Pineoblastoma—The Experience at St. Jude Children's Research Hospital

Neurosurgery ◽  
2017 ◽  
Vol 81 (1) ◽  
pp. 120-128 ◽  
Author(s):  
Kara A. Parikh ◽  
Garrett T. Venable ◽  
Brent A. Orr ◽  
Asim F. Choudhri ◽  
Frederick A. Boop ◽  
...  

Abstract BACKGROUND: Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors. OBJECTIVE: To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome. METHODS: A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed. RESULTS: Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection—compared with subtotal resection or biopsy—had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer. CONCLUSION: Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii205-ii206
Author(s):  
Alexander Yahanda ◽  
Bhuvic Patel ◽  
Amar Shah ◽  
Daniel Cahill ◽  
Garnette Sutherland ◽  
...  

Abstract BACKGROUND Few studies use large, multi-institutional patient cohorts to examine the role of intraoperative MRI (iMRI) in the resection of grade II gliomas. We assessed the impact of iMRI and other factors on overall survival (OS) and progression-free survival (PFS) for newly-diagnosed grade II astrocytomas and oligodendrogliomas. METHODS Retrospective analyses of a multicenter database assessed the impact of patient-, treatment-, and tumor-related factors on OS/PFS. RESULTS 232 resections (112 astrocytomas, 120 oligodendrogliomas; 135 males; mean age 36.2 ± 0.9 years) were analyzed. Oligodendrogliomas had longer OS (p< 0.001) and PFS (p=0.009) than astrocytomas. Multivariate regression showed that extent of resection (EOR), including gross-total (GTR) versus near-total (NTR) resection (p=0.02, HR: 0.64, 95% CI: 0.25-.79) and GTR versus subtotal resection (STR) (p=0.006, HR: 0.23, 95% CI: 0.08-0.66), was associated with longer OS. GTR versus NTR (p=0.04, HR: 0.49, 95% CI: 0.29-.85), GTR versus STR (p=0.02, HR: .54, 95% CI: .32-.91) and iMRI use (p=0.02, HR: 0.54, 95% CI: 0.32-0.92) were associated with longer PFS. Frontal (p=0.048, HR: 2.11, 95% CI: 1.01-4.43) and occipital/parietal (p=0.003, HR: 3.59, 95% CI: 1.52-8.49) locations were associated with shorter PFS (versus temporal). Kaplan-Meier analyses showed longer OS with increasing EOR (p=0.03) and 1p/19q gene deletions (p=0.02). PFS improved with increasing EOR (p=0.01), GTR versus NTR (p=0.02), and resections above STR (p=0.04). Factors influencing adjuvant treatment (35.3% of patients) included age (p=0.002, OR: 1.04) and EOR (p=0.037, OR: 0.41 for NTR versus STR; p=0.003, OR: 0.39 for GTR versus STR), but not glioma subtype or location, as determined by logistic regression. Additional tumor resection after iMRI was performed in 105/159 (66%) iMRI cases, yielding GTR in 54.5% of these cases. CONCLUSIONS EOR significantly improves OS and PFS for patients with grade II astrocytomas and oligodendrogliomas. Intraoperative MRI may improve EOR and was associated with increased PFS.


Neurosurgery ◽  
1987 ◽  
Vol 21 (2) ◽  
pp. 201-206 ◽  
Author(s):  
Mario Ammirati ◽  
Nicholas Vick ◽  
Liao Youlian ◽  
Ciric Ivan ◽  
Michael Mikhael

