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Long-term radiographic and endocrinological outcomes of stereotactic radiosurgery for recurrent or residual nonfunctioning pituitary adenomas

Ahmed Shaaban, Chloé Dumot, Georgios Mantziaris, Sam Dayawansa, Selcuk Peker, Yavuz Samanci, Ahmed M. Nabeel, Wael A. Reda, Sameh R. Tawadros, Khaled Abdel Karim, Amr M. N. El-Shehaby, Reem M. Emad Eldin, Ahmed Ragab Abdelsalam, Roman Liscak, Jaromir May, Elad Mashiach, Fernando De Nigris Vasconcellos, Kenneth Bernstein, Douglas Kondziolka, Herwin Speckter, Ruben Mota, Anderson Brito, Shray K. Bindal, Ajay Niranjan, L. Dade Lunsford, Carolina Gesteira Benjamin, Timoteo Almeida, Jennifer Z. Mao, David Mathieu, Jean-Nicolas Tourigny, Manjul Tripathi, Joshua David Palmer, Jennifer Matsui, Joseph Crooks, Rodney E. Wegner, Matthew J. Shepard, and Jason P. Sheehan

OBJECTIVE

Stereotactic radiosurgery (SRS) is used for the treatment of residual/recurrent nonfunctional pituitary adenoma (NFPA). The aim of this study was to evaluate the factors related to long-term tumor control and delayed endocrinopathies following SRS.

METHODS

This retrospective, multicenter study included patients with recurrent/residual NFPA treated with single-fraction SRS; they were then divided into two arms. The first arm included patients with at least 5 years of radiographic follow-up and all patients with local tumor progression. The second arm included patients with at least 5 years of endocrinological follow-up and all patients who developed endocrinopathy. Study endpoints were tumor control and new or worsening hypopituitarism after SRS and were analyzed using Cox regression and Kaplan-Meier methodology.

RESULTS

There were 360 patients in the tumor control arm (median age 52.7 [IQR 42.9–61] years, 193 [53.6%] males) and 351 patients in the hypopituitarism arm (median age 52.5 [IQR 43–61] years, 186 [53.0%] males). The median follow-up in the tumor control evaluation group was 7.95 (IQR 5.7–10.5) years. Tumor control rates at 5, 8, 10, and 15 years were 93% (95% CI 90%–95%), 87% (95% CI 83%–91%), 86% (95% CI 82%–90%), and 69% (95% CI 59%–81%), respectively. The median follow-up in the endocrinopathy evaluation group was 8 (IQR 5.9–10.7) years. Pituitary function preservation rates at 5, 8, 10, and 15 years were 83% (95% CI 80%–87%), 81% (95% CI 77%–85%), 78% (95% CI 74%–83%), and 71% (95% CI 63%–79%), respectively. A margin dose > 15 Gy (HR 0.8, 95% CI 0.7–0.9; p < 0.001) and a delay from last resection to SRS > 1 year (HR 0.9, 95% CI 0.7–0.9; p = 0.04) were significant factors related to tumor control in multivariable analysis. A maximum dose to the pituitary stalk ≤ 10 Gy (HR 1.1, 95% CI 1.09–1.2; p < 0.001) was associated with pituitary function preservation. New visual deficits after SRS occurred in 7 (1.94%) patients in the tumor control group and 8 (2.3%) patients in the endocrinopathy group. Other new cranial nerve deficits post-SRS occurred in 4 of 160 patients with data in the tumor control group and 3 of 140 patients with data in the endocrinopathy group.

CONCLUSIONS

SRS affords favorable and durable tumor control for the vast majority of NFPAs. Post-SRS hypopituitarism occurs in a minority of patients, but this risk increases with time and warrants long-term follow-up.

