Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation

2013 ◽  
Vol 11 (5) ◽  
pp. 518-525 ◽  
Author(s):  
Yui Mano ◽  
Toshihiro Kumabe ◽  
Ichiyo Shibahara ◽  
Ryuta Saito ◽  
Yukihiko Sonoda ◽  
...  

Dysembryoplastic neuroepithelial tumors (DNETs) have conventionally been regarded as benign and stable tumors and considered curable with surgery without adjunctive therapy. Recently, recurrent DNETs with or without malignant transformation have been described. The authors report 2 unusual cases of DNET: 1) an enlarging lesion that developed an enhancing component over the natural course of 4 years, and 2) a recurrent DNET that developed an enhancing component 10–11 years after gross-total resection. The patient in the first case was treated with subtotal resection and adjuvant radiochemotherapy; histological examination of the tumor led to the diagnosis of DNET, WHO Grade I, for the nonenhancing component and anaplastic oligodendroglioma, WHO Grade III, for the enhancing component. The patient in the second case was treated with repeat gross-total resection; the original tumor had been histologically diagnosed as DNET, and the nonenhancing and enhancing components of the recurrent tumor were diagnosed as simple and complex forms of DNET, respectively. These and previous reports suggest an aggressive subtype of DNETs. If follow-up MRI reveals progressive behavior, resection should be performed without delay. Additional radiochemotherapy is needed if the histological diagnosis demonstrates malignant transformation.

2020 ◽  
Vol 36 (11) ◽  
pp. 2693-2700
Author(s):  
Stephanie T. Jünger ◽  
Felipe Andreiuolo ◽  
Martin Mynarek ◽  
Evelyn Dörner ◽  
Anja zur Mühlen ◽  
...  

Abstract Introduction Young age is an adverse prognostic factor in children with ependymomas. Treatment of these infants is challenging since beneficial therapeutic options are limited. As ependymomas are considered a biologically heterogeneous group, we aimed to characterize infant ependymomas with regard to their histological and genetic features. Materials and methods We analyzed 28 ependymomas occurring in children younger than 18 months at diagnosis enrolled into the HIT2000-E protocols with the aim to postpone irradiation until the age of 18 months if possible. All cases underwent neuropathological review, including immunohistochemical characterization. Genome-wide copy number alterations (CNA) were assessed by molecular inversion probe assays, and RELA and YAP1 fusions were detected by RT-PCR and sequencing. Results All infant ependymomas were anaplastic (WHO grade III). Twenty-one (75%) cases were located in the posterior fossa. Gross total resection was accomplished in 12 (57%) of these cases. All posterior fossa tumors showed loss of H3-K27me3 characteristic of PFA ependymomas. CNA analysis showed a stable genome in all cases with lack of chromosome 1q gain, an adverse prognostic marker in PFA ependymomas of older children. However, after a median follow-up of 5.4 years, 15 (71%) relapsed, and 9 (43%) died. Seven ependymomas (25%) occurred in the supratentorial region. Gross total resection could be achieved in only two of these cases. Four tumors carried C11orf95-RELA fusions, and two cases had typical YAP1-MAMLD1 fusions (one case was not analyzable). The RELA-fused cases did not display CDKN2A loss as an adverse indicator of prognosis in this disease entity. Although three infants (43%) with supratentorial ependymomas relapsed, all patients survived (median follow-up, 8.0 years). Conclusion Infant ependymomas seem to fall into three biological entities, with supratentorial tumors carrying RELA or YAP fusions and PFA posterior fossa ependymomas. The latter showed a poor outcome even though chromosome 1q gain was absent.


2011 ◽  
Vol 68 (suppl_1) ◽  
pp. ons90-ons94 ◽  
Author(s):  
Elisa J Kucia ◽  
Peter H Maughan ◽  
Udaya K Kakarla ◽  
Nicholas C Bambakidis ◽  
Robert F Spetzler

Abstract BACKGROUND: Myxopapillary ependymomas usually occur in the filum terminale of the spinal cord. OBJECTIVE: This report summarizes our experience treating myxopapillary ependymomas. METHODS: The records of 34 patients (14 men, 20 women; mean age 45.5 years; age range, 14-88 years) who underwent resection of a myxopapillary ependymoma between 1983 and 2006 were reviewed for age, sex, tumor location, symptoms at diagnosis, duration of symptoms, treatment before presentation, extent of surgical resection, adjuvant therapy, length of follow-up, evidence of recurrence, and complications. Neurological examinations performed at presentation, immediately after surgery, and last follow-up were graded according to the McCormick grading scale. RESULTS: The average duration of symptoms before diagnosis was 22.2 months. The most common symptom was pain followed by weakness, bowel/bladder symptoms, and numbness. The rate of gross total resection was 80%. All patients with a subtotal resection (20%) underwent postoperative radiation therapy. Presentation and outcomes of patients who underwent subtotal resection followed by radiation therapy were compared with those who underwent gross total resection. There was no significant difference in neurological grade between the groups at presentation or final follow-up. The overall recurrence rate was 10% (3/34 patients). CONCLUSION: The goal of surgical treatment of myxopapillary ependymomas is resection to the greatest extent possible with preservation of function. In cases of subtotal resection, postoperative radiation therapy may improve outcome. If neurological function is maintained at treatment, these indolent lesions allow years of good function.


