scholarly journals Surgical management of complex spinal cord lipomas: how, why, and when to operate. A review

2019 ◽  
Vol 23 (5) ◽  
pp. 537-556 ◽  
Author(s):  
Dachling Pang

This review summarizes the classification, anatomy, and embryogenesis of complex spinal cord lipomas, and it describes in some detail the new technique of total lipoma resection and radical reconstruction of the affected neural placode. Its specific mission is to tackle two main issues surrounding the management of complex dysraphic lipomas: whether total resection confers better long-term benefits than partial resection and whether total resection fares better than conservative treatment—i.e., no surgery—for asymptomatic lipomas. Accordingly, the 24-year progression-free survival data of the author and colleagues’ series of over 300 cases of total resection are compared with historical data from multiple series (including the author and colleagues’ own) of partial resection, and total resection data specifically for asymptomatic lesions are compared with the two known series of nonsurgical treatment of equivalent numbers of patients. These comparisons amply support the author’s recommendation of total resection for most complex lipomas, with or without symptoms. The notable exception is the asymptomatic chaotic lipoma, whose peculiar anatomical relationship with the neural tissue defies even this aggressive surgical approach and consequently projects worse results (admittedly of a small number of cases) than for the other two lipoma subtypes of dorsal and transitional lesions. Prophylactic resection of asymptomatic chaotic lipomas is therefore not currently endorsed.

2009 ◽  
Vol 11 (5) ◽  
pp. 591-599 ◽  
Author(s):  
Giannina L. Garcés-Ambrossi ◽  
Matthew J. McGirt ◽  
Vivek A. Mehta ◽  
Daniel M. Sciubba ◽  
Timothy F. Witham ◽  
...  

Object With the introduction of electrophysiological spinal cord monitoring, surgeons have been able to perform radical resection of intramedullary spinal cord tumors (IMSCTs). However, factors associated with tumor resectability, tumor recurrence, and long-term neurological outcome are poorly understood. Methods The authors retrospectively reviewed 101 consecutive cases of IMSCT resection in adults and children at a single institution. Neurological function and MR images were evaluated preoperatively, at discharge, 1 month after surgery, and every 6 months thereafter. Factors associated with gross-total resection (GTR), progression-free survival (PFS), and long-term neurological improvement were assessed using multivariate regression analysis. Results The mean age of the patients was 41 ± 18 years and 17 (17%) of the patients were pediatric. Pathological type included ependymoma in 51 cases, hemangioblastoma in 15, pilocytic astrocytoma in 16, WHO Grade II astrocytoma in 10, and malignant astrocytoma in 9. A GTR was achieved in 60 cases (59%). Independent of histological tumor type, an intraoperatively identifiable tumor plane (OR 25.3, p < 0.0001) and decreasing tumor size (OR 1.2, p = 0.05) were associated with GTR. Thirty-four patients (34%) experienced acute neurological decline after surgery (associated with increasing age [OR 1.04, p = 0.02] and with intraoperative change in motor evoked potentials [OR 7.4, p = 0.003]); in 14 (41%) of these patients the change returned to preoperative baseline within 1 month. In 31 patients (31%) tumor progression developed by last follow-up (mean 19 months). Tumor histology (p < 0.0001) and the presence of an intraoperatively identified tumor plane (hazard ratio [HR] 0.44, p = 0.027) correlated with improved PFS. A GTR resulted in improved PFS for hemangioblastoma (HR 0.004, p = 0.04) and ependymoma (HR 0.2, p = 0.02), but not astrocytoma. Fifty-five patients (55%) maintained overall neurological improvement by last follow-up. The presence of an identifiable tumor plane (HR 3.1, p = 0.0004) and improvement in neurological symptoms before discharge (HR 2.3, p = 0.004) were associated with overall neurological improvement by last follow-up (mean 19 months). Conclusions Gross-total resection can be safely achieved in the vast majority of IMSCTs when an intraoperative plane is identified, independent of pathological type. The incidence of acute perioperative neurological decline increases with patient age but will improve to baseline in nearly half of patients within 1 month. Long-term improvement in motor, sensory, and bladder dysfunction may be achieved in a slight majority of patients and occurs more frequently in patients in whom a surgical plane can be identified. A GTR should be attempted for ependymoma and hemangioblastoma, but it may not affect PFS for astrocytoma. For all tumors, the intraoperative finding of a clear tumor plane of resection carries positive prognostic significance across all pathological types.


