scholarly journals A systematic review of overall survival in pediatric primary glioblastoma multiforme of the spinal cord

2017 ◽  
Vol 19 (2) ◽  
pp. 239-248 ◽  
Author(s):  
Subhas K. Konar ◽  
Shyamal C. Bir ◽  
Tanmoy K. Maiti ◽  
Anil Nanda

OBJECTIVE The incidence of primary spinal cord glioblastoma multiforme (GBM) in the pediatric age group is very rare. Only a few case series and case reports have been published in the literature; therefore, overall survival (OS) outcome and the as-yet poorly defined management options are not discussed in detail. The authors performed a cumulative survival analysis of all reported cases of pediatric spinal cord GBM to identify the predictive factors related to final survival outcome. METHODS A comprehensive search for relevant articles was performed on PubMed's electronic database MEDLINE for the period from 1950 to 2015 using the search words “malignant spinal cord tumor” and “spinal glioblastoma multiforme.” This study was limited to patients younger than 18 years of age. Survival rates for children with various tumor locations and treatments were collected from the published articles and analyzed. RESULTS After an extensive literature search, 29 articles met the study inclusion criteria. From the detailed information in these articles, the authors found 53 children eligible for the survival analysis. The majority (45%) of the children were more than 12 years old. Thirty-four percent of the cases were between 7 and 12 years of age, and 21% were younger than 7 years. In the Kaplan-Meier survival analysis, children younger than 7 years of age had better survival (13 months) than the children older than 7 years (7–12 years: 10 months, > 12 years: 9 months; p = 0.01, log-rank test). Fifty-five percent of the children were female and 45% were male. A cervical tumor location (32%) was the most common, followed by thoracic (28.3%). Cervicothoracic (18.9%) and conus (18.8%) tumor locations shared the same percentage of cases. Cervical tumors had a worse outcome than tumors in other locations (p = 0.003, log-rank test). The most common presenting symptom was limb weakness (53%), followed by sensory disturbances (25%). Median OS was 10 months. The addition of adjuvant therapy (radiotherapy [RT] and/or chemotherapy [CT]) after surgery significantly improved OS (p = 0.01, log-rank test). Children who underwent gross-total resection and RT had better outcomes than those who underwent subtotal resection and RT (p = 0.04, log-rank test). Cerebrospinal fluid spread, hydrocephalus, brain metastasis, and spinal metastasis were not correlated with OS in primary spinal GBM. CONCLUSIONS Adjuvant therapy after surgery had a beneficial effect on overall outcome of spinal GBM in the pediatric age group. Gross-total resection followed by RT produced a better outcome than subtotal resection with RT. Further large-scale prospective study is required to establish the genetic and molecular factors related to OS in primary GBM of the spinal cord in pediatric patients.

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Nil Comunoglu ◽  
Ozgur Kilickesmez ◽  
Buge Oz

Glioneuronal neoplasms are rare tumors. Recently, an unusual glioneuronal tumor histologically showing neuropil-like islands has been described. Here, we present such a tumor originating from spinal cord of a 14-year-old girl, who has scoliosis and urinary incontinence. Microscopically, the glial component was chiefly fibrillary astrocytic, punctuated by neuropil-like islands. Immunohistochemically, glial tissue was GFAP positive, and neuropil-like areas and big neurons were synaptophysin reactive. For astrocytic component Ki-67 proliferation index was 1% and p53 was immunonegative. This case is unique in that in the literature it is the second reported case in pediatric age group that is located at spinal cord.


2014 ◽  
Vol 13 (4) ◽  
pp. 400-407 ◽  
Author(s):  
Sunil Kukreja ◽  
Sudheer Ambekar ◽  
Anthony Hunkyun Sin ◽  
Anil Nanda

Object Reports of myxopapillary ependymomas (MPEs) of the spinal cord in pediatric patients are scarce. In the literature, various authors have shared their experiences with small groups of patients, which makes it difficult to create a consensus regarding the treatment approach for spinal MPEs in young patients. The aim of this study was to perform a survival analysis of patients in the first 2 decades of life whose cases were selected from the published studies, and to examine the influence of various factors on outcomes. Methods A comprehensive search of studies published in English was performed on PubMed. Patients whose age was ≤ 20 years were included for integrative analysis. Information about age, treatment characteristics, critical events (progression, recurrence, and death), time to critical events, and follow-up duration was recorded. The degree of association of the various factors with the survival outcome was calculated by using Kaplan-Meier estimator and Cox proportional hazard model techniques. Results A total of 95 patients were included in the analysis. The overall rate of recurrence (RR) was 34.7% (n = 33), with a median time to recurrence of 36 months (range 2–100 months). Progression-free survival (PFS) and overall survival rates at 5 years were 73.7% and 98.9%, respectively. Addition of radiotherapy (RT) following resection significantly improved PFS (log-rank test, p = 0.008). In patients who underwent subtotal resection (STR), administering RT (STR + RT) improved outcome with the lowest failure rates (10.3%), superior to patients who underwent gross-total resection (GTR) alone (RR 43.1%; log-rank test, p < 0.001). Addition of RT to patients who underwent GTR was not beneficial (log-rank test, p = 0.628). In patients who had disseminated tumor at presentation, adjuvant RT controlled the disease effectively. High-dose RT (≥ 50 Gy) did not change PFS (log-rank test, p = 0.710). Conclusions Routine inclusion of RT in the treatment protocol for spinal MPEs in young patients should be considered. Complete resection is always the goal of tumor resection. However, when complete resection does not seem to be possible in complex lesions, RT should be used as an adjunct to avoid aggressive resection and to minimize inadvertent injury to the surrounding neural tissues. High-dose RT (≥ 50 Gy) did not provide additional survival benefits, although this association needs to be evaluated by prospective studies.


