scholarly journals Germline BAP1 Mutation in a Family With Multi-Generational Meningioma With Rhabdoid Features: A Case Series and Literature Review

2021 ◽  
Vol 11 ◽  
Author(s):  
Rahul N. Prasad ◽  
Ulysses G. Gardner ◽  
Alexander Yaney ◽  
Daniel M. Prevedello ◽  
Daniel C. Koboldt ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
External Beam Radiation ◽  
Proliferation Index ◽  
Adjuvant Radiation ◽  
Lower Grade ◽  
Beam Radiation ◽  
Who Grade ◽  
Rhabdoid Cells ◽  
Rhabdoid Features ◽  
Germline Testing

Meningioma is the most common primary brain tumor, and recurrence risk increases with increasing WHO Grade from I to III. Rhabdoid meningiomas are a subset of WHO Grade III tumors with rhabdoid cells, a high proliferation index, and other malignant features that follow an aggressive clinical course. Some meningiomas with rhabdoid features either only focally or without other malignant features are classified as lower grade yet still recur early. Recently, inactivating mutations in the tumor suppressor gene BAP1 have been associated with poorer prognosis in rhabdoid meningioma and meningioma with rhabdoid features, and germline mutations have been linked to a hereditary tumor predisposition syndrome (TPDS) predisposing patients primarily to melanoma and mesothelioma. We present the first report of a familial BAP1 inactivating mutation identified after multiple generations of a family presented with meningiomas with rhabdoid features instead of with previously described BAP1 loss-associated malignancies. A 24-year-old female presented with a Grade II meningioma with rhabdoid and papillary features treated with subtotal resection, adjuvant external beam radiation therapy, and salvage gamma knife radiosurgery six years later. Around that time, her mother presented with a meningioma with rhabdoid and papillary features managed with resection and adjuvant radiation therapy. Germline testing was positive for a pathogenic BAP1 mutation in both patients. Sequencing of both tumors demonstrated biallelic BAP1 inactivation via the combination of germline BAP1 mutation and either loss of heterozygosity or somatic mutation. No additional mutations implicated in oncogenesis were noted from either patient’s germline or tumor sequencing, suggesting that the inactivation of BAP1 was responsible for pathogenesis. These cases demonstrate the importance of routine BAP1 tumor testing in meningioma with rhabdoid features regardless of grade, germline testing for patients with BAP1 inactivated tumors, and tailored cancer screening in this population.

scholarly journals RADT-42. ADJUVANT RADIATION THERAPY FOR SUBTOTALLY RESECTED CEREBELLAR LIPONEUROCYTOMA

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii190-ii190
Author(s):  
Shearwood McClelland ◽  
Ulysses Gardner ◽  
Mitesh Shah ◽  
Gordon Watson ◽  
Kevin Shiue
Keyword(s):  
Radiation Therapy ◽  
Rare Disease ◽  
Residual Disease ◽  
Treatment Plan ◽  
External Beam Radiation ◽  
Adjuvant Radiation ◽  
Beam Radiation ◽  
Who Grade ◽  
Adjuvant Radiation Therapy ◽  
Cerebellar Liponeurocytoma

Abstract INTRODUCTION Classified as a benign glioneural tumor, cerebellar liponeurocytoma is a rare disease (fewer than 80 reported cases) and was upgraded from WHO grade I to grade II in 2007 due to its high recurrence rate. The authors report a case of definitive radiation therapy for recurrent subtotally resected cerebellar liponeurocytoma. METHODS An 80-year-old man having undergone seven resections for his left cerebellar liponeurocytoma without adjuvant therapy over the previous decade at outside institutions was referred for radiation therapy two months following his eighth resection, where gross total resection was limited by the lesion proximity to his brainstem resulting in a 2 cm residual left cerebellar lesion. Pathology revealed tumor cells strongly positive for synaptophysin and a Ki-67 labeling index < 1%. Due to the propensity of this disease to recur following resection, his entire resection cavity was treated with external beam radiation therapy (EBRT) to 46 Gy, followed by a 10 Gy boost to his residual disease yielding a total of 56 Gy to the residual disease. RESULTS Reimaging following the initial 46 Gy revealed the residual disease remained amenable (< 3 cm) to stereotactic radiosurgery (SRS), which was delivered via linear accelerator (10 Gy to the 80% isodose line) in a single fraction. Following EBRT + SRS, the patient responded well. At last follow-up, he has demonstrated no evidence of disease progression, brainstem-related morbidity or surgical incision-related morbidity. CONCLUSIONS The first reported case of SRS treatment of cerebellar liponeurocytoma as the culmination of a coordinated definitive plan beginning with EBRT supports the applicability and feasibility of this treatment strategy following subtotal resection. This case indicates that a radiation treatment plan similar to that for central neurocytoma may be an optimal strategy, and suggests that adjuvant radiation therapy following operative resection of this rare disease may be underutilized.