Neurosurgery ◽  
2009 ◽  
Vol 65 (3) ◽  
pp. 511-529 ◽  
Author(s):  
Dachling Pang ◽  
John Zovickian ◽  
Angelica Oviedo

Abstract OBJECTIVE Partial resection of complex spinal cord lipomas is associated with a high rate of symptomatic recurrence caused by retethering, presumably promoted by a tight content-container relationship between the spinal cord and the dural sac, and incomplete detachment of the terminal neural placode from residual lipoma. Since 1991, we have performed more than 250 total/near-total resections of complex lipomas with radical reconstruction of the neural placodes. Sixteen years of follow-up have proven the long-term benefits of this technique. Part I of this series introduces our technique of total resection and reports the immediate surgical results. Part II will analyze the long-term outcomes of both total and partial resection and identify the factors affecting outcome. METHODS From 1991 to 2006, 238 patients (age range, 2 months–72 years) with dorsal, transitional, and chaotic lipomas underwent total or near-total lipoma resection and radical placode reconstruction. Eighty-four percent of the patients were children younger than 18 years and 16% were adults. The technique consisted of wide bony exposure, complete unhinging of the lateral adhesions of the lipoma-placode assembly from the inner dura, untethering of the terminal conus, radical resection of the fat off the neural plate along a white fibrous plane at the cord-lipoma interface, meticulous pia-to-pia neurulation of the supple neural placode with microsutures, and expansile duraplasty with a bovine pericardial graft. Elaborate electrophysiological monitoring was used. RESULTS Three postoperative observations concern us. The first is that of the 238 patients, 138 (58%) had no residual fat on postoperative magnetic resonance imaging; 81 patients (36%) had less than 20 mm3 of residual fat, the majority of which were small bits enclosed by neurulation; and 19 patients (8%), mainly of the chaotic lipoma group, had more than 20 mm3 of fat. There are no significant differences in the amount of residual fat among lipoma types, but redo lipomas are more likely than virgin (previously unoperated on) lipomas to have residual fat by a factor of 2 (P = 0.0214). The second concern is that the state of the reconstructed placode is objectively measured by the cord-sac ratio, obtained by dividing the sagittal diameter of the reconstructed neural tube by the sagittal diameter of the thecal sac. A total of 162 patients (68%) had cord-sac ratios less than 30% (low), 61 (25.6%) had ratios between 30% and 50% (medium), and only 15 (6.3%) had high ratios of more than 50%. Seventy-four percent of patients with virgin lipomas had low cord-sac ratios compared with 56.3% in the redo lipoma patients. The overall distribution of cord-sac ratio is significantly different between redo and virgin lipomas (P = 0.00376) but not among lipoma types. Finally, the incidence of combined neurological and urological complications was 4.2%. The combined cerebrospinal fluid leak and wound infection/dehiscence incidence was 2.5%. Both sets of surgical morbidity compared favorably with the published rates reported for partial resection. CONCLUSION Total/near-total resection of spinal cord lipomas and complete reconstruction of the neural placode can be achieved with low surgical morbidity and a high yield of agreeable postoperative cord-sac relationship. Some large rambling transitional lipomas and most chaotic lipomas are the most difficult lesions to resect and tend to have less favorable results on postresection magnetic resonance imaging.


Neurosurgery ◽  
2010 ◽  
Vol 66 (2) ◽  
pp. 253-273 ◽  
Author(s):  
Dachling Pang ◽  
John Zovickian ◽  
Angelica Oviedo