Open access

The relevance of biologically effective dose for pain relief and sensory dysfunction after Gamma Knife radiosurgery for trigeminal neuralgia: an 871-patient multicenter study

Ronald E. Warnick, Ian Paddick, David Mathieu, Elizabeth Adam, Christian Iorio-Morin, William Leduc, Andréanne Hamel, Sarah E. Johnson, Mohamad Bydon, Ajay Niranjan, L. Dade Lunsford, Zhishuo Wei, Kaitlin Waite, Shalini Jose, Selcuk Peker, Mustafa Yavuz Samanci, Ece Tek, Georgios Mantziaris, Stylianos Pikis, Jason P. Sheehan, Manjul Tripathi, Narendra Kumar, Juan Diego Alzate, Kenneth Bernstein, Peter Ahorukomeye, Varun R. Kshettry, Herwin Speckter, Wenceslao Hernandez, Dušan Urgošík, Roman Liščák, Andrew I. Yang, John Y. K. Lee, Samir Patel, Dorian M. Kusyk, Matthew J. Shepard, and Douglas Kondziolka

OBJECTIVE

Recent studies have suggested that biologically effective dose (BED) is an important correlate of pain relief and sensory dysfunction after Gamma Knife radiosurgery (GKRS) for trigeminal neuralgia (TN). The goal of this study was to determine if BED is superior to prescription dose in predicting outcomes in TN patients undergoing GKRS as a first procedure.

METHODS

This was a retrospective study of 871 patients with type 1 TN from 13 GKRS centers. Patient demographics, pain characteristics, treatment parameters, and outcomes were reviewed. BED was compared with prescription dose and other dosimetric factors for their predictive value.

RESULTS

The median age of the patients was 68 years, and 60% were female. Nearly 70% of patients experienced pain in the V2 and/or V3 dermatomes, predominantly on the right side (60%). Most patients had modified BNI Pain Intensity Scale grade IV or V pain (89.2%) and were taking 1 or 2 pain medications (74.1%). The median prescription dose was 80 Gy (range 62.5–95 Gy). The proximal trigeminal nerve was targeted in 77.9% of cases, and the median follow-up was 21 months (range 6–156 months). Initial pain relief (modified BNI Pain Intensity Scale grades I–IIIa) was noted in 81.8% of evaluable patients at a median of 30 days. Of 709 patients who achieved initial pain relief, 42.3% experienced at least one pain recurrence after GKRS at a median of 44 months, with 49.0% of these patients undergoing a second procedure. New-onset facial numbness occurred in 25.3% of patients after a median of 8 months. Age ≥ 63 years was associated with a higher probability of both initial pain relief and maintaining pain relief. A distal target location was associated with a higher probability of initial and long-term pain relief, but also a higher incidence of sensory dysfunction. BED ≥ 2100 Gy2.47 was predictive of pain relief at 30 days and 1 year for the distal target, whereas physical dose ≥ 85 Gy was significant for the proximal target, but the restricted range of BED values in this subgroup could be a confounding factor. A maximum brainstem point dose ≥ 29.5 Gy was associated with a higher probability of bothersome facial numbness.

CONCLUSIONS

BED and physical dose were both predictive of pain relief and could be used as treatment planning goals for distal and proximal targets, respectively, while considering maximum brainstem point dose < 29.5 Gy as a potential constraint for bothersome numbness.

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Repeat stereotactic radiosurgery for persistent cerebral arteriovenous malformations in pediatric patients

Gracie Garcia, Georgios Mantziaris, Stylianos Pikis, Chloe Dumot, L. Dade Lunsford, Ajay Niranjan, Zhishuo Wei, Priyanka Srinivasan, Lilly W. Tang, Roman Liscak, Jaromir May, Cheng-Chia Lee, Huai-Che Yang, Selcuk Peker, Yavuz Samanci, Ahmed M. Nabeel, Wael A. Reda, Sameh R. Tawadros, Khaled Abdel Karim, Amr M. N. El-Shehaby, Reem Emad Eldin, Ahmed Hesham Elazzazi, Nuria Martínez Moreno, Roberto Martínez Álvarez, Varun Padmanaban, Francis J. Jareczek, James McInerney, Kevin M. Cockroft, Juan Diego Alzate, Douglas Kondziolka, Manjul Tripathi, and Jason P. Sheehan

OBJECTIVE

The purpose of this study was to describe the long-term outcomes and associated risks related to repeat stereotactic radiosurgery (SRS) for persistent arteriovenous malformations (AVMs) in pediatric patients.

