Contemporary Management of Jugular Paragangliomas With Neural Preservation

2020 ◽  
pp. 019459982093866
Author(s):  
Nauman F. Manzoor ◽  
Kristen L. Yancey ◽  
Joseph M. Aulino ◽  
Alexander D. Sherry ◽  
Mohamed H. Khattab ◽  
...  

Objectives Management of jugular paragangliomas (PGL) has evolved toward subtotal resection (STR). The purpose of this study is to analyze neural preservation and adjuvant treatment for long-term local control. Study Design Retrospective chart review. Settings Tertiary neurotology practice. Subjects and Methods Adults undergoing surgical treatment of jugular PGL between 2006 and 2019. Patients, disease, and treatment variables were collected retrospectively. Single predictor logistic regression was used to ascertain predictors of regrowth or need for salvage radiation. Results A total of 41 patients (median age, 47 years; 76% female) were identified. Most patients presented with advanced-stage disease (Glasscock-Jackson stage III-IV = 76%). Subtotal resection (STR) was performed in 32 (78%) patients. Extended STR (type 1) was the most commonly performed conservative procedure (n = 19, 59%). Postoperative new low cranial neuropathy (LCN) involving CN X and XII was rare (n = 3 and n = 1, respectively). Seventeen patients (41%) underwent postsurgical therapy for tumor regrowth or recurrence, including 15 patients who underwent adjuvant (n = 4) or salvage (n = 11) radiation. Overall tumor control of 94.7% was achieved at a mean follow-up of 35 months. All patients treated with combined modality treatment had local control at last follow-up. Logistic regression identified no single predictor for postsurgical radiation treatment or salvage-free survival. Conclusion Management of jugular PGL with a conservative approach is safe and effective with a low rate of new LCN deficit. Active surveillance of residual tumor with salvage radiation for growth results in excellent long-term tumor control.

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi47-vi48
Author(s):  
Michael Carrasquilla ◽  
Alexander Tai ◽  
Matthew Forsthoefel ◽  
Edina Wang ◽  
Siyuan Lei ◽  
...  

Abstract PURPOSE Meningiomas are the most commonly diagnosed primary intracranial tumor. Resection and single-fraction radiosurgery are treatment options with well-established long-term outcomes data. Multisession radiosurgery is an alternative treatment option with promising early results. However, mature outcomes literature does not yet exist. In this study, we report our institution’s interim results on the efficacy and safety of 5-fraction radiosurgery alone for radiographically diagnosed meningiomas. MATERIALS AND METHODS Between 2005-2015 all patients who completed treatment on a single institution protocol utilizing 5-fraction robotic radiosurgery alone for the treatment of progressing radiographically diagnosed meningiomas were eligible for inclusion. Local control was calculated using the Kaplan-Meier Method. RESULTS Forty-four consecutive predominately female patients (84%) ranging in age from 33-85 (median: 59) were included in the present study. Median tumor volume was 4.05mm3 (range: 0.94-15.4mm3) and the majority of tumors were located at the base of skull (66%). A median dose of 25Gy (range: 25Gy-35Gy), was delivered to a median isodose line of 82%, (range: 70%-90%) over a median of 7 days (range: 5-11 days). Acute toxicity was minimal with 7 patients (15%) requiring a short course of steroids for symptomatic edema during treatment. Of 16 patients who presented with a cranial nerve deficit, symptom improvement was noted in 11 patients (69%). No permanent treatment related toxicity was noted in our cohort. The median radiographic follow-up was 6.9 years (range: 0.5-14.8 years). The 5 and 8-year local control rates were 100% and 95%. The median time to local failure (n=2) in our cohort was 8.2 years. CONCLUSIONS The treatment of radiographically diagnosed meningiomas with 5-fraction robotic radiosurgery provides excellent local control to date, with low rates of acute and late toxicity. However, with late failures noted in our series, continued follow-up is needed to determine the optimal dose required for long-term tumor control.


2008 ◽  
Vol 1 (2) ◽  
pp. 126-130 ◽  
Author(s):  
Lilie L. Lin ◽  
Issam El Naqa ◽  
Jeffrey R. Leonard ◽  
Tae Sung Park ◽  
Abby S. Hollander ◽  
...  

