, to the best of our knowledge, is the first such review. Relevant studies were identified by a search of the MEDLINE and SCOPUS databases, using the following combination of search strings: (anaplastic
ganglioglioma or
malignant ganglioglioma or high grade
ganglioglioma) AND (spine or spinal or spinal cord). We included studies related to primary or recurrent AGGs and malignant transformation of low-grade GGs. The search produced 15 eligible studies, plus two studies from the references, all of which were case reports of patients with spinal AGGs (17 studies with 22 patients). The mean age of the patients was 21.4 years and the sex ratio was 1:1, with male predominance. Motor impairment was the most common presentation, followed by sensory impairment, gait problems, urinary disturbances, and
back pain. The thoracic spine was the most frequently involved area (14/22) followed by the cervical (6/22) and lumbar (5/22) spine. In terms of histology, the anaplastic features were usually predominant in the glial
element, resembling high-grade
astrocytomas, while the neuronal
element was composed of the so-called dysplastic
ganglion (neuronal)
synaptophysin-positive cells, without mitotic figures. Complete surgical resection of the
tumor without neurological compromise, plus
adjuvant chemotherapy and
radiotherapy, was the treatment protocol implemented in the two patients with the best outcome. Primary spinal AGG is an exceedingly rare entity, with only 22 cases being retrieved after an extensive literature search. They appear to affect children and young adults and tend to manifest aggressive behavior. Most studies report that only the glial component of AGGs presents high-grade malignant features, with low mitotic activity in the neuronal component. We therefore suggest that, pending novel targeted
therapy, AGGs should be treated as high-grade
gliomas, with an aggressive treatment protocol consisting of maximal safe resection and
adjuvant chemotherapy and
radiotherapy.