scholarly journals Infratentorial choroid plexus tumors in children

2020 ◽  
Vol 36 (8) ◽  
pp. 1761-1766 ◽  
Author(s):  
S. Joy Trybula ◽  
Constantine Karras ◽  
Robin M. Bowman ◽  
Tord D. Alden ◽  
Arthur J. DiPatri ◽  
...  

Abstract Objective Choroid plexus tumors (CPTs) are rare pediatric intracranial neoplasms, and mostly occur in the lateral ventricle. CPTs located in the infratentorial location are considered to be rare in the pediatric population. We present a series of eight patients treated in the last decade at our institution focusing on clinical presentations and their outcome after excision. Methods We performed an institutional retrospective review of patients who underwent surgical resection of infratentorial CPTs during the period from 2008 to 2017. Patients’ charts were reviewed for demographic data, clinical presentation, surgical treatment, and follow-up. Results There were eight patients (6 females and 2 males), with mean age for the cohort at presentation was 9.0 years. They represent 75% of 12 CPTs of all locations treated at the same period in our institution. These 8 infratentorial CPTs were in the fourth ventricle in seven, and in the cerebellopontine angle (CPA) in one. Seven patients had choroid plexus papillomas (WHO grade I) and 1 had an atypical choroid plexus papilloma (WHO grade II). Gross total resection was attempted in all patients. However, two of 3 patients with fourth ventricle floor invasion had subtotal resection with a thin layer of tumor left on the floor. The remaining 6 had a gross total resection. Six patients with preoperative hydrocephalus had a perioperative external ventricular drainage but none required permanent shunting after tumor resection. None showed recurrence/tumor progression without adjuvant therapy during the follow-up period of 20 months to 11 years. Conclusion Infratentorial dominance among pediatric CPTs in this series contradicts previous reports. Infratentorial CPTs are amenable to surgical resection. Unresected small residuals due to invasion to the fourth ventricle floor showed no regrowth during 2 to 3 years follow-up without adjuvant therapy. However, these patients with incomplete resection need watchful observations.

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.


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 ◽  
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.


2018 ◽  
Vol 79 (S 05) ◽  
pp. S424-S425
Author(s):  
Ilhan Aydin ◽  
Sima Sayyahmelli ◽  
Mark Pyle ◽  
Mustafa Baskaya

Surgical resection of jugular foramen tumors poses a significant challenge to skull base surgeons with the selection of an appropriate surgical approach, a matter of some debate. Jugular foramen metastatic tumors may mimic paragangliomas, and in some selected cases surgical resection is needed. In this video, we demonstrate the microsurgical gross total resection of a jugular foramen tumor via a postauricular trans-jugular trans-sigmoid approach. The patient is a 61-year-old man with a 7-year history of medullary thyroid cancer, who underwent three neck operations and radiation to the neck. He developed lower cranial nerve palsies (IX, X, and XI) with preoperative aspiration deficits, dysphonia, status post phonosurgery for vocal cord paralysis, profound sensorineural hearing loss, and muscle atrophy of the left shoulder. He initially received stereotactic radiation of the jugular foramen tumor at an outside hospital without histopathological diagnosis. Follow-up magnetic resonance images (MRIs) showed progressive enlargement of the tumor over the postradiation year. The decision was made to resect this tumor to enable histopathological diagnosis, and to provide local tumor control, since his primary disease has been stable. He underwent microsurgical gross total resection via a transjugular transsigmoid approach. After skeletonizing the sigmoid sinus and jugular bulb, the sigmoid sinus was ligated and rolled toward the jugular bulb, where the major part of the tumor was. Then, using the transjugular route, the tumor was removed en bloc. The surgery and postoperative course were uneventful. The histopathology was a thyroid medullary cancer metastasis. He was followed with serial MRIs, and there was no recurrent tumor at 2 years follow-up. In this video, microsurgical techniques and important steps for the resection of a jugular foramen metastatic tumor are demonstrated.The link to the video can be found at: https://youtu.be/oXC6fX2CC84.


2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii72-iii72
Author(s):  
F Colò ◽  
L Larrouquere ◽  
R Rivoirard ◽  
H Loiseau ◽  
A Lortholary ◽  
...  

Abstract BACKGROUND Choroid plexus tumours represent less than 1% of brain tumours. Low-grade papilloma may be treated with gross total surgical resection, while in disseminated progressive atypical choroid plexus papilloma (APP), there is no standard treatment: various chemotherapy regimens have been reported. Since this tumour is characterized by a rich vascular component, antiangiogenic therapy is an attractive treatment. The use of Bevacizumab has already been reported in three patients. Authors expand this experience with 5 further patients diagnosed with progressive APP treated with bevacizumab. MATERIAL AND METHODS Patients were recruited through the weekly Adolescent Young Adult French web conference. They have been treated with bevacizumab 10 mg/kg by intravenous injection each 2 to 3 weeks. Their clinical status and radiological response are reported: Karnofsky Index (KI), Pain Scale (PS) and RANO criteria were used. RESULTS All our patients had a progressive disease prior to bevacizumab. Pt 1: 41 years old; APP with cranio-spinal dissemination; Previous treatments: local and cranio-spinal irradiation in 2012 and in 2014; surgery: complete and partial resection in 2010, 2014, and VP shunt in 2018; Bevacizumab: total of 33 cures in 18 months. Result: radiological and clinical stabilization. Pt 2: 58 years old; APP with cranio-spinal dissemination; Previous treatments: surgery: VP shunt and gross total resection, in 2006; VP shunt, in 2011. Bevacizumab: total of 4 cures, in 2 months. Result: radiological and clinical stabilization. Pt 3: 34 years old; APP with cranio-spinal dissemination; Previous treatments: surgery: gross total resection, in 1992, and shunt in 2008. Bevacizumab: total of 21 cures, in 21 months. Result: radiological and clinical stabilization. Pt 4: 63 years old; APP with cranio-spinal dissemination; Previous treatments: surgery: surgical resection and VP shunt in 2013; chemotherapy: temozolomide. Bevacizumab: 29 months (still treated). Result: radiological and clinical stabilization. Pt 5: 62 years old; APP with cranio-spinal dissemination; Previous treatments: surgery: gross total resection in 1999 and VP shunt in 2009; chemotherapy: Carboplatin Vespesid. Bevacizumab: 23 cures, in 14 months. Result: radiological stabilization and clinical amelioration. CONCLUSION Despite their previous worsening disease, all patients obtained a stabilization or amelioration of their IK and PS under bevacizumab. Bevacizumab should be evaluated in a multicentric trial as standard therapy for disseminated metastasized progressive choroid plexus tumours.