Adjuvant Radiation for Gynecologic Cancers

2019 ◽  
Author(s):  
Joanne Jang
Keyword(s):  
Cervical Cancer ◽  
Radiation Therapy ◽  
Endometrial Cancer ◽  
Vulvar Cancer ◽  
Vaginal Cancer ◽  
External Beam Radiation ◽  
Adjuvant Radiation ◽  
Gynecologic Cancers ◽  
Beam Radiation ◽  
Postoperative Setting

Radiation therapy plays a significant role in the treatment of nearly all gynecologic cancers, including endometrial cancer, cervical cancer, vaginal cancer, and vulvar cancer. Radiotherapy can be given as the primary modality for curative treatment of gynecologic cancers, most often for cervical, vaginal, and vulvar cancers, but can also be used adjuvantly in the postoperative setting. Radiation can be delivered in the form of external beam radiation therapy or as gynecologic implants for brachytherapy, which is radiation that is delivered internally. This review highlights the data supporting radiation therapy for gynecologic cancers and explains the different methods of radiation delivery. This review contains 5 figures, and 4 tables, and 40 references.  Key Words: adjuvant treatment, brachytherapy, cervical cancer, endometrial cancer, IMRT, ovarian cancer, radiation therapy, vaginal cancer, vulvar cancer

Radiation Therapy for Residual or Recurrent Atypical Meningioma

Neurosurgery ◽  
2015 ◽  
Vol 79 (1) ◽  
pp. 23-32 ◽  
Author(s):  
Sam Q. Sun ◽  
Chunyu Cai ◽  
Rory K.J. Murphy ◽  
Todd DeWees ◽  
Ralph G. Dacey ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Tumor Progression ◽  
Cox Regression ◽  
Atypical Meningioma ◽  
Locoregional Control ◽  
External Beam Radiation ◽  
Subtotal Resection ◽  
Adjuvant Radiation ◽  
Beam Radiation ◽  
Brain Invasion

Abstract BACKGROUND Optimal use of stereotactic radiosurgery (SRS) vs external beam radiation therapy (EBRT) for treatment of residual/recurrent atypical meningioma is unclear. OBJECTIVE: To analyze features associated with progression after radiation therapy. METHODS: Fifty radiation-naive patients who received SRS or EBRT for residual and/or recurrent atypical meningioma were examined for predictors of progression using Cox regression and Kaplan-Meier analyses. RESULTS: Thirty-two patients (64%) received adjuvant radiation after subtotal resection, 12 patients (24%) received salvage radiation after progression following subtotal resection, and 6 patients (12%) received salvage radiation after recurrence following gross total resection. Twenty-one patients (42%) received SRS (median 18 Gy), and 7 (33%) had tumor progression. Twenty-nine patients (58%) received EBRT (median 54 Gy), and 13 (45%) had tumor progression. Whereas tumor volume (P = .53), SRS vs EBRT (P = .45), and adjuvant vs salvage (P = .34) were not associated with progression after radiation therapy, spontaneous necrosis (hazard ratio [HR] = 82.3, P < .001), embolization necrosis (HR = 15.6, P = .03), and brain invasion (HR = 3.8, P = .008) predicted progression in univariate and multivariate analyses. Tumors treated with SRS/EBRT had 2- and 5-year actuarial locoregional control rates of 91%/88% and 71%/69%, respectively. Tumors with spontaneous necrosis, embolization necrosis, and no necrosis had 2- and 5-year locoregional control rates of 76%, 92%, and 100% and 36%, 73%, and 100%, respectively (P < .001). CONCLUSION: This study suggests that necrosis may be a negative predictor of radiation response regardless of radiation timing or modality.

