Primary Spinal Anaplastic Ganglioglioma

Abstract

Gangliogliomas of the spinal cord are very unusual and the anaplastic variant of these tumors is extremely rare. In spinal anaplastic gangliogliomas, malignant changes (hypercellularity, vascular proliferation, necrosis, high numbers of mitotic figures) are typically evident in the glial element of the tumor. Gangliogliomas can arise in any part of the central nervous system and the features of adult and pediatric cases differ significantly. We describe the case of a pediatric patient who had a spinal anaplastic ganglioglioma extending from the T₉ to the L₃ level. The signs at presentation were bilateral knee flexion (quadriceps weakness) while walking and intermittent urinary incontinence. The tumor was partially resected with an ultrasonic aspirator, but the patient’s neurological status deteriorated as a result and adjuvant radiotherapy did not resolve the deficit.

Anaplastic Ganglioglioma: A Very Rare Intramedullary Spinal Cord Tumor

“…Four other case reports of pediatric primary spinal aGG were found in the literature, as follows: one comparable to the patient presented here with stable disease at 61 months after diagnosis but no information about her clinical status (table 1, gray shading) [4]; one patient with postoperative right leg palsy and a progression-free follow-up of 22 months [9]; one with progressive disease after 7 months [4], and one without information regarding outcome (high-grade tumor in a series of cerebral and spinal low-grade GGs that was not further discussed) [11]. In adult patients, 4 descriptions of primary intramedullary aGG were found [5,6,10,12].…”

“…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].…”

“…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]. …”

Anaplastic Ganglioglioma in the Spinal Cord: Case Report and Literature Review

“…Radiotherapy is generally given to patients with incomplete resection of the tumor or recurrent tumor, and to those with a tumor histology showing anaplastic features or oligodendroglial-like cells [6]. There are only a few reports of AGG of the spinal cord [1,2,3,4,5,6,7,8,9,10,11] (table 1). Blümcke and Wiestler [1] published a series of 326 gangliogliomas, only 17 of which were reported as AGG.…”

“…At 61 months, this patient had stable disease, whereas the boy had meningeal carcinomatosis presenting 1 month after surgery and died 7 months later. Karabekir et al [8] reported a 2-year-old female patient with a primary spinal AGG extending from T 9 to L 3 level. The patient presented with quadriceps weakness and was treated with partial resection followed by adjuvant radiotherapy (total dose of 40 Gy at 1.8 Gy per day).…”