Conus medullaris and cauda equina tumors

Conus and cauda equina tumors

J Neurosurg Spine 20:335–343, 2014

Intradural cauda equina and conus medullaris tumors (CECMTs) are rare. Only a few large clinical series exist to date. Therefore, clinical symptoms, surgical complications, and outcomes are poorly understood. The aim of the present study was to evaluate outcome after surgery of CECMTs and to identify the factors associated with a worse clinical prognosis based on the results of a series with sufficiently high number of cases.

Methods. All cases of intradural CECMTs treated surgically at the authors’ department between March 2006 and May 2012 were retrospectively evaluated. Arachnoid cysts and multifocal tumors were excluded. Sixty-eight adult patients met the inclusion criteria (35 female and 33 male patients; median age 56 years). Follow-up data were available for 72% (n = 49) in a median period of 9 months.

Results. Overall, 18 tumors were located intramedullary and 50 extramedullary. The majority were nerve sheath tumors (n = 27), ependymomas (n = 17), and meningiomas (n = 9). The most common preoperative symptom was pain. The rate of new transient postoperative impairment was 18% (n = 12), and new permanent deficits were observed in only 6% (n = 4). Overall neurological improvement was achieved in 62%. The reversibility of preoperative symptoms was related to the interval between the time of symptom onset and the time of surgery and to the presence of preoperative neurological deficits. Surgery of ependymoma and carcinoma metastases was associated with a higher rate of morbidity.

Conclusions. Intradural CECMTs present as a group of tumors with varying histological features and clinical symptoms. Symptomatic manifestation is usually unspecific, mimicking degenerative lumbar spine syndromes. Despite a significant risk of transient deterioration, early surgery is advisable because more than 94% of patients maintain at least their preoperative status and more than 60% improve during follow-up. The reversibility of preoperative symptoms is related to the duration between symptom onset and surgery and to the presence of preoperative neurological deficits. The prognosis for recovery from cauda equina or conus medullaris syndrome is less favorable than for other deficits. Surgery of ependymoma is associated with a higher morbidity rate than other benign entities.

Long-term Outcome After Resection of Intraspinal Ependymomas: Report of 86 Consecutive Cases

Neurosurgery 67:1622–1631, 2010 DOI: 10.1227/NEU.0b013e3181f96d41

Objective: To evaluate progression-free survival, overall survival (OS) and long-term clinical outcome in a consecutive series of 86 patients with intraspinal ependymomas.

METHODS: Medical charts were retrospectively reviewed. Surviving patients voluntarily participated in a clinical history and physical examination that focused on neurological function and current tumor status.

RESULTS: Follow-up data are nearly 100% complete; mean follow-up time was 82 months. Eighty-five patients (99%) had surgery as a first-line treatment; 14 (17%) of these patients received adjuvant radiotherapy. Of the 85 patients who underwent primary surgery, gross total resection was performed in 60 patients (71%) and subtotal resection in 25 patients (29%). Ten-year progression-free survival rate was 75%; 5-year OS, 97%; and 10-year OS, 91%. Reduced preoperative neurological function and older age at diagnosis were significantly associated with increased risk of death. At follow-up, spontaneous regression of residual tumor after primary surgery may have occurred in 7 of 19 patients (37%). More than 75% of patients had neurological function compatible with an independent life at follow-up. Good preoperative neurological function was significantly associated with favorable outcome. It was not possible to evaluate the effect of radiotherapy on progression-free survival and OS.

CONCLUSION: Gross total resection remains the optimal treatment for patients with spinal ependymoma. Patients should be monitored with a clinical examination and magnetic resonance imaging at regular intervals up to 10 years after surgery.

Primary Intramedullary Tumors of the Spinal Cord

SPINE Volume 34, Number 22S, pp S69 –S77, 2009

Study Design. Clinically based systematic review.

Objective. To define optimal clinical care for primary intramedullary spinal cord tumors using a systematic re- view with expert opinion.

Methods. Focused questions on the treatment of primary intramedullary spinal cord tumors were refined by a panel of spine oncology surgeons, medical and radiation oncologist. Keyword were searched through Medline database and pertinent abstracts and manuscripts obtained. The quality of literature was rated as high, moderate, low, or very low. Using the GRADE evidence based review system the proposed questions were answered using the literature review and expert opinion. These treatment recommendations were then rated as either strong or weak based on the quality of evidence and clinical expertise.

Results. The literature searches revealed low and very low quality evidence with no prospective or randomized studies. The MEDLINE search engine returned 9000 articles which was restricted to articles about human subjects and written in the English language. The subsequent search resulted in a return of: “spinal cord tumor” (5053), “ependymoma” (580), “astrocytoma” (420), and “glioma” (235) articles. Seventeen articles referenced timing of surgical intervention and symptomatology for intramedullary spinal cord tumors. One hundred fifty-eight chemotherapy and 183 radiation therapy articles for intramedullary spinal cord tumors were reviewed.

Conclusion. The most important factor in determining the IMSCT patient’s long-term neurologic and functional outcome after surgery is the patient’s preoperative neurologic status. However, this must be taken in the context of the underlying tumor histology. Therefore, resection is reserved for progressive neurologic decline and serial monitoring for asymptomatic individuals. Adjuvant therapy is an option for high grade astrocytomas (WHO grades 3–4).

Prognostic factors in intramedullary astrocytomas

Eur Spine J (2009) 18:1397–1422 DOI 10.1007/s00586-009-1076-8

Astrocytomas affect a significant portion of patients with intramedullary tumors. These infiltratively growing tumors are treated by a variety of methods—biopsy and decompressive surgery, maximal safe resection, adju- vant oncological therapy. Also, numerous prognostic factors are reported in the literature. Better understanding of factors that influence prognosis may help in treatment planning with the goal of prolonging survival. We have thus undertaken an extensive literature review in order to define factors affecting prognosis. A total of 38 articles were studied. Only tumor grade was consistently reported as the major factor affecting prognosis. The influence of other clinical factors (age, gender, history length, functional status, tumor location or extent, syrinx or cyst presence) can be speculated upon, but cannot be assessed adequately from the available literature. For both low- and high-grade (HG) astrocytomas, maximal safe tumor resection should be the primary treatment objective but is often not feasible in contrast to other intramedullary and spinal neoplasms. Since the biological nature of spinal cord HG glioma is identical to that of the brain, the same treatment algorithm of maximal safe resection followed by concomitant radio and chemotherapy would be sensible to implement.