2020 ◽  
Vol 11 ◽  
pp. 409
Author(s):  
Diego Ochoa-Cacique ◽  
María Córdoba-Mosqueda ◽  
José Ramón Aguilar-Calderón ◽  
Martha Cristina Sánchez-Silva ◽  
Rosa María Vicuña-González ◽  
...  

Background: Choroid plexus papillomas (CPPs) are benign extra-axial tumors that originate from the choroid plexus; these tumors rarely have metastases, being at the spinal level the location with few reported cases. Case Description: We report the case of a 48-year-old man with a history of atypical fourth ventricular CPP and gross total resection (GTR) in 2008. In 2015, he presented with radicular pain, decreased strength, and paresthesia in the left leg. Magnetic resonance imaging revealed a well-defined intradural ovoid lesion in the vertebral canal at the level of the L3-L4 intersomatic space. Subtotal resection of the tumor was performed. The patient recovered well, with relief of pain and no neurological deficit. A literature research few cases of CPP metastasis in adults. We describe here a fifth-decade male patient with a lumbar neoplasm, which according to the histopathologic characteristics and location is the first case of an atypical papilloma implant of the choroid plexus at this spinal level. Conclusion: GTR of primary lesions and associated implants remains the gold standard for surgical treatment of CPP. Radiotherapy, stereotactic radiosurgery, and chemotherapy are adjuvant therapies for CPP but there is no definitive protocol for the management of implants. Proper follow-up of these patients is essential since spinal drop can appear many years after the initial presentation of CPP.


2021 ◽  
Author(s):  
Congxin Dai ◽  
Ming Feng ◽  
Lin Lu ◽  
Bowen Sun ◽  
Yanghua Fan ◽  
...  

Abstract Objective: Surgery is first-line treatment for corticotroph adenomas. Although most of corticotroph adenomas are noninvasive microadenomas that show expansive growth to surrounding tissues, a small subset of them is locally invasive and difficult to manage. The aim of this study was to evaluate surgical outcome of invasive corticotroph adenomas from a single-center. Patients and Methods: The clinical features and outcomes of CD patients who underwent transsphenoidal surgery (TSS) between January 2000 and September 2019 at Peking Union Medical College Hospital were collected from medical records. The clinical, endocrinological, radiological, histopathological, surgical outcomes and a minimum 12-month follow-up of 86 consecutive CD patients with invasive corticotroph adenomas were retrospectively reviewed. Results: Eighty-six patients with invasive corticotroph adenomas were included in the study. The average age at TSS was 37.7 years (range, 12 to 67 years), with a female-to-male ratio of 3.1:1 (65/21). The median duration of symptoms was 52.6 months (range, 1.0 to 264 months). The average of maximum diameter of tumor was 17.6 mm (range, 4.5–70 mm). All 86 patients with invasive corticotroph adenomas were performed TSS by microscopic or endoscopic approach. Gross-total resection was achieved in 63 patients (73.3%), subtotal resection in 18 (20.9%), and partial resection in 5 (5.8%). After surgery, the overall postoperative immediate remission rate was 48.8% (42/86), 51.2 % (44/86) of patients maintained persistent hypercortisolism. In 42 patients with initial remission, 16.7 % (7/42) of them experienced a recurrence. In these patients with persistent disease and recurrent CD, data about further treatment was available for 30 patients. The radiotherapy was used for 15 patients, and 4 (26.7%) of them achieved biochemical remission. Repeat TSS was performed in 5 patients, and none achieved remission. Medication was administrated in 4 patients, and one of them obtained disease control. Adrenalectomy was performed in 6 patients, and 5 (83.3 %) achieved biochemical remission. At last follow-up, (33.3%) 10 of 30 patients were in remission, and 20 patients still had persistent disease. The remission rate in patients with invasive corticotroph adenomas who underwent gross-total resection and first TSS were significantly higher than that in patients undergoing subtotal resection, partial resection, and a second TSS (all P<0.05). However, there was no significant difference in the remission rate between patient with different tumor size, Knosp Grade and surgical approaches (P>0.05).Conclusion: The management of invasive corticotroph adenomas remain a therapeutic challenge due to incomplete resection of invasive and/or a large adenoma. With application of multiple techniques assistance, approximately half of the patients could achieve gross-total resection and biochemical remission via TSS by experienced neurosurgeons. The extent of tumor resection and number of operations were associated with surgical remission rate in invasive corticotroph adenomas. If the remission was not achieved by surgery, other treatments including radiotherapy, medical therapy, and even bilateral adrenalectomy are required.