scholarly journals Case-Based Review: meningioma

2016 ◽  
Vol 3 (2) ◽  
pp. 120-134 ◽  
Author(s):  
Shannon E. Fogh ◽  
Derek R. Johnson ◽  
Fred G. Barker ◽  
Priscilla K. Brastianos ◽  
Jennifer L. Clarke ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Extent Of Resection ◽  
Chemotherapeutic Agents ◽  
External Beam Radiation ◽  
Beam Radiation ◽  
Who Grade ◽  
Additional Chemotherapy ◽  
Proven Efficacy ◽  
Tremendous Amount ◽  
Primary Intracranial Tumor

AbstractMeningioma is by far the most common primary intracranial tumor in adults. Treatment of meningioma is complex due to a tremendous amount of variability in tumor behavior. Many patients are incidentally found to have tumors that will remain asymptomatic throughout their lives. It is important to identify these patients so that they can be spared from potentially morbid interventions. On the other end of the spectrum, high-grade meningiomas can behave very aggressively. When treatment is necessary, surgical resection is the cornerstone of meningioma therapy. Studies spanning decades have demonstrated that extent of resection correlates with prognosis. Radiation therapy, either in the form of external beam radiation therapy or stereotactic radiosurgery, represents another important therapeutic tool that can be used in place of or as a supplement to surgery. There are no chemotherapeutic agents of proven efficacy against meningioma, and chemotherapy treatment is generally reserved for patients who have exhausted surgical and radiotherapy options. Ongoing and future studies will help to answer unresolved questions such as the optimum use of radiation in resected WHO grade II meningiomas and the efficacy of additional chemotherapy agents.

The role of radiosurgery in the management of WHO Grade II and III intracranial meningiomas

2013 ◽  
Vol 35 (6) ◽  
pp. E16 ◽  
Author(s):  
Dale Ding ◽  
Robert M. Starke ◽  
John Hantzmon ◽  
Chun-Po Yen ◽  
Brian J. Williams ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Stereotactic Radiosurgery ◽  
Case Reports ◽  
Case Series ◽  
External Beam Radiation ◽  
Beam Radiation ◽  
Who Grade ◽  
Intracranial Meningiomas ◽  
Grade Ii ◽  
Grade Iii

Object WHO Grade II and III intracranial meningiomas are uncommon, but they portend a significantly worse prognosis than their benign Grade I counterparts. The mainstay of current management is resection to obtain cytoreduction and histological tissue diagnosis. The timing and benefit of postoperative fractionated external beam radiation therapy and stereotactic radiosurgery remain controversial. The authors review the stereotactic radiosurgery outcomes for Grade II and III meningiomas. Methods A comprehensive literature search was performed using PubMed to identify all radiosurgery series reporting the treatment outcomes for Grade II and III meningiomas. Case reports and case series involving fewer than 10 patients were excluded. Results From 1998 to 2013, 19 radiosurgery series were published in which 647 Grade II and III meningiomas were treated. Median tumor volumes were 2.2–14.6 cm3. The median margin doses were 14–21 Gy, although generally the margin doses for Grade II meningiomas were 16–20 Gy and the margin doses for Grade III meningiomas were 18–22 Gy. The median 5-year PFS was 59% for Grade II tumors and 13% for Grade III tumors, which may have been affected by patient age, prior radiation therapy, tumor volume, and radiosurgical dose and timing. The median complication rate following radiosurgery was 8%. Conclusions The current data for radiosurgery suggest that it has a role in the management of residual or recurrent Grade II and III meningiomas. However, better studies are needed to fully define this role. Due to the relatively low prevalence of these tumors, it is unlikely that prospective studies will be feasible. As such, well-designed retrospective analyses may improve our understanding of the effect of radiosurgery on tumor recurrence and patient survival and the incidence and impact of treatment-induced complications.

scholarly journals RADT-40. TRENDS IN THE USE OF RADIATION FOR MENINGIOMA ACROSS THE UNITED STATES

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii190-ii190
Author(s):  
Hirsch Matani ◽  
Stephen Abel ◽  
Linda Xu ◽  
Alexander Yu ◽  
Tulika Ranjan ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Tumor Size ◽  
Survival Benefit ◽  
Survival Curve ◽  
Survival Rates ◽  
The United States ◽  
External Beam Radiation ◽  
Treatment Facility ◽  
Beam Radiation ◽  
Who Grade