Abstract OBJECTIVE To show the long-term benefits of total and near-total resection of complex spinal cord lipomas and reconstruction of the neural placode. METHODS We analyzed 238 patients with dorsal, transitional, and chaotic lipomas who had total resection as described in part I for overall progression-free survival probability (PFS, Kaplan-Meier analysis) over 16 years. We also analyzed subgroup proportional recurrence hazard (Cox analysis) of 6 outcome predictors of sex, lipoma type, age, preoperative symptoms, previous surgery, and postoperative cord-sac ratio. These results were compared with an age-matched, lesion-matched series of 116 patients followed for 11 years after partial lipoma resection and with the Parisian series of nonsurgical treatment. RESULTS The immediate effects of surgery were similar between total and partial resection: both achieved greater than 95% symptom stabilization or improvement rate. The neuro-urologic complication rates for the groups were also similar, 4.2% and 5.2% for total and partial resection, respectively. The combined cerebrospinal fluid leakage and wound complication rate of total resection was much lower at 2.5% than the 6.9% for partial resection, but both were better than published rates. The overall PFS for total resection was 82.8% at 16 years, comparing much more favorably with 34.6% for partial resection at 10.5 years (P &lt; .0001). Culling only the asymptomatic patients with virgin (previously unoperated) lipomas to match the patient profile of the Parisian series, the PFS for prophylactic total resection for this subgroup increased to 98.4% at 16 years, versus 67% at 9 years for no surgery and 43.3% at 10.5 years for our own partial resection series, with a remarkable statistical difference between total and partial resection (P = .00001). Subgroup analyses showed that sex and lipoma type did not affect outcome. For the other predictor variables, while univariate analyses showed that young age, absence of symptom, and virgin lipomas correlated with better statistical PFS than older age, symptoms, and redo lipomas, these effects vanished with multivariate analyses. Cord-sac ratio stood alone as the only influential outcome predictor in multivariate analysis, with a 96.6% PFS for a low ratio of &lt;30% and an 80.6% progression-free probability for a high ratio of &gt;50%, and a 3-fold increase in recurrence hazard for high ratios (P = .0009). This suggested that all the individual effects of the other predictor variables could be reduced to whether a low cord-sac ratio could be achieved with total lipoma resection and placode reconstruction. Cord-sac ratio was the obvious factor that differentiated the outcomes between total and partial resection, the latter associated with a &gt;90% chance of having a high cord-sac ratio. CONCLUSION Total and near-total resection of lipomas and complete reconstruction of the neural placode produced a much better long-term progression-free probability than partial resection and nonsurgical treatment. The perioperative complications for total resection were low and compared favorably with published results. A low postoperative cord-sac ratio and well-executed placode neurulation were strongly correlated with good outcome. The ideal preoperative patient profile with early disease stabilization and the best recurrence-free probability is an asymptomatic child less than 2 years without previous lipoma surgery. There are strong indications that partial resection in many cases produces worse scarring on the neural placode and worse prognosis than no surgery.


Neurosurgery ◽  
2015 ◽  
Vol 77 (4) ◽  
pp. 585-593 ◽  
Author(s):  
Charlotte Marie Halvorsen ◽  
Pål Rønning ◽  
John Hald ◽  
Tom Børge Johannesen ◽  
Frode Kolstad ◽  
...  

Abstract BACKGROUND: The existing literature on recurrence rates and long-term clinical outcome after resection of intraspinal nerve sheath tumors is limited. OBJECTIVE: To evaluate progression-free survival, overall survival, and long-term clinical outcome in a consecutive series of 131 patients with symptomatic intraspinal nerve sheath tumors. 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 100% complete; median follow-up time was 6.1 years. All patients (100%) had surgery as the first line of treatment; gross total resection was performed in 112 patients (85.5%) and subtotal resection in 19 patients (14.5%). Five-year progression-free survival was 89%. The following risk factors for recurrence were identified: neurofibroma, malignant peripheral nerve sheath tumor, subtotal resection, neurofibromatoses/schwannomatosis, and advancing age at diagnosis. More than 95% of patients had neurological function compatible with an independent life at follow-up. The rate of tumor recurrence in nonneurofibromatosis patients undergoing total resection of a single schwannoma was 3% (3/93), in comparison with a recurrence rate of 32% (12/38) in the remaining patients. CONCLUSION: Gross total resection is the gold standard treatment for patients with intraspinal nerve sheath tumors. In a time of limited health care resources, we recommend that follow-up be focused on the subgroup of patients with a high risk of recurrence. The benefit of long-term, yearly magnetic resonance imaging follow-up with respect to recurrence in nonneurofibromatosis patients undergoing gross total resection of a single schwannoma is, in our opinion, questionable.