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.


Author(s):  
Per Sveino Strand ◽  
Ole Solheim

Abstract Background The role of adjuvant radiotherapy after gross total resection (GTR) of WHO grade 2 meningioma remains unclear, and conflicting results have been published. We hypothesized that authors’ medical specialties could be associated with reported findings on the role of adjuvant radiotherapy after GTR of WHO grade 2 meningiomas. Method A systematic review was conducted in Embase and Medline databases, in addition to screening of all relevant bibliographies. Articles including patients aged 18 years or older, with histologically confirmed WHO grade 2 meningioma, were included. We extracted data on medical subspecialties using the author list. We registered study design, median follow-up, number of included patients, WHO classification in use, and years of study inclusion. Results Thirty-seven relevant studies were identified, where 34 (92%) were retrospective cohort studies, two studies (5%) were systematic reviews, and one study (3%) was a meta-analysis. If the last author was a radiation-oncologist, the study was more likely to favor adjuvant radiotherapy, and if a neurosurgeon was last author, the study was more likely to not advocate adjuvant radiotherapy (p=0.009). There was no significant association between study result and whether the study was published in a neurosurgical or oncological journal (p=0.802). There was no significant difference in follow-up time, years of inclusion, or number of included patients between studies favoring or not favoring adjuvant radiotherapy. Conclusions In this systematic review of the literature, we found that if a radiation-oncologist was the last author of the study, the study was more likely to favor adjuvant radiotherapy after gross total resection of WHO grade 2 meningioma. Clinicians and researchers should be aware of a possible genealogy bias in the neuro-oncological literature.


Author(s):  
Sima Sayyahmelli ◽  
Emel Avci ◽  
Burak Ozaydin ◽  
Mustafa K. Başkaya

AbstractTrigeminal schwannomas are rare nerve sheet tumors that represent the second most common intracranial site of occurrence after vestibular nerve origins. Microsurgical resection of giant dumbbell-shaped trigeminal schwannomas often requires complex skull base approaches. The extradural transcavernous approach is effective for the resection of these giant tumors involving the cavernous sinus.The patient is a 72-year-old man with headache, dizziness, imbalance, and cognitive decline. Neurological examination revealed left-sided sixth nerve palsy, a diminished corneal reflex, and wasting of temporalis muscle. Magnetic resonance imaging (MRI) showed a giant homogeneously enhancing dumbbell-shaped extra-axial mass centered within the left cavernous sinus, Meckel's cave, and the petrous apex, with extension to the cerebellopontine angle. There was a significant mass effect on the brain stem causing hydrocephalus. Computed tomography (CT) scan showed erosion of the petrous apex resulting in partial anterior autopetrosectomy (Figs. 1 and 2).The decision was made to proceed with tumor resection using a transcavernous approach. Gross total resection was achieved. The surgery and postoperative course were uneventful, and the patient woke up the same as in the preoperative period. MRI confirmed gross total resection of the tumor. The histopathology was a trigeminal schwannoma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence at 15-month follow-up.This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors.The link to the video can be found at https://youtu.be/TMK5363836M


2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Sachi Kawagishi ◽  
Naoko Ose ◽  
Masato Minami ◽  
Soichiro Funaki ◽  
Takashi Kanou ◽  
...  

Abstract Background Thymic carcinoma has been classified into 12 subtypes, thymic lymphoepithelioma-like carcinoma (LELC) is a type of them, and has a pathological organization similar to that of lymphoepithelioma, an undifferentiated type of nasopharyngeal carcinoma. According to a report from the International Thymic Malignancy Interest Group (ITMIG), thymic LELC is a rare tumor and accounts for 6% of all thymic carcinoma cases. We report two cases of surgical resection for thymic LELC and perform a search of other reports of thymic LELC, and clinical manifestations and follow-up data thus obtained are summarized. Case presentation Two patients underwent surgical resection for thymic LELC. In both, tumors were detected in the anterior mediastinum and a total thymectomy was performed. Each was diagnosed with thymic LELC and classified in accordance with the Masaoka staging system as modified stage II. In recent examinations, one patient was doing well after undergoing total resection, whereas early recurrence of distant lymph node metastasis was noted in the other at 5 months after the total resection procedure and died thereafter from a different disease. Conclusion We report two cases of surgical resection for thymic LELC. A successful total resection may positively affect prognosis: thus, long-term follow-up examinations must be performed.


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