Intra-Axial Tumors of the Cervicomedullary Junction: Surgical Results and Long-Term Outcome

Abstract

Until recently, intra-axial brainstem tumors were traditionally regarded as surgically inaccessible lesions with a uniformly poor prognosis. However, increasing data indicate that distinct subgroups of brainstem tumors exist that are amenable to surgical intervention. To address this question, we reviewed our experience in the operative management of 39 consecutive patients, in the magnetic resonance imaging (MRI) era, with intra-axial cervicomedullary tumors, in order to determine those factors associated with long-term outcome. Thirty-nine patients (26 male, 13 female) underwent surgery by a single surgeon (F.J.E.) between 1985 and 1994. Mean age of diagnosis was 14 years (range 3 months – 60 years); mean duration of preoperative symptoms was 24 weeks (range 1–168). Twenty patients presented with lower cranial nerve dysfunction and 19 presented with motor and/or sensory dysfunction. All patients were graded according to the McCormick Scale, pre- and postoperatively, and at the time of follow-up. All patients were evaluated with MRI scanning. Twenty-three patients had either previous biopsy or subtotal resection, 13 previous radiation therapy, and 6 previous chemotherapy. The mean time to follow-up was 48 months (range 7–138). Twelve patients underwent gross total resection, 7 near total resection (>90%), 15 subtotal resection (50–90%), and 5 partial resection (< 50%). Histologically, there were 15 low-grade fibrillary astrocytomas, 9 ependymomas, 7 gangliogliomas, 3 anaplastic astrocytomas, 3 juvenile pilocytic astrocytomas, and 2 mixed gliomas. Although the vast majority of tumors were low grade histologically, a higher proportion of the patients with high-grade lesions experienced tumor progression when compared to low-grade tumors (75 vs. 30%). Overall, the 5-year progression-free and total survivals were 60 and 89%, respectively. There was 1 death within the first postoperative month. Preoperative duration of symptoms greater than 15 weeks was associated with a longer progression-free survival. There was a trend for preoperative neurologic grade to predict functional neurologic outcome at follow-up. In summary, intra-axial tumors of the cervicomedullary junction are a distinct subset of brainstem tumors, predominantly of low-grade histology, with favorable long-term progression-free and total survivals following surgical resection. A long duration of preoperative symptoms may indicate an indolent clinical course and a more favorable prognosis. Our data also indicate that early surgical intervention is warranted prior to neurologic deterioration.

Favorable outcome with conservative treatment for children with low grade brainstem tumors

“…Furthermore, treatment delay to time of progression (median 1.21 years, range 0.05-13.95) conferred no survival disadvantage for these patients. In agreement with our results, Weiner et al [22] reported PFS and OS of 60% and 89% in 39 patients with partially resected cervicomedullary lesions with varied pathologies undergoing observation. Similar outcomes were reported by other authors [23,24].…”

Prognostic factors in intramedullary astrocytomas: a literature review

“…If a fundamentally important article was missed in the initial search, it would surely be encountered in the reference lists of the reviewed papers and as such would be cited extensively. In fact, articles not found on Medline were discovered by this strategy [19,48,72] and neither proved to substantially influence the resulting review. Any newly published article is today most likely to appear on Medline, we used the ''What's new'' function to ascertain that no such article [46] escaped our attention.…”

Cervicomedullary tumors in children

“…4,16,33,38,42 Cervicomedullary tumors are a rare group of diverse tumors. The majority of patients have low-grade tumors with long duration of preoperative symptoms.…”

“…20 Some believe that resection should be the treatment of choice for CMTs. 4,16,24,38,40 Patients with longer prodrome and less severe neurological deficits prior to resection typically have the lowest risk of sustaining a significant neurological deficit after surgery, and in general have a favorable overall survival prognosis. 1,14,33,38 However, depending on the tumor pathological and radiographic characteristics, radiotherapy (RT) and/or chemotherapy may supplement or be considered a better primary treatment than surgery.…”