Abstract Thirty-one patients operated upon for supratentorial glioblastomas or anaplastic astrocytomas were studied to evaluate the effect of the extent of surgical resection on the length and quality of survival. The median age was 50 years and the median preoperative Karnofsky rate was 80. Twenty-one patients (68%) had glioblastoma multiforme, and 10 patients (32%) had anaplastic astrocytoma. Early postoperative enhanced computed tomography was used to determine the extent of tumor resection. Gross total tumor resection was accomplished in 19 patients (61%), and subtotal resection was performed in 12 patients (39%). The two groups were comparable regarding age, sex, pathological condition, preoperative Karnofsky rating, tumor location, postoperative radiation therapy, and postoperative chemotherapy (P > 0.05). The gross total resection group lived longer than the subtotal resection group by life table analysis (P < 0.001; median survival of 90 and 43 weeks, respectively). Postoperatively, the mean functional ability measured by the Karnofsky rating was significantly increased in the gross total resection group (P = 0.006), but not in the subtotal resection group (P > 0.05). The difference in degree of change between preoperative and postoperative Karnofsky rating in the two groups was statistically significant (P = 0.002). The gross total resection group spent significantly more time after the operation in an independent status (Karnofsky rating ≥ 80) compared to the subtotal resection group (P = 0.007; median time of 185 and 12.5 weeks, respectively). Gross total resection of supratentorial glioblastomas and anaplastic astrocytomas is feasible and is directly associated with longer and better survival when compared to subtotal resection.


2021 ◽  
pp. 1-9
Author(s):  
Maria R. H. Castro ◽  
Stephen T. Magill ◽  
Ramin A. Morshed ◽  
Jacob S. Young ◽  
Steve E. Braunstein ◽  
...  

OBJECTIVE Tumors compressing the trigeminal nerve can cause facial pain, numbness, or paresthesias. Limited data exist describing how these symptoms change after resection and what factors predict symptom improvement. The objective of this study was to report trigeminal pain and sensory outcomes after tumor resection and identify factors predicting postoperative symptom improvement. METHODS This retrospective study included patients with tumors causing facial pain, numbness, or paresthesias who underwent resection. Trigeminal schwannomas were excluded. Logistic regression, recursive partitioning, and time-to-event analyses were used to report outcomes and identify variables associated with facial sensory outcomes. RESULTS Eighty-six patients met inclusion criteria, and the median follow-up was 3.1 years; 63 patients (73%) had meningiomas and 23 (27%) had vestibular schwannomas (VSs). Meningioma patients presented with pain, numbness, and paresthesias in 56%, 76%, and 25% of cases, respectively, compared with 9%, 91%, and 39%, respectively, for patients with VS. Most meningioma patients had symptoms for less than 1 year (60%), whereas the majority of VS patients had symptoms for 1–5 years (59%). The median meningioma and VS diameters were 3.0 and 3.4 cm, respectively. For patients with meningiomas, gross-total resection (GTR) was achieved in 27% of patients, near-total resection (NTR) in 29%, and subtotal resection (STR) in 44%. For patients with VS, GTR was achieved in 9%, NTR in 30%, and STR in 61%. Pain improved immediately after tumor resection in 81% of patients and in 92% of patients by 6 weeks. Paresthesias improved immediately in 80% of patients, increasing to 84% by 6 weeks. Numbness improved more slowly, with 52% of patients improving immediately, increasing to 79% by 2 years. Pain recurred in 22% of patients with meningiomas and 0% of patients with VSs. After resection, the Barrow Neurological Institute (BNI) facial pain intensity score improved in 73% of patients. The tumor diameter significantly predicted improvement in BNI score (OR 0.47/cm larger, 95% CI 0.22–0.99; p = 0.047). Complete decompression of the trigeminal nerve was associated with qualitative improvement in pain (p = 0.037) and decreased pain recurrence (OR 0.08, 95% CI 0.01–0.67; p = 0.024). CONCLUSIONS Most patients with facial sensory symptoms caused by meningiomas or VSs experienced improvement after resection. Surgery led to immediate and sustained improvement in pain and paresthesias, whereas numbness was slower to improve. Patients with smaller tumors and complete decompression of the trigeminal nerve were more likely to experience improvement in facial pain.