2020 ◽  
pp. 1-10 ◽  
Author(s):  
Cecilia L. Dalle Ore ◽  
Stephen T. Magill ◽  
Roberto Rodriguez Rubio ◽  
Maryam N. Shahin ◽  
Manish K. Aghi ◽  
...  

OBJECTIVEHyperostosing sphenoid wing meningiomas cause bony hyperostosis that may extend into the orbit, resulting in proptosis, restriction of extraocular movements, and/or compressive optic neuropathy. The extent of bony removal necessary and the optimal reconstruction strategy to prevent enophthalmos is debated. Herein, the authors present their surgical outcomes and reconstruction results.METHODSThis is a retrospective review of 54 consecutive patients undergoing resection of sphenoid wing meningiomas associated with bony hyperostosis. The majority of cases were operated on by the senior author. Extent of tumor resection, volumetric bone resection, radiographic exophthalmos index, complications, and recurrence were analyzed.RESULTSThe median age of the cohort was 52.1 years, with women comprising 83% of patients. Proptosis was a presenting symptom in 74%, and 52% had decreased visual acuity. The WHO grade was I (85%) or II (15%). The median follow-up was 2.6 years. On volumetric analysis, a median 86% of hyperostotic bone was resected. Gross-total resection of the intracranial tumor was achieved in 43% and the orbital tumor in 27%, and of all intracranial and orbital components in 20%. Orbital reconstruction was performed in 96% of patients. Postoperative vision was stable or improved in 98% of patients and diplopia improved in 89%. Postoperative complications occurred in 44% of patients, and 26% of patients underwent additional surgery for complication management. The most frequent complications were medical complications and extraocular movement deficits. The median preoperative exophthalmos index was 1.26, which improved to 1.12 immediately postoperatively and to 1.09 at the 6-month follow-up (p < 0.001). Postoperatively, 18 patients (33%) underwent adjuvant radiotherapy after subtotal resection. Tumors recurred/progressed in 12 patients (22%).CONCLUSIONSResection of hyperostosing sphenoid wing meningiomas, particularly achieving gross-total resection of hyperostotic bone with a good aesthetic result, is challenging and associated with notable medical and ocular morbidity. Recurrence rates in this series are higher than previously reported. Nevertheless, the authors were able to attain improvement in proptosis and visual symptoms in the majority of patients by using a multidisciplinary approach.


Neurosurgery ◽  
2006 ◽  
Vol 58 (6) ◽  
pp. 1090-1098 ◽  
Author(s):  
Fotios Tzortzidis ◽  
Foad Elahi ◽  
Donald C. Wright ◽  
Nancy Temkin ◽  
Sabareesh K. Natarajan ◽  
...  

Abstract OBJECTIVE: To evaluate patient clinical outcome and survival at long-term follow-up after aggressive microsurgical resection of chondrosarcomas of the cranial base. METHODS: Over a 20-year period, 47 patients underwent 72 operative procedures for resection of cranial base chondrosarcomas. Thirty-three patients were previously untreated, whereas 14 patients previously had undergone surgery or radiation. Twenty-three patients had a single operation and 24 underwent staged (more than one) operations because of extensive disease. Patients who underwent subtotal resection also underwent radiotherapy or radiosurgery. Patients were evaluated at follow-up clinically and by imaging studies. RESULTS: Gross total resection was accomplished in 29 (61.7%) patients, and subtotal resection was accomplished in 18 patients (38.3%). The resection was better in patients who underwent a primary operation (gross total resection, 68.8 versus 46.7%) rather than a reoperation. Patients who underwent incomplete resection underwent postoperative radiotherapy, which included proton beam radiotherapy (15.6%), radiosurgery (68%), and fractionated radiation (15.6%). There were no operative deaths. Postoperative complications (cerebrospinal fluid leakage, quadriparesis, infections, cranial nerve palsies, etc.) were observed in 10 patients (18%). The follow-up ranged from 2 to 255 months, with an average of 86 months. At the conclusion of study, 36 (76.6%) patients were alive, and 21 (44.7%) patients were alive without disease. Recurrence-free survival was 32% at 10 years in all patients, 42.3% in primary patients and 13.8% in those who underwent reoperation. The Karnofsky performance score was 82.4 ± 9.8 before surgery, 85 ± 12.5 at 1 year after surgery, and 85.3 ± 5.8 at the latest follow-up. Two patients died as a result of radiotherapy complications (malignancy, radiation necrosis). CONCLUSION: Cranial base chondrosarcomas can be managed well by complete surgical resection or by a combination of surgery and radiotherapy. The study cannot comment about the efficacy of radiotherapy. Approximately half of the patients survived without recurrence at long-term follow-up (&gt;132 mo). The functional status of the surviving patients was excellent at follow-up.