Object The authors report the results of surgery alone or in combination with radiotherapy in the management of craniopharyngiomas in children. Methods The authors retrospectively reviewed the outcomes in 31 patients treated for craniopharyngiomas at the Department of Radiation Oncology at Washington University in St. Louis and the St. Louis Children's Hospital. The median age at diagnosis was 8.1 years (range 1.1–21 years). Fourteen patients underwent gross-total resection (GTR) with observation, and 6 patients underwent subtotal resection (STR) with observation. Ten patients underwent STR or cyst aspiration followed by external-beam radiotherapy, and 1 patient underwent cyst aspiration followed by intracystic 32P installation. Results The median follow-up for all surviving patients was 78.2 months. Overall survival and local control rates at 10 years were 96 and 58%, respectively. One patient died of the disease, and 12 patients had subsequent recurrences. Of those with recurrences, 6 patients had undergone initial STR with observation and 6 had been treated with GTR and observation. The median time to progression was 17.9 months in the patients who underwent limited resection, and 55 months for those who underwent GTR. There were no recurrences in the patients who received radiotherapy at the time of initial diagnosis. Conclusions Radiotherapy delivered as part of the initial management of craniopharyngiomas in children or at the time of recurrence provides effective local control.


2021 ◽  
pp. 1-9
Author(s):  
Saniya S. Godil ◽  
Umberto Tosi ◽  
Mina Gerges ◽  
Andrew L. A. Garton ◽  
Georgiana A. Dobri ◽  
...  

OBJECTIVE Surgical management of craniopharyngiomas (CPAs) is challenging. Controversy exists regarding the optimal goals of surgery. The purpose of this study was to compare the long-term outcomes of patients who underwent gross-total resection with the outcomes of those who underwent subtotal resection of their CPA via an endoscopic endonasal approach. METHODS From a prospectively maintained database of all endoscopic endonasal approaches performed at Weill Cornell Medicine, only patients with CPAs with > 3 years of follow-up after surgery were included. The primary endpoint was radiographic progression. Data were collected on baseline demographics, imaging, endocrine function, visual function, and extent of resection. RESULTS A total of 44 patients with a mean follow-up of 5.7 ± 2.6 years were included. Of these patients, 14 (31.8%) had prior surgery. GTR was achieved in 77.3% (34/44) of all patients and 89.5% (34/38) of patients in whom it was the goal of surgery. Preoperative tumor volume < 10 cm3 was highly predictive of GTR (p < 0.001). Radiation therapy was administered within the first 3 months after surgery in 1 (2.9%) of 34 patients with GTR and 7 (70%) of 10 patients with STR (p < 0.001). The 5-year recurrence-free/progression-free survival rate was 75.0% after GTR and 25.0% after STR (45% in subgroup with STR plus radiotherapy; p < 0.001). The time to recurrence after GTR was 30.2 months versus 13 months after STR (5.8 months in subgroup with STR plus radiotherapy; p < 0.001). Patients with GTR had a lower rate of visual deterioration and higher rate of return to work or school compared with those with STR (p = 0.02). Patients with GTR compared to STR had a lower rate of CSF leakage (0.0% vs 30%, p = 0.001) but a higher rate of diabetes insipidus (85.3% vs 50%, p = 0.02). CONCLUSIONS GTR, which is possible to achieve in smaller tumors, resulted in improved tumor control, better visual outcome, and better functional recovery but a higher rate of diabetes insipidus compared with STR, even when the latter was supplemented with postoperative radiation therapy. GTR should be the goal of craniopharyngioma surgery, when achievable with minimal morbidity.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4510-4510
Author(s):  
Meredith Jackson ◽  
Sean All ◽  
Samantha Brocklehurst ◽  
Neil B Desai ◽  
Jennifer L Shah ◽  
...  