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.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e16702-e16702
Author(s):  
Benjamin Edward Ueberroth ◽  
Alex John Liu ◽  
Thorvardur Ragnar Halfdanarson ◽  
Mohamad Bassam Sonbol

e16702 Background: There are only a few reports examining the treatment patterns for poorly differentiated rectal/anal NEC. In this study, we sought to report treatment and survival outcomes for patients with NEC of the anus and rectum seen at Mayo Clinic. Methods: We identified patients with a primary NEC of the anus or rectum using Mayo Clinic databases (Minnesota, Arizona, Florida) from the year 2000 to present. NEC identified within polyps on colonoscopy was excluded from this study. Patients’ demographics were compiled. Kaplan Meier analyses were performed to evaluate overall survival (OS) for all patients, as well as subgroups with locoregional disease (LRD) and metastatic disease at diagnosis. Progression free survival (PFS) was also evaluated for patients with LRD treated with chemoradiation therapy (CRT). Results: 38 patients with NEC of the anus/rectum were identified with a median age of 55.5 years. 23 patients (61%) had metastatic disease at diagnosis and 15 (39%) had LRD. The most common site of metastasis was the liver (n = 20). Patients with metastatic disease at presentation had significantly shorter OS compared to patients with LRD (median OS 10.4 vs. 18.9 months, p = 0.039; HR 2.46, 95% CI [1.001-6.050]). 34 total patients received chemotherapy (ORR 11.8%), with platinum + etoposide (EP) being the most common first-line chemotherapy (n = 26). 10 of 15 patients with LRD received CRT (9 with EP and 1 with 5-FU) with median PFS of 6.3 months (95% CI 2.3-16.5 months), a 1-year PFS rate of 20%, and median OS of 18.6 months (95% CI 13.5-23.7 months). 2/10 received induction chemotherapy prior to CRT with 6/10 undergoing primary tumor resection (1 prior to CRT and 5 after CRT). Only 3 CRT patients were living at time of analysis (all underwent surgical resection), 2 without any active disease, 1 who achieved complete response with CRT then suffered recurrence and is still undergoing treatment. Conclusions: NEC of the anus and rectum is an aggressive malignancy with most patients experiencing metastatic disease at presentation and poor response to chemotherapy at all stages. Even those with LRD experience a very poor prognosis with OS of 18.9 months from diagnosis. For LRD, most patients underwent CRT with only a short-lived response (median PFS 6.3 months). Those achieving sustained response also underwent surgical resection. Overall, prognosis is poor for all stages and further study on a large scale is warranted to better characterize this rare, aggressive malignancy.


2011 ◽  
Vol 68 (suppl_1) ◽  
pp. ons57-ons63 ◽  
Author(s):  
Elisa J Kucia ◽  
Nicholas C Bambakidis ◽  
Steve W Chang ◽  
Robert F Spetzler

Abstract BACKGROUND: Intramedullary spinal ependymomas are rare tumors. OBJECTIVE: To provide a large retrospective review in the modern neuroimaging era from a tertiary center where aggressive surgical resection is favored. METHODS: Charts of intramedullary spinal ependymomas treated between 1983 and 2006 were reviewed. RESULTS: Sixty-seven cases were reviewed. The mean age was 45.6 years (range, 11-78 years) with a male-to-female ratio of 2:1. The most common location was the cervical spine, followed by the thoracic and lumbar spine. The average duration of symptoms was 33 months, with the most common symptom being pain and/or dysesthesias, followed by weakness, numbness, and urinary or sexual symptoms. Gross total resection was achieved in 55 patients and a subtotal resection was performed in 12 patients; 9 patients were treated with adjuvant radiation therapy. Mean follow-up was 32 months. The mean McCormick neurological grade at last follow-up was 2.0. The preoperative outcome correlated significantly with postoperative outcome (P < .001). A significant number of patients who initially worsened improved at their 3-month follow-up examination. Outcomes were significantly worse in patients undergoing subtotal resection with or without radiation therapy (P < .05). There were 3 recurrences. The overall complication rate was 34%. The primary complications were wound infections or cerebrospinal fluid leaks. CONCLUSION: Spinal cord ependymomas are difficult lesions to treat. Aggressive surgical resection is associated with a high overall complication rate. However, when gross total resection can be achieved, overall outcomes are excellent and the recurrence rate is low.