Abstract BACKGROUND Meningiomas are tumors originating from arachnoid cap cells on the surface of the brain or spinal cord. Treatment differs by grade but can consist of surgery, radiation therapy or both. We utilized the national cancer database (NCDB) to compare trends in the use stereotactic radiosurgery (SRS) and external beam radiation therapy (EBRT) in the management of meningioma. METHODS We queried the NCDB from 2004-15 for meningioma patients (Grade 1-3) treated with radiation therapy, either SRS or EBRT. Multivariable logistic regression was used to identify predictors of each treatment and to generate a propensity score. Propensity adjusted Kaplan-Meier survival curve analysis and multivariable cox hazards ratios were used to identify predictors of survival. RESULTS We identified 5406 patients with meningioma meeting above criteria. Median follow up was 43 months. 45%, 44%, and 11% were Grade 1, 2, and 3, respectively. Predictors for SRS were distance from treatment facility and histology. Predictors of EBRT were tumor size and WHO grade 2 or 3 disease. Tumor size, treatment year, and receipt of chemotherapy were associated with improved survival. Five and ten year survival rates were 89.2% vs. 72.6% (p < 0.0001) and 80.3% vs. 61.4% (p = 0.29) for SRS and EBRT respectively. After propensity matching 226 pairs were generated. For SRS, 5 year survival was not significantly improved at 88.2% (p = 0.056) CONCLUSIONS In the present analysis, predictors of SRS utilization in management of meningioma include distance from treatment facility and histology whereas conventional EBRT utilization was associated with tumor size and grade 2 or 3 disease. Despite a possible survival benefit with SRS, inherent selection bias may confound interpretation of the apparent survival benefit reflected in our study.

scholarly journals Radiation Therapy in Addition to Gross Total Resection of Retroperitoneal Sarcoma Results in Prolonged Survival: Results from a Single Institutional Study

2008 ◽  
Vol 2008 ◽  
pp. 1-11 ◽  
Author(s):  
Timothy M. Zagar ◽  
Robert R. Shenk ◽  
Julian A. Kim ◽  
Deb Harpp ◽  
Charles A. Kunos ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Disease Free Survival ◽  
Retroperitoneal Sarcoma ◽  
Gross Total Resection ◽  
External Beam Radiation ◽  
Adjuvant Radiation ◽  
Median Dose ◽  
Total Resection ◽  
Beam Radiation ◽  
Intraoperative Radiation

Purpose. Typical treatment of retroperitoneal sarcomas (RPSs) is surgery with or without radiation therapy for localized disease. With surgery alone, local failure rates are as high as 90%; this led to radiation therapy playing an important role in the treatment of RPSs.Methods. Thirty-one patients with retroperitoneal sarcoma treated with gross total resection and radiation therapy make up this retrospective analysis. Nineteen were treated preoperatively and 12 postoperatively (median dose, 59.4 Gy)—sixteen also received intraoperative radiation therapy (IORT) (median dose, 11 Gy). Patients were followed with stringent regimens, including frequent CT scans of the chest, abdomen, and pelvis.Results. With a median follow-up of 19 months (range 1–66 months), the 2-year overall survival (OS) rate is 70% (median, 52 months). The 2-year locoregional control (LRC) rate is 77% (median, 61.6 months). The 2-year distant disease free survival (DDFS) rate is 70% (median not reached). There were no differences in radiation-related acute and late toxicities among patients treated pre- versus postoperatively, whether with or without IORT.Conclusions. Compared to surgery alone, neoadjuvant or adjuvant radiation therapy offers patients with RPS an excellent chance for long-term LRC, DDS, and OS. The integration of modern treatment planning for external beam radiation therapy and IORT allows for higher doses to be delivered with acceptable toxicities.

Analysis of adjuvant chemotherapy and radiotherapy for stage II endometroid type endometrial cancer.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e17118-e17118
Author(s):  
Raanan Alter ◽  
William Adams ◽  
Alec Block ◽  
William Small ◽  
Matthew M. Harkenrider
Keyword(s):  
Radiation Therapy ◽  
Endometrial Cancer ◽  
Tumor Size ◽  
Tumor Grade ◽  
Figo Stage ◽  