Abstract Introduction For indolent non-Hodgkin lymphoma (NHL), there is level 1 evidence (FoRT) demonstrating superior long term local control with 24 Gy radiation treatment (RT) over ultra-low-dose 4 Gy RT (Hoskin et al Lancet 2021). However, it's notable that over 2/3 of those receiving ultra-low-dose RT did achieve durable local control. Further, with RT alone to any dose, the predominant pattern of failure is distal. Thus, the TROG study showed that the addition of systemic therapy (rituximab, cyclophosphamide, vincristine) after RT (30 Gy) significantly improved progression free survival (PFS). However, cytotoxic chemotherapy and higher dose RT can both cause significant, and potentially unnecessary, toxicities. We hypothesize that for indolent NHL, the combination of single-agent rituximab and ultra-low-dose adaptive RT, with repeat treatment as needed, will result in excellent local and systemic disease control with minimal toxicities. Methods We conducted an IRB approved, retrospective review of patients with indolent NHL who were treated with both ultra-low-dose RT (2 Gy x 2 or "boom boom") and single-agent rituximab (4 cycles) either concurrently or within a short interval (median 13 days) at our institution from 2017-2020. 17 treatments [follicular (9), marginal zone (4), mucosal associated lymphoid tissue (3), other (1)] from 15 patients were identified. Treatment sites included pelvis (5), parotid (4), abdomen (4), mediastinum (1), and other (3). The median ECOG performance status was 1 (range: 0-2), the median age was 74 (range: 25-90), and 9/15 patients were male. 7 patients were stage I, 5 stage II, 2 stage III, and 1 stage IV. 3 patients had prior RT with 1 patient having the same spot irradiated twice, and the other 2 both having RT at 2 distal sites. Only 1 patient had prior systemic treatment (ibrutinib) for their low-grade lymphoma. The primary outcomes were rates of complete response (CR), partial response (PR), overall response (defined as CR or PR), stable disease (SD), or progressive disease (PD). Secondary outcomes included PFS, overall survival (OS), symptom relief, and acute and long-term toxicities. Radiographic studies (predominantly PET/CT) were used to determine treatment response and disease control. Results In our cohort with median follow up of 16 months, the PFS and OS at one year was 93% (14/15)and 100% (15/15), respectively. The overall response rate was 94% (16/17), of which 13 sites (76%) achieved CR, 3 (18%) had PR, and 1 (6%) had SD. Of those with PR, 1 had residual disease in the field of RT, 1 outside the field of RT, and 1 both in and out of the field of RT. For the first 2, repeat ultra-low-dose RT was given to sites of PR and both achieved CR. The remaining patient with both in and out of field PR was managed with active surveillance with SD on last follow up. 2 patients experienced acute toxicities, 1 with mild diarrhea from pre-sacral RT that resolved within days, and 1 with dysgeusia from parotid RT that resolved within 2 months. Only 1 patient noted long-term toxicity of dry mouth with about 50% reduction in saliva after left parotid gland RT, but of note this patient also had preexisting Sjogren's syndrome. Symptoms were present in 10 patients, of which 9 noted improvement after treatment. The only patient whose symptoms did not improve was the patient with SD. This patient had neuro involvement of the lymphoma with multiple confounding factors. Conclusion Combined modality treatment with single-agent rituximab and ultra-low-dose adaptive RT, with retreatment as needed, in patients with indolent NHL appears to provide effective palliation and disease control with minimal toxicity. For patients with concerns for radiation or chemotherapy related toxicity, this presents an attractive alternate treatment paradigm that warrants further evaluation in larger prospective studies. Disclosures Desai: Boston Scientific: Consultancy, Research Funding. Awan: ADCT therapeutics: Consultancy; Cardinal Health: Consultancy; Merck: Consultancy; BMS: Consultancy; Dava Oncology: Consultancy; Johnson and Johnson: Consultancy; Beigene: Consultancy; Incyte: Consultancy; Verastem: Consultancy; MEI Pharma: Consultancy; Karyopharm: Consultancy; Celgene: Consultancy; Kite pharma: Consultancy; Gilead sciences: Consultancy; Pharmacyclics: Consultancy; Janssen: Consultancy; Abbvie: Consultancy; Astrazeneca: Consultancy; Genentech: Consultancy.