2020 ◽  
Vol 3 (2) ◽  
pp. 108-119
Author(s):  
Manna Ramadan ◽  
Shaswar Ali

The Posterior fossa is the commonest site of primary intracranial tumors in children, for the last two decades the over-all survival and 5-years progression-free survival of children with posterior fossa tumors (PFT) like Medulloblastoma& Ependymomas has been doubled due to the improvement in the diagnostic tools and the advances in the surgical techniques approaching total or near total resection. The aim of the study is to find the relation of histopathology and the extent of excision with mortality and survival. A total of twenty eight cases with Histologically (26 cases) and two cases radiologically (CT-scan and MRI) confirmed pediatric posterior fossa tumors treated in Erbil Teaching Hospital between,Jan.2013 and Dec.2015 were included in the study As a result the twenty-eight pediatric patients were included in the study, mean age was (8 years),16 boys and 12 girls, mean follow-up period was 14 months,11 cases had Medulloblastoma (39%), 5 cases had Ependymoma (18%), 9 cases had Astrocytoma(32%) , 2 cases had Brainstem mass (7%) and one case had Choroid plexus papilloma (4%).Tumor resection was performed in 26 patients, Twenty cases had total resection (77%), and six Pts .had subtotal resection (23%), two cases without surgery During the follow-up period out of the 20 cases that had total resection nineteen are still alive(95%) and only one died (5%),six cases that had Subtotal resection; four of them are dead (67%) and only two cases are still alive(33%). Two cases that had no surgery both of them are dead (100%).Eleven cases of Medulloblastoma 8 of them had gross total resection and three of them had subtotal resection, 9 of them still alive (82%) and two are dead (18%). Nine Pts of Astrocytoma, 8 of them had total resection and one subtotal; eight of them are still .)alive (89%) and only one died (11%Five pts with Ependymoma, three had total resection and two had subtotal resection,three are still alive (60%) and two dead (40%). Two cases of Brainstem mass not operated and both are dead, One case of Choroid papilloma totally resected & still alive. In conclusion treatment of posterior fossa tumors in children with surgery yields long survival rates, children with gross total resection or a near total resection had better outcome. Histopathology subtypes of the tumors were associated with a favorable outcome for Astrocytoma which has less mortality and better survival rate than others


Author(s):  
Valeri Borger ◽  
Motaz Hamed ◽  
Inja Ilic ◽  
Anna-Laura Potthoff ◽  
Attila Racz ◽  
...  

Abstract Introduction The postoperative seizure freedom represents an important secondary outcome measure in glioblastoma surgery. Recently, supra-total glioblastoma resection in terms of anterior temporal lobectomy (ATL) has gained growing attention with regard to superior long-term disease control for temporal-located glioblastoma compared to conventional gross-total resections (GTR). However, the impact of ATL on seizure outcome in these patients is unknown. We therefore analyzed ATL and GTR as differing extents of resection in regard of postoperative seizure control in patients with temporal glioblastoma and preoperative symptomatic seizures. Methods Between 2012 and 2018, 33 patients with preoperative seizures underwent GTR or ATL for temporal glioblastoma at the authors’ institution. Seizure outcome was assessed postoperatively and 6 months after tumor resection according to the International League Against Epilepsy (ILAE) classification and stratified into favorable (ILAE class 1) versus unfavorable (ILAE class 2–6). Results Overall, 23 out of 33 patients (70%) with preoperative seizures achieved favorable seizure outcome following resection of temporal located glioblastoma. For the ATL group, postoperative seizure freedom was present in 13 out of 13 patients (100%). In comparison, respective rates for the GTR group were 10 out of 20 patients (50%) (p = 0.002; OR 27; 95% CI 1.4–515.9). Conclusions ATL in terms of a supra-total resection strategy was associated with superior favorable seizure outcome following temporal glioblastoma resection compared to GTR. Regarding above mentioned survival benefit following ATL compared to GTR, ATL as an aggressive supra-total resection regime might constitute the surgical modality of choice for temporal-located glioblastoma.


2017 ◽  
Vol 126 (2) ◽  
pp. 418-430 ◽  
Author(s):  
Sivashanmugam Dhandapani ◽  
Harminder Singh ◽  
Hazem M. Negm ◽  
Salomon Cohen ◽  
Mark M. Souweidane ◽  
...  