METHODS

Under the auspices of the International Radiosurgery Research Foundation, this retrospective multicenter study analyzed pediatric patients who underwent repeat, single-session SRS between 1987 and 2022. The primary outcome variable was a favorable outcome, defined as nidus obliteration without hemorrhage or neurological deterioration. Secondary outcomes included rates and probabilities of hemorrhage, radiation-induced changes (RICs), and cyst or tumor formation.

RESULTS

The cohort included 83 pediatric patients. The median patient age was 11 years at initial SRS and 15 years at repeat SRS. Fifty-seven children (68.7%) were managed exclusively using SRS, and 42 (50.6%) experienced hemorrhage prior to SRS. Median AVM diameter and volume were substantially different between the first (25 mm and 4.5 cm3, respectively) and second (16.5 mm and 1.6 cm3, respectively) SRS, while prescription dose and isodose line remained similar. At the 5-year follow-up evaluation from the second SRS, nidus obliteration was achieved in 42 patients (50.6%), with favorable outcome in 37 (44.6%). The median time to nidus obliteration and hemorrhage was 35.5 and 38.5 months, respectively. The yearly cumulative probability of favorable outcome increased from 2.5% (95% CI 0.5%–7.8%) at 1 year to 44% (95% CI 32%–55%) at 5 years. The probability of achieving obliteration followed a similar pattern and reached 51% (95% CI 38%–62%) at 5 years. The 5-year risk of hemorrhage during the latency period after the second SRS reached 8% (95% CI 3.2%–16%). Radiographically, 25 children (30.1%) had RICs, but only 5 (6%) were symptomatic. Delayed cyst formation occurred in 7.2% of patients, with a median onset of 47 months. No radiation-induced neoplasia was observed.

CONCLUSIONS

The study results showed nidus obliteration in most pediatric patients who underwent repeat SRS for persistent AVMs. The risks of symptomatic RICs and latency period hemorrhage were quite low. These findings suggest that repeat radiosurgery should be considered when treating pediatric patients with residual AVM after prior SRS. Further study is needed to define the role of repeat SRS more fully in this population.

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Effect of cerebral arteriovenous malformation location on outcomes of repeat, single-fraction stereotactic radiosurgery: a matched-cohort analysis

Georgios Mantziaris, Stylianos Pikis, Chloe Dumot, Sam Dayawansa, Roman Liscak, Jaromir May, Cheng-Chia Lee, Huai-Che Yang, Nuria Martínez Moreno, Roberto Martinez Álvarez, L. Dade Lunsford, Ajay Niranjan, Zhishuo Wei, Priyanka Srinivasan, Lilly W. Tang, Ahmed M. Nabeel, Wael A. Reda, Sameh R. Tawadros, Khaled Abdel Karim, Amr M. N. El-Shehaby, Reem M. Emad Eldin, Ahmed Hesham Elazzazi, Selcuk Peker, Yavuz Samanci, Varun Padmanaban, Francis J. Jareczek, James McInerney, Kevin M. Cockroft, David Mathieu, Salman Aldakhil, Juan Diego Alzate, Douglas Kondziolka, Manjul Tripathi, Joshua D. Palmer, Rituraj Upadhyay, Michelle Lin, Gabriel Zada, Cheng Yu, Christopher P. Cifarelli, Daniel T. Cifarelli, Ahmed Shaaban, Zhiyuan Xu, and Jason P. Sheehan

OBJECTIVE

Patients with deep-seated arteriovenous malformations (AVMs) have a higher rate of unfavorable outcome and lower rate of nidus obliteration after primary stereotactic radiosurgery (SRS). The aim of this study was to evaluate and quantify the effect of AVM location on repeat SRS outcomes.

METHODS

This retrospective, multicenter study involved 505 AVM patients managed with repeat, single-session SRS. The endpoints were nidus obliteration, hemorrhage in the latency period, radiation-induced changes (RICs), and favorable outcome. Patients were split on the basis of AVM location into the deep (brainstem, basal ganglia, thalamus, deep cerebellum, and corpus callosum) and superficial cohorts. The cohorts were matched 1:1 on the basis of the covariate balancing score for volume, eloquence of location, and prescription dose.