2005 ◽  
Vol 4 (6) ◽  
pp. 675-682 ◽  
Author(s):  
Ratna Sajja ◽  
Gene H. Barnett ◽  
Shih-Yuan Lee ◽  
Gayle Harnisch ◽  
Glen H. J. Stevens ◽  
...  

The purpose of this study was to evaluate tumor control, complications, and outcome from intensity-modulated radiation therapy (IMRT) for intracranial meningiomas. Between July 1997 and November 2003, patients with intracranial meningiomas were treated at our institution with the NOMOS Peacock system utilizing the Multileaf Intensity Modulating Collimator (MIMiC). Thirty-five patients with 37 lesions (35 benign and two atypical histology) were identified with a minimum of six months of radiologic follow-up for this retrospective review. The median age of the patients was 65 years with a median KPS of 90 prior to treatment with IMRT. The median MRI/CT follow-up for the 37 treated lesions was 19.1 months (range 6.4–62.4 months). Twenty meningiomas (54%) were previously treated with surgery/radiosurgery prior to IMRT, and 17 meningiomas (46%) were treated with IMRT primarily after diagnosis was established by MRI/CT. The median time from previous surgery to treatment with IMRT was 18.1 months. The median tumor dose was 50.4 Gy prescribed to the 87% isodose line providing a median target coverage of 95%. Local control was at 97% three years after treatment with IMRT. Only three patients exhibited local failure after treatment. Although local control was slightly better in the upfront-IMRT lesions as compared to the lesions treated with prior surgery/radiosurgery (100% vs 95%), this difference was not statistically significant. On univariate analysis, the IMRT prescription dose and maximum dose were found to be predictors for local control (p=0.05). On multivariate analysis, these factors did not remain significant for influencing local control. No long-term complications from IMRT were documented among the 35 patients. In conclusion, intensity-modulated radiation therapy is a safe and effective treatment for some intracranial meningiomas. A greater number of patients with longer follow-up after treatment may be needed to determine treatment variables predicting for long-term tumor control.


Skull Base ◽  
2007 ◽  
Vol 16 (S 1) ◽  
Author(s):  
Fred Barker ◽  
Steven Kalkanis ◽  
Michael McKenna ◽  
Dennis Poe ◽  
Joseph Nadol ◽  
...  

2020 ◽  
Vol 26 (1) ◽  
pp. 22-26 ◽  
Author(s):  
Tryggve Lundar ◽  
Bernt Johan Due-Tønnessen ◽  
Radek Frič ◽  
Petter Brandal ◽  
Paulina Due-Tønnessen

OBJECTIVEEpendymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.METHODSThe authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.RESULTSThe authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.CONCLUSIONSPediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.


2011 ◽  
Vol 114 (5) ◽  
pp. 1241-1249 ◽  
Author(s):  
Soichi Oya ◽  
Burak Sade ◽  
Joung H. Lee

Object The aim of this study was to describe the surgical technique used for removal of sphenoorbital meningiomas in the authors' practice and to review the operative outcome. Methods Review of the senior author's practice between 1994 and 2009 revealed 39 patients (mean age 48 years) eligible for this study. Clinical presentation, surgical technique, postoperative outcome, and follow-up data are presented. Surgical technique is detailed, with an emphasis on aggressive removal consisting of drilling of the hypertrophied sphenoid bone, orbital wall, and anterior clinoid process, followed by tumor removal and a wide resection of the involved dura. A simple dural closure without reconstruction of the orbital roof or the lateral wall of the orbit is also described. Results Gross-total resection was achieved in 15 cases (38.5%), near-total resection with small residual in the cavernous sinus or periorbita in 20 cases (51.3%), and subtotal resection in 4 cases (10.3%). Postoperative complications included trigeminal hypesthesia in 9 patients, oculomotor palsy in 3 patients, and seizure in 2 patients. Seven patients had recurrence within the mean follow-up period of 40.7 months. Preoperative visual deficits were present in 21 patients (53.8%). Of these, 14 (66.7%) experienced visual recovery to normal levels postoperatively. Statistical analyses revealed preoperative severe visual deficit and sphenoid bone hypertrophy as an independent risk factor and an independent favorable factor, respectively, for a favorable visual outcome. Proptosis was resolved (≤ 2 mm) in 73.5% of the authors' patients. No patient had postoperative enophthalmos. Conclusions In the authors' practice, surgery for sphenoorbital meningiomas consists of resection of the orbital/sphenoid intraosseous, intraorbital, and intradural tumor components. The authors believe that aggressive removal of the orbital/sphenoid intraosseous tumor is critical for a favorable visual outcome and tumor control. Furthermore, satisfactory cosmetic results can be achieved with simple reconstruction techniques as described.