2014 ◽  
Vol 13 (6) ◽  
pp. 600-612 ◽  
Author(s):  
Raheel Ahmed ◽  
Arnold H. Menezes ◽  
Olatilewa O. Awe ◽  
James C. Torner

Object Radical resection is recommended as the first-line treatment for pediatric intramedullary spinal cord tumors (IMSCTs), but it is associated with morbidity, including risk of neurological decline and development of postoperative spinal deformity. The authors report long-term data on clinical and treatment determinants affecting disease survival and neurological outcomes. Methods Case records for pediatric patients (< 21 years of age at presentation) who underwent surgery for IMSCTs at the authors' institution between January 1975 and January 2010 were analyzed. The patients' demographic and clinical characteristics (including baseline neurological condition), the treatment they received, and their disease course were reviewed. Long-term disease survival and functional outcome measures were analyzed. Results A total of 55 patients (30 male and 25 female) were identified. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2–37.2 years). Astrocytomas were the most common tumor subtype (29 tumors [53%]). Gross-total resection (GTR) was achieved in 21 (38%) of the 55 patients. At the most recent follow-up, 30 patients (55%) showed neurological improvement, 17 (31%) showed neurological decline, and 8 (15%) remained neurologically stable. Patients presenting with McCormick Grade I were more likely to show functional improvement by final follow-up (p = 0.01) than patients who presented with Grades II–V. Kaplan-Meier actuarial tumor progression-free survival rates at 5, 10, and 20 years were 61%, 54%, and 44%, respectively; the overall survival rates were 85% at 5 years, 74% at 10 years, and 64% at 20 years. On multivariate analysis, GTR (p = 0.04) and tumor histological grade (p = 0.02) were predictive of long-term survival; GTR was also associated with improved 5-year progression-free survival (p = 0.01). Conclusions The prognosis for pediatric IMSCTs is favorable with sustained functional improvement expected in a significant proportion of patients on long-term follow-up. Long-term survival at 10 years (75%) and 20 years (64%) is associated with aggressive resection. Gross-total resection was also associated with improved 5-year progression-free survival (86%). Hence, the treatment benefits of GTR are sustained on extended follow-up.


2010 ◽  
Vol 6 (2) ◽  
pp. 137-144 ◽  
Author(s):  
Martin Benesch ◽  
Daniela Weber-Mzell ◽  
Nicolas U. Gerber ◽  
Katja von Hoff ◽  
Frank Deinlein ◽  
...  

Object Reports on spinal cord ependymoma in children are rare. The aim of this study was to evaluate the clinical spectrum, treatment, and outcome of children with primary ependymoma of the spinal cord who were registered in the database of the pediatric German brain tumor studies Hirntumor (HIT) '91 and HIT 2000. Methods Between 1991 and 2007, 29 patients (12 male and 17 female, median age at diagnosis 13.6 years) with primary spinal cord ependymoma (myxopapillary ependymoma WHO Grade I, II, and III tumors in 6, 17, and 6 patients, respectively) were identified. Four patients had neurofibromatosis Type 2. Results With a median follow-up of 4.2 years (range 0.48–15 years), 28 patients (96.6%) were alive. Seven patients (24.1%) developed progressive disease or relapse, 2 after gross-total resection (GTR) and 5 after incomplete resection or biopsy. One patient with anaplastic ependymoma (WHO Grade III) died 65 months after diagnosis of disease progression. Primary adjuvant treatment (radiotherapy, chemotherapy, or both) was used in 8 (50%) of 16 patients following GTR and in 9 (82%) of 11 patients who underwent less than a GTR. Three additional patients were treated adjuvantly following progression. Estimated progression-free survival and overall survival rates at 5 years were 72.3% (95% CI 50%–86%) and 100%, respectively. Progression-free survival at 5 years is 84.4% (95% CI 50%–96%) for patients following GTR compared with 57.1% (95% CI 25%–69%) for patients who achieved a less than GTR (p = 0.088, log-rank test). A high relapse incidence (4 of 6) was observed among patients with myxopapillary ependymoma. Conclusions Gross-total resection is the mainstay of treatment for patients with primary spinal cord ependymoma and may be achieved in about 50% of the patients using modern surgical techniques. Primary adjuvant treatment was commonly used in children with spinal cord ependymoma irrespective of the extent of resection or tumor grade. The impact of adjuvant treatment on progression-free and overall survival has to be investigated in a prospective trial.


