Ganglioglioma of the spinal cord

Abstract

A case of ganglioglioma or neuroastrocytoma of the spinal cord in a 78-year-old man is reported. Diagnosis was based on the histological identification of the neoplastic cells and on the study of the architecture of the tumour. The presence of cellular anaplasia, sometimes of marked degree, and of small nests of infiltration suggested an initial malignant behaviour regarding both cellular types. A survey of the five reported cases of spinal ganglioglioma is presented.

Primary Spinal Anaplastic Ganglioglioma

“…Malignant transformation of intracranial gangliogliomas has been documented in about 10% of reported cases, and has been attributed to the glial component of these neoplasms [8, 9]. The potential for malignant transformation of spinal gangliogliomas is unknown [10] because the literature describes only 4 spinal neoplasms identified as malignant gangliogliomas [11,12,13,14]. As noted, ours is only the fifth documented case of primary spinal anaplastic ganglioglioma, and the presenting signs in this young child were vague.…”

Anaplastic Ganglioglioma: A Very Rare Intramedullary Spinal Cord Tumor

“…In adult patients, 4 descriptions of primary intramedullary aGG were found [5,6,10,12]. Spinal dissemination of a primary cerebral aGG or malignant transformation of a low-grade GG to aGG was described in 7 patients, including adults and children (table 1) [1,3,7,8].…”

“…Usually, complete resection of low-grade tumors in children results in a favorable long-term oncologic outcome [14] and a favorable neurologic outcome when motor pathways remain intact [15]. However, the few anecdotal precedents of this rare tumor variant provided some evidence for an unfavorable outcome with surgical resection followed by either radio- or chemotherapy [1,4,5,6,7,8,9]. There is only one report of a long-term survivor in the pediatric population (table 1, gray shading), and this patient received combined adjuvant radiochemotherapy treatment [4].…”

“…In 3 of the 4 case descriptions of primary spinal aGG in adult patients, the outcome was fatal due to tumor-related complications [5,6,10]. The fourth adult patient suffered from fragile X syndrome and primary aGG.…”

“…Only 15 case reports exist of aGGs occurring in the spinal cord, 5 of them as a result of spinal dissemination of a cerebral aGG (metastatic spinal aGG) and 2 with histological and cytogenetic evidence of malignant transformation of a former low-grade GG (secondary spinal aGG). Of the remaining 8 primary spinal cord aGGs, only 4 were reported in pediatric patients [1,3,4,5,6,7,8,9,10,11,12]. …”