2010 ◽  
Vol 113 (2) ◽  
pp. 333-339 ◽  
Author(s):  
Martin J. Rutkowski ◽  
Michael E. Sughrue ◽  
Ari J. Kane ◽  
Derick Aranda ◽  
Steven A. Mills ◽  
...  

Object Intracranial hemangiopericytoma (HPC) is a rare and malignant extraaxial tumor with a high proclivity toward recurrence and metastasis. Given this lesion's rarity, little information exists on prognostic factors influencing mortality rates following treatment with surgery or radiation or both. A systematic review of the published literature was performed to ascertain predictors of death following treatment for intracranial HPC. Methods The authors identified 563 patients with intracranial HPC in the published literature, 277 of whom had information on the duration of follow-up. Statistical analysis of survival was performed using Kaplan-Meier and Cox regression analysis. Results Hemangiopericytoma was diagnosed in 246 males and 204 females, ranging in age from 1 month to 80 years. Among patients treated for HPC, overall median survival was 13 years, with 1-, 5-, 10-, and 20-year survival rates of 95%, 82%, 60%, and 23%, respectively. Gross-total resection alone (105 patients) was associated with superior survival rates overall, with a median survival of 13 years, whereas subtotal resection alone (23 patients) resulted in a median survival of 9.75 years. Subtotal resection plus adjuvant radiotherapy led to a median survival of 6 years. Gross-total resection was associated with a superior survival benefit to patients regardless of the addition or absence of radiation, and patients receiving > 50 Gy of radiation had worse survival outcomes (median survival 4 vs 18.6 years, p < 0.01, log-rank test). Patients with tumors of the posterior fossa had a median survival of 10.75 versus 15.6 years for those with non–posterior fossa tumors (p < 0.05, log-rank test). Conclusions Treatment with gross-total resection provides the greatest survival advantage and should be pursued aggressively as an initial therapy. The addition of postoperative adjuvant radiation does not seem to confer a survival benefit.


Spine ◽  
1986 ◽  
Vol 11 (1) ◽  
pp. 23-25 ◽  
Author(s):  
ROBERTO PADOVANI ◽  
FRANCESCO TOGNETTI ◽  
SILVIA LAUDADIO ◽  
BRUNO BERNARDI

2011 ◽  
Vol 02 (02) ◽  
pp. 186-189 ◽  
Author(s):  
Mehmet Basmaci ◽  
Askin Esen Hasturk ◽  
Isin Pak

ABSTRACTCystic mature teratomas of the spinal cord are rare lesions. Teratomas account for up to 0.1% of all spinal cord tumors. Teratomas include tissues that originate from the three germ layers. Several congenital disorders may accompany the teratoma. Teratomas are classified as mature, immature or malignant type according to their histological characteristics. Thoracic spinal teratomas are uncommon in the pediatric age group. More than half of the patients are adults. We present herein a five-year-old male patient who was referred to our clinic with cystic mature teratoma at the T12 level.


2017 ◽  
pp. 28-31
Author(s):  
Shashi Sharma ◽  
Sakshi Dewan ◽  
Naveen Bhardwaj ◽  
Mir Aziz ◽  
Shilpa Singh ◽  
...  

2021 ◽  
Vol 15 (1) ◽  
pp. 431-435
Author(s):  
Mohamed Mansy ◽  
Mostafa Kotb ◽  
Mohamed Abouheba

Congenital lumbar hernias are uncommonly seen in the pediatric age group, with only about 60 cases reported in the literature. It is usually accompanied by a multitude of congenital anomalies involving different organ systems of the body. For instance, it may involve the ribs, spine, muscles, and the kidneys. Herein, we report a case of congenital lumbar hernia in an 8-month-old boy who underwent an operative repair using a mesh with an uneventful outcome.


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