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.


Neurosurgery ◽  
2014 ◽  
Vol 75 (3) ◽  
pp. 205-214 ◽  
Author(s):  
Chiaojung Jillian Tsai ◽  
Yucai Wang ◽  
Pamela K. Allen ◽  
Anita Mahajan ◽  
Ian E. McCutcheon ◽  
...  

Abstract BACKGROUND: The role of radiotherapy after surgery for myxopapillary ependymoma (MPE) is unclear. OBJECTIVE: To review long-term outcomes after surgery, with or without radiation, for spinal MPE. METHODS: Fifty-one patients with spinal MPE treated from 1968 to 2007 were included. Associations between clinical variables and overall survival (OS), progression-free survival (PFS), and local control (LC) were tested with Cox regression analysis. RESULTS: The median age at diagnosis was 35 years (range, 8-63 years). Twenty patients (39%) had surgery alone, 30 (59%) had surgery plus radiotherapy (RT), and 1 (2%) had RT only. At a median follow-up of 11 years (range, 0.2-37 years), 10-year OS, PFS, and LC for the entire group were 93%, 63%, and 67%, respectively. Nineteen patients (37%) had disease recurrence, and the recurrence was mostly local (79%). Twenty-eight of 50 patients who had surgery (56%) had gross total resection; 10-year LC was 56% after surgery vs 92% after surgery and RT (log-rank P = .14); the median time of LC was 10.5 years for patients receiving gross total resection plus RT, and 4.75 years for gross total resection only (P = .03). Among 16 patients with subtotal resection and follow-up data, 10-year LC was 0% after surgery vs 65% for surgery plus RT (log-rank P = .008). On multivariate analyses adjusting for resection type, age older that 35 years at diagnosis and receipt of adjuvant radiation were associated with improved PFS (hazard ratio [HR]: 0.14, P = .003 and HR: 0.45, P = .009) and LC (HR: 0.22, P = .02 and HR: 0.45, P = .009). CONCLUSION: Postoperative radiotherapy after resection of MPE was associated with improved PFS and LC.


2019 ◽  
Vol 23 (6) ◽  
pp. 726-731
Author(s):  
Arvind C. Mohan ◽  
Howard L. Weiner ◽  
Carrie A. Mohila ◽  
Adekunle Adesina ◽  
Murali Chintagumpala ◽  
...  

OBJECTIVEThe indication for and timing of surgery for epilepsy associated with low-grade mixed neuronal-glial tumors may be controversial. The purpose of this study was to evaluate the effect of resection and associated variables on epilepsy and on progression-free survival (PFS).METHODSA retrospective chart review of patients treated between 1992 and 2016 was conducted to identify individuals with epilepsy and low-grade gliomas or neuronal-glial tumors who underwent resective surgery. Data analyzed included age at epilepsy onset, age at surgery, extent of resection, use of electrocorticography, the number of antiepileptic drugs (AEDs) before and after surgery, the presence of dysplasia, Engel class, histological findings, and PFS. The institutional review board protocol was specifically approved to conduct this study.RESULTSA total of 107 patients were identified. The median follow-up was 4.9 years. The most common pathology was dysembryoplastic neuroepithelial tumor (36.4%), followed by ganglioglioma (31.8%). Eighty-four percent of patients had Engel class I outcomes following surgery. Gross-total resection was associated with a higher likelihood of an Engel class I outcome (90%) as compared to subtotal resection (58%) (p = 0.0005). Surgery reduced the AED burden, with 40% of patients requiring no AEDs after surgery (p < 0.0001). Children with neurodevelopmental comorbidities (n = 5) uniformly did not experience seizure improvement following resection (0% vs 83% overall; p < 0.0001). Electrocorticography was used in 33% of cases and did not significantly increase class I outcomes. PFS was 90% at 5 years. Eleven percent of tumors recurred, with subtotal resection more likely to result in recurrence (hazard ratio 5.3, p = 0.02). Histological subtype showed no significant impact on recurrence.CONCLUSIONSGross-total resection was strongly associated with Engel class I outcome and longer PFS. Further studies are needed to elucidate the suitable time for surgery and to identify factors associated with oncological transformation.


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 &lt; .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 &lt; .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.


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