Neurosurgery ◽  
2010 ◽  
Vol 67 (6) ◽  
pp. 1622-1631 ◽  
Author(s):  
Charlotte Marie Halvorsen ◽  
Frode Kolstad ◽  
John Hald ◽  
Tom Børge Johannesen ◽  
Bård Kronen Krossnes ◽  
...  

Abstract BACKGROUND: 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.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii325-iii326
Author(s):  
Björn-Ole Juhnke ◽  
Marco Gessi ◽  
Nicolas Ulrich Gerber ◽  
Carsten Friedrich ◽  
Christine Haberler ◽  
...  

Abstract BACKGROUND Embryonal tumours with multilayered rosettes (ETMR) are highly aggressive tumors, mostly occurring in infants. Published clinical data refer to retrospective cohorts of inhomogeneously treated patients. Here, we describe the outcome of patients, who were prospectively treated within the P-HIT2000-trial, the subsequent HIT2000-interim-registry and earlier HIT-trials. PATIENTS AND METHODS Nineteen patients from the P-HIT2000-trial (2001–2011), 12 patients from the subsequent HIT2000-interim-registry (2012–2014) and 4 patients from earlier HIT-trials with centrally reviewed neuropathological and molecularly-confirmed diagnosis of ETMR were included. Outcome of 18 patients treated with carboplatin-etoposide-induction followed by tandem-high-dose chemotherapy (“CARBO-ETO+HDCT”) with stage-stratified radiotherapy administered in case of persistant disease, relapse or progression were compared to patients treated with HIT-SKK chemotherapy ± radiotherapy (n=9) or other regimens (n=8). RESULTS Median age at diagnosis was 2.9(1.0–5.3) years. Metastases at diagnosis were detected in 9 patients (26%). For the entire cohort of n=35, 5-year overall survival (OS) was 26.7%, and progression-free survival (PFS) was 18.5%. Five-year OS for patients with CARBO-ETO+HDCT, SKK chemotherapy or other regimens was 44.4%, 13.0% and 0%, respectively (p=0.006). Five-year PFS was 33.3%, 0% and 0%, respectively (p=0.119). Of 10 survivors, n=8 were treated with CARBO-ETO+HDCT; n=4 had craniospinal, n=2 local and n=4 no radiotherapy. Impact of initial gross-total-resection (p=0.231) and non-metastatic disease (p=0.097) was limited. CONCLUSIONS We show improved survival with carboplatin-etoposide-induction followed by tandem-high-dose chemotherapy, indicating that a cure is possible for some patients. However, despite intensive treatment, outcome is unsatisfactory and innovative therapies urgently need to be included in an upfront setting.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Lingcheng Zeng ◽  
Qi Mei ◽  
Hua Li ◽  
Changshu Ke ◽  
Jiasheng Yu ◽  
...  

AbstractTo evaluate the surgical effect on survival in patients with incidental low-grade glioma (LGG) through comparison between asymptomatic and symptomatic patients. The medical records of surgically treated adult cerebral incidental LGG (iLGG) patients in our department between January 2008 and December 2015 were retrospectively reviewed. The survival of patients was calculated starting from the initial imaging diagnosis. Factors related to progression-free survival (PFS), overall survival (OS) and malignant progression-free survival (MPFS) were statistically analyzed. Seventy-five iLGG patients underwent surgery: 49 in the asymptomatic group, who underwent surgery in the asymptomatic period, and 26 in the symptomatic group, who underwent surgery after the tumor had grown and the patients had developed tumor-related symptoms. Significantly more tumors were initially located adjacent to the functional area in the symptomatic group than in the asymptomatic group (P < 0.05), but there was no significant difference in the total resection rate between the two groups. The incidence of postoperative complications (15.4%) and postoperative epilepsy (23.1%) was higher in the symptomatic group than in the asymptomatic group (4.1% and 10.2%, respectively). Multivariate analysis showed that surgical timing, namely, surgery performed before or after symptom occurrence, had no significant effect on PFS, OS or MPFS, while total resection significantly prolonged PFS, OS and MPFS, and the pathology of oligodendroglioma was positively correlated with PFS and OS (P < 0.05). Surgical timing for iLGGs should facilitate total resection. If total resection can be achieved, even after symptom occurrence, patients can achieve comparable survival benefits to those treated with surgery in the asymptomatic phase.


Sign in / Sign up

Export Citation Format

Share Document