RESULTS

After matching, 149 patients remained in each cohort. The 5-year cumulative probability rates for favorable outcome (probability difference −18%, 95% CI −30.9 to −5.8%, p = 0.004) and AVM obliteration (probability difference –18%, 95% CI –30.1% to −6.4%, p = 0.007) were significantly lower in the deep AVM cohort. No significant differences were observed in the 5-year cumulative probability rates for hemorrhage (probability difference 3%, 95% CI –2.4% to 8.5%, p = 0.28) or RICs (probability difference 1%, 95% CI –10.6% to 11.7%, p = 0.92). The median time to delayed cyst formation was longer with deep-seated AVMs (deep 62 months vs superficial 12 months, p = 0.047).

CONCLUSIONS

AVMs located in deep regions had significantly lower favorable outcomes and obliteration rates compared with superficial lesions after repeat SRS. Although the rates of hemorrhage in the latency period and RICs in the two cohorts were comparable, delayed cyst formation occurred later in patients with deep-seated AVMs.

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Local control and survival after stereotactic radiosurgery for colorectal cancer brain metastases: an international multicenter analysis

Othman Bin-Alamer, Hussam Abou-Al-Shaar, Raj Singh, Arka N. Mallela, Andrew Legarreta, Greg Bowden, David Mathieu, Haley K. Perlow, Joshua D. Palmer, Shahed Elhamdani, Matthew Shepard, Yun Liang, Ahmed M. Nabeel, Wael A. Reda, Sameh R. Tawadros, Khaled Abdelkarim, Amr M. N. El-Shehaby, Reem Emad Eldin, Ahmed Hesham Elazzazi, Ronald E. Warnick, Yair M. Gozal, Megan Daly, Brendan McShane, Marcel Addis-Jackson, Gokul Karthikeyan, Sian Smith, Piero Picozzi, Andrea Franzini, Tehila Kaisman-Elbaz, Huai-che Yang, Judith Hess, Kelsey Templeton, Xiaoran Zhang, Zhishuo Wei, Stylianos Pikis, Georgios Mantziaris, Gabriela Simonova, Roman Liscak, Selcuk Peker, Yavuz Samanci, Veronica Chiang, Charles R. Kersh, Cheng-Chia Lee, Daniel M. Trifiletti, Ajay Niranjan, Constantinos G. Hadjipanayis, L. Dade Lunsford, and Jason P. Sheehan

OBJECTIVE

The goal of this study was to characterize local tumor control (LC), overall survival (OS), and safety of stereotactic radiosurgery for colorectal brain metastasis (CRBM).

METHODS

Ten international institutions participating in the International Radiosurgery Research Foundation provided data for this retrospective case series. This study included 187 patients with CRBM (281 tumors), with a median age of 62 years and 56.7% being male. Most patients (53.5%) had solitary tumors, although 10.7% had > 5 tumors. The median tumor volume was 2.7 cm3 (IQR 0.22–8.1 cm3), and the median margin dose was 20 Gy (IQR 18–22 Gy).

RESULTS

The 3-year LC and OS rates were 72% and 20%, respectively. Symptomatic adverse radiation effects occurred in 1.6% of patients. In the multivariate analysis, age > 65 years and tumor volume > 4.0 cm3 were significant predictors of tumor progression (hazard ratio [HR] 2.6, 95% CI 1.4–4.9; p = 0.003 and HR 3.4, 95% CI 1.7–6.9; p < 0.001, respectively). Better performance status (Karnofsky Performance Scale score > 80) was associated with a reduced risk of tumor progression (HR 0.38, 95% CI 0.19–0.73; p = 0.004). Patient age > 62 years (HR 1.6, 95% CI 1.1–2.3; p = 0.03) and the presence of active extracranial disease (HR 1.7, 95% CI 1.1–2.4; p = 0.009) were significantly associated with worse OS.

CONCLUSIONS

Stereotactic radiosurgery offers a high LC rate and a low rate of symptomatic adverse radiation effects for the majority of CRBMs. The OS and LC favored younger patients with high functional performance scores and inactive extracranial disease.