2018 ◽  
Vol 79 (S 04) ◽  
pp. S362-S370 ◽  
Author(s):  
Constantin Tuleasca ◽  
Alda Rocca ◽  
Mercy George ◽  
Etienne Pralong ◽  
Luis Schiappacasse ◽  
...  

Objective Planned subtotal resection followed by Gamma Knife surgery (GKS) in patients with large vestibular schwannoma (VS) has emerged during the past decade, with the aim of a better functional outcome for facial and cochlear function. Methods We prospectively collected patient data, surgical, and dosimetric parameters of a consecutive series of patients treated by this method at Lausanne University Hospital during the past 8 years. Results A consecutive series of 47 patients were treated between July 2010 and January 2018. The mean follow-up after surgery was 37.5 months (median: 36, range: 0.5–96). Mean presurgical tumor volume was 11.8 mL (1.47–34.9). Postoperative status showed normal facial nerve function (House–Brackmann I) in all patients. In a subgroup of 28 patients, with serviceable hearing before surgery and in which cochlear nerve preservation was attempted at surgery, 26 (92.8%) retained serviceable hearing. Nineteen had good or excellent hearing (Gardner–Robertson class 1) before surgery, and 16 (84.2%) retained it after surgery. Mean duration between surgery and GKS was 6 months (median: 5, range: 3–13.9). Mean residual volume as compared with the preoperative one at GKS was 31%. Mean marginal dose was 12 Gy (11–12). Mean follow-up after GKS was 34.4 months (6–84). Conclusion Our data show excellent results in large VS management with a combined approach of microsurgical subtotal resection and GKS on the residual tumor, with regard to the functional outcome and tumor control. Longer term follow-up is necessary to fully evaluate this approach, especially regarding tumor control.


Cancers ◽  
2021 ◽  
Vol 13 (13) ◽  
pp. 3244
Author(s):  
Jenny Pettersson-Segerlind ◽  
Alexander Fletcher-Sandersjöö ◽  
Charles Tatter ◽  
Gustav Burström ◽  
Oscar Persson ◽  
...  

Spinal meningiomas are the most common adult primary spinal tumor, constituting 24–45% of spinal intradural tumors and 2% of all meningiomas. The aim of this study was to assess postoperative complications, long-term outcomes, predictors of functional improvement and differences between elderly (≥70 years) and non-elderly (18–69 years) patients surgically treated for spinal meningiomas. Variables were retrospectively collected from patient charts and magnetic resonance images. Baseline comparisons, paired testing and regression analyses were used. In conclusion, 129 patients were included, with a median follow-up time of 8.2 years. Motor deficit was the most common presenting symptom (66%). The median time between diagnosis and surgery was 1.3 months. A postoperative complication occurred in 10 (7.8%) and tumor growth or recurrence in 6 (4.7%) patients. Surgery was associated with significant improvement of motor and sensory deficit, gait disturbance, bladder dysfunction and pain. Time to surgery, tumor area and the degree of spinal cord compression significantly predicted postoperative improvement in a modified McCormick scale (mMCs) in the univariable regression analysis, and spinal cord compression showed independent risk association in multivariable analysis. There was no difference in improvement, complications or tumor control between elderly and non-elderly patients. We concluded that surgery of spinal meningiomas was associated with significant long-term neurological improvement, which could be predicted by time to surgery, tumor size and spinal cord compression.


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