OBJECTIVE Craniopharyngiomas can be difficult to remove completely based on their intimate relationship with surrounding visual and endocrine structures. Reoperations are not uncommon but have been associated with higher rates of complications and lower extents of resection. So radiation is often offered as an alternative to reoperation. The endonasal endoscopic transsphenoidal approach has been used in recent years for craniopharyngiomas previously removed with craniotomy. The impact of this approach on reoperations has not been widely investigated. METHODS The authors reviewed a prospectively acquired database of endonasal endoscopic resections of craniopharyngiomas over 11 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital, performed by the senior authors. Reoperations were separated from first operations. Pre- and postoperative visual and endocrine function, tumor size, body mass index (BMI), quality of life (QOL), extent of resection (EOR), impact of prior radiation, and complications were compared between groups. EOR was divided into gross-total resection (GTR, 100%), near-total resection (NTR, > 95%), and subtotal resection (STR, < 95%). Univariate and multivariate analyses were performed. RESULTS Of the total 57 endonasal surgical procedures, 22 (39%) were reoperations. First-time operations and reoperations did not differ in tumor volume, radiological configuration, or patients' BMI. Hypopituitarism and diabetes insipidus (DI) were more common before reoperations (82% and 55%, respectively) compared with first operations (60% and 8.6%, respectively; p < 0.001). For the 46 patients in whom GTR was intended, rates of GTR and GTR+NTR were not significantly different between first operations (90% and 97%, respectively) and reoperations (80% and 100%, respectively). For reoperations, prior radiation and larger tumor volume had lower rates of GTR. Vision improved equally in first operations (80%) compared with reoperations (73%). New anterior pituitary deficits were more common in first operations compared with reoperations (51% vs 23%, respectively; p = 0.08), while new DI was more common in reoperations compared with first-time operations (80% vs 47%, respectively; p = 0.08). Nonendocrine complications occurred in 2 (3.6%) first-time operations and no reoperations. Tumor regrowth occurred in 6 patients (11%) over a median follow-up of 46 months and was not different between first versus reoperations, but was associated with STR (33%) compared with GTR+NTR (4%; p = 0.02) and with not receiving radiation after STR (67% vs 22%; p = 0.08). The overall BMI increased significantly from 28.7 to 34.8 kg/m2 over 10 years. Six months after surgery, there was a significant improvement in QOL, which was similar between first-time operations and reoperations, and negatively correlated with STR. CONCLUSIONS Endonasal endoscopic transsphenoidal reoperation results in similar EOR, visual outcome, and improvement in QOL as first-time operations, with no significant increase in complications. EOR is more impacted by tumor volume and prior radiation. Reoperations should be offered to patients with recurrent craniopharyngiomas and may be preferable to radiation in patients in whom GTR or NTR can be achieved.


2007 ◽  
Vol 61 (suppl_5) ◽  
pp. ONS202-ONS211 ◽  
Author(s):  
Nicholas C. Bambakidis ◽  
U. Kumar Kakarla ◽  
Louis J. Kim ◽  
Peter Nakaji ◽  
Randall W. Porter ◽  
...  

Abstract Objective: We examined the surgical approaches used at a single institution to treat petroclival meningioma and evaluated changes in method utilization over time. Methods: Craniotomies performed to treat petroclival meningioma between September of 1994 and July of 2005 were examined retrospectively. We reviewed 46 patients (mean follow-up, 3.6 yr). Techniques included combined petrosal or transcochlear approaches (15% of patients), retrosigmoid craniotomies with or without some degree of petrosectomy (59% of patients), orbitozygomatic craniotomies (7% of patients), and combined orbitozygomatic-retrosigmoid approaches (19% of patients). In 18 patients, the tumor extended supratentorially. Overall, the rate of gross total resection was 43%. Seven patients demonstrated progression over a mean of 5.9 years. No patients died. At 36 months, the progression-free survival rate for patients treated without petrosal approaches was 96%. Of 14 patients treated with stereotactic radiosurgery, none developed progression. Conclusion: Over the study period, a diminishing proportion of patients with petroclival meningioma were treated using petrosal approaches. Utilization of the orbitozygomatic and retrosigmoid approaches alone or in combination provided a viable alternative to petrosal approaches for treatment of petroclival meningioma. Regardless of approach, progression-free survival rates were excellent over short-term follow-up period.


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