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Long-term outcomes of deep pediatric arteriovenous malformations

Prateek Agarwal, Nikhil Sharma, Gautam Nayar, Rachel C. Jacobs, Alhamza Al-Bayati, L. Dade Lunsford, Michael M. McDowell, and Stephanie Greene

OBJECTIVE

Multimodality treatment has been shown to be the optimal management strategy for pediatric arteriovenous malformations (AVMs). Deep AVMs represent a subset of AVMs for which optimal management may be achieved with a combination of radiosurgery and highly selective embolization, in the absence of compelling features requiring operative intervention. The objective of this study was to identify predictors of good functional outcomes in pediatric patients with deep AVMs.

METHODS

A retrospective cohort study of the outcomes of 79 patients with deep AVMs from January 1988 through December 2021 was performed. Deep AVMs were defined as those with the majority of the nidus centered in the basal ganglia, thalamus, or brainstem. Collected data included patient demographics and presenting symptoms, presenting modified Rankin Scale (mRS) score, radiographic findings and outcomes, management strategy, complications, and clinical outcomes as indicated by follow-up mRS score. A good outcome was defined as a follow-up mRS score ≤ 2, while a poor outcome was defined as a follow-up mRS score ≥ 3. Statistical analysis was performed to identify factors associated with functional outcomes.

RESULTS

With a mean follow-up duration of 85.6 months, there was a 72.2% angiographic obliteration rate, with 75.9% of patients having a good clinical outcome (mRS score ≤ 2). Presenting symptoms and radiographic characteristics were not significantly associated with long-term functional outcomes. There was a significantly higher rate of posttreatment hemorrhage in patients with a poor versus good outcome (11.8% vs 0%, p = 0.010). On multivariate logistic regression analysis, poor long-term functional outcome was only associated with poor presenting mRS score (p = 0.002).

CONCLUSIONS

Satisfactory angiographic obliteration rates and good long-term functional outcomes can be achieved for deep AVMs, with stereotactic radiosurgery as the cornerstone of multimodality treatment.

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Stereotactic radiosurgery and local control of brain metastases from triple-negative breast cancer

Roman O. Kowalchuk, Ajay Niranjan, Judith Hess, Joseph P. Antonios, Michael Y. Zhang, Steve Braunstein, Richard B. Ross, Stylianos Pikis, Christopher P. Deibert, Cheng-chia Lee, Huai-che Yang, Anne-Marie Langlois, David Mathieu, Selcuk Peker, Yavuz Samanci, Chad G. Rusthoven, Veronica Chiang, Zhishuo Wei, L. Dade Lunsford, Daniel M. Trifiletti, and Jason P. Sheehan

OBJECTIVE

Stereotactic radiosurgery (SRS) is an effective treatment for intracranial metastatic disease, but its role in triple-negative breast cancer requires further study. Herein, the authors report overall survival (OS) and local tumor control in a multiinstitutional cohort with triple-negative breast cancer metastases treated with SRS.

METHODS

Patients treated from 2010 to 2019 at 9 institutions were included in this retrospective study if they had biopsy-proven triple-negative breast cancer with intracranial metastatic lesions treated with SRS. Patients were excluded if they had undergone prior SRS, whole-brain radiation therapy, or resection of the metastatic lesions. A retrospective chart review was conducted to determine OS, local control, and treatment efficacy.

RESULTS

Sixty-eight patients with 315 treated lesions were assessed. Patients had a median Karnofsky Performance Status of 80 (IQR 70–90) and age of 57 years (IQR 48–67 years). Most treated patients had 5 or fewer intracranial lesions, with 34% of patients having a single lesion. Treated lesions were small, having a median volume owf 0.11 cm3 (IQR 0.03–0.60 cm3). Patients were treated with a median margin dose of 18 Gy (IQR 18–20 Gy) to the median 71% isodose line (IQR 50%–84%). Overall, patients had a 1-year OS of 43% and 2-year OS of 20%. Most patients (88%) were followed until death, by which time local tumor progression had occurred in only 7% of cases. Furthermore, 76% of the lesions demonstrated regression. Tumor volume was correlated with local tumor progression (p = 0.012). SRS was very well tolerated, and only 3 patients (5%) developed symptomatic radiation necrosis.

CONCLUSIONS

SRS is a safe and efficacious treatment for well-selected patients with triple-negative breast cancer, especially for those with a favorable performance status and small- to moderate-volume metastatic lesions.

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Adjuvant versus on-progression Gamma Knife radiosurgery for residual nonfunctioning pituitary adenomas: a matched-cohort analysis

Georgios Mantziaris, Stylianos Pikis, Tomas Chytka, Roman Liščák, Kimball Sheehan, Darrah Sheehan, Selcuk Peker, Yavuz Samanci, Shray K. Bindal, Ajay Niranjan, L. Dade Lunsford, Rupinder Kaur, Renu Madan, Manjul Tripathi, Dhiraj J. Pangal, Ben A. Strickland, Gabriel Zada, Anne-Marie Langlois, David Mathieu, Ronald E. Warnick, Samir Patel, Zayda Minier, Herwin Speckter, Zhiyuan Xu, Rithika Kormath Anand, and Jason P. Sheehan

OBJECTIVE

Radiological progression occurs in 50%–60% of residual nonfunctioning pituitary adenomas (NFPAs). Stereotactic radiosurgery (SRS) is a safe and effective management option for residual NFPAs, but there is no consensus on its optimal timing. This study aims to define the optimal timing of SRS for residual NFPAs.

METHODS

This retrospective, multicenter study involved 375 patients with residual NFPAs managed with SRS. The patients were divided into adjuvant (ADJ; treated for stable residual NFPA within 6 months of resection) and progression (PRG) cohorts (treated for residual NFPA progression). Factors associated with tumor progression and clinical deterioration were analyzed.

RESULTS

Following propensity-score matching, each cohort consisted of 130 patients. At last follow-up, tumor control was achieved in 93.1% of patients in the ADJ cohort and in 96.2% of patients in the PRG cohort (HR 1.6, 95% CI 0.55–4.9, p = 0.37). Hypopituitarism was associated with a maximum point dose of > 8 Gy to the pituitary stalk (HR 4.5, 95% CI 1.6–12.6, p = 0.004). No statistically significant difference was noted in crude new-onset hypopituitarism rates (risk difference [RD] = −0.8%, p > 0.99) or visual deficits (RD = −2.3%, p = 0.21) between the two cohorts at the last follow-up. The median time from resection to new hypopituitarism was longer in the PRG cohort (58.9 vs 29.7 months, p = 0.01).

CONCLUSIONS

SRS at residual NFPA progression does not appear to alter the probability of tumor control or hormonal/visual deficits compared with adjuvant SRS. Deferral of radiosurgical management to the time of radiological progression could significantly prolong the time to radiosurgically induced pituitary dysfunction. A lower maximum point dose (< 8 Gy) to the pituitary stalk portended a more favorable chance of preserving pituitary function after SRS.

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Adjuvant stereotactic radiosurgery with or without postoperative fractionated radiation therapy in adults with skull base chordomas: a systematic review

Othman Bin-Alamer, Arka N. Mallela, Paolo Palmisciano, Zachary C. Gersey, Turki Elarjani, Mohamed A. Labib, Georgios A. Zenonos, Amir R. Dehdashti, Jason P. Sheehan, William T. Couldwell, L. Dade Lunsford, and Hussam Abou-Al-Shaar

OBJECTIVE

The objective of this retrospective study was to compare the survival of patients with biopsy-proven skull base chordoma who had undergone stereotactic radiosurgery (SRS) with versus without prior fractionated radiation therapy (RT).

METHODS

Relevant articles from database inception to September 2021 were retrieved from the PubMed, Scopus, Web of Science, and Cochrane databases for a systematic review of treatment protocols. Studies were included if they 1) involved adult patients (age ≥ 18 years) with histologically and radiologically confirmed chordomas located within the clival skull base region and treated with SRS; 2) reported data on clinical features, SRS protocols, and outcomes; and 3) were written in the English language. Studies were excluded if they 1) were literature reviews, case reports, technical notes, abstracts, or autopsy reports; 2) did not clearly differentiate the data of patients with chordomas from the data of patients with different tumors or the data of patients with chordomas in locations other than the skull base; or 3) lacked histological confirmation or treatment and outcome data. Extracted data included the following: study author and publication year, patient age and sex, symptoms, cranial nerve involvement, invaded structures, lesion size, treatment modality, surgical details, histopathological type, RT modality, SRS parameters, complications, postradiosurgery outcomes, complications, and survival outcomes.

RESULTS

After the selection process, 15 articles describing 130 patients met the study eligibility criteria, including 94 patients who had undergone postresection SRS (NoRT group) and 36 who had undergone postresection fractionated RT and subsequent SRS (RT group). The NoRT and RT groups were comparable in age (51.3 vs 47.4 years, respectively), sex (57.1% vs 58.3% male), tumor volume (9.5 vs 11.2 cm3), SRS treatment parameters (maximum dose: 35.4 vs 42.2 Gy, marginal dose: 19.6 vs 20.6 Gy, treatment isodose line: 60.2% vs 65.2%), and SRS adverse effects (10.9% vs 17.6%). For the entire cohort, the 3-, 5-, and 10-year progression-free survival (PFS) rates were 23%, 9%, and 3%, respectively, and the overall survival (OS) rates were 94%, 82%, and 76%, respectively. In the NoRT group, SRS was adjuvant treatment after resection in 38 patients (40.4%), salvage treatment for recurrent tumor treated with resection alone in 10 (10.6%), and not specified in 46 (48.9%). In the RT group, SRS was boost treatment in 9 patients (25.0%), salvage treatment after recurrence in 22 (61.1%), and not specified in 5 (13.9%). There was no difference between the two groups in terms of median PFS (24.0 months [Q1 34.0, Q3 15.0] vs 23.8 months [34.0, 18.0], respectively; p = 0.8) or median OS (293.0 months [not reached, 137.4] vs not reached [not reached, 48.0], respectively; p = 0.36). The adverse radiation effect rates were comparable between the groups (10.9% vs 17.6%, respectively; p = 0.4).

CONCLUSIONS

The role of SRS in the management of skull base chordomas is still evolving. This systematic literature review of biopsy-proven chordoma revealed that tumor control and survival rates for SRS alone after chordoma surgery were not inferior to those encountered after SRS plus fractionated RT.

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Stereotactic radiosurgery as the initial management option for small-volume hypothalamic hamartomas with intractable epilepsy: a 35-year institutional experience and systematic review

Zhishuo Wei, Lena Vodovotz, Diego D. Luy, Hansen Deng, Ajay Niranjan, and L. Dade Lunsford

OBJECTIVE

Young patients with hypothalamic hamartomas (HHs) often present with intractable epilepsy. Currently there are no established management guidelines for HH. The authors retrospectively reviewed their single-institution experience to delineate the role of stereotactic radiosurgery (SRS).

METHODS

Seven patients with HHs (4 females; median age 13.7 years, range 2.5–25 years) with no prior resection underwent SRS between 1987 and 2022. The clinical history, epilepsy profile, radiographic findings, and neurological outcomes were characterized. HH topographical types were classified according to the Régis classification. Outcome measures included Engel seizure classification, HH response, and the need for additional surgical interventions.

RESULTS

All patients had Engel class IV epilepsy. A Leksell Gamma Knife was used to deliver a median margin dose of 18 Gy (range 16–20 Gy) to a median hamartoma volume of 0.37 cm3 (range 0.20–0.89 cm3). Seizure reduction was confirmed in 6 patients, and 2 patients had regression of their hamartoma. Two patients underwent resection and/or laser interstitial thermal therapy after SRS. At follow-up, 1 patient was seizure free, 4 patients achieved Engel class II, 1 patient had Engel class III, and 1 patient had Engel class IV seizure outcomes.

CONCLUSIONS

SRS as the initial management option for HH was associated with a low risk of adverse effects. In this institutional series reviewing small-volume HHs treated with SRS, no adverse radiation effect was detected, and the majority of patients experienced seizure reduction. SRS should be considered as the first-line treatment for seizure control in patients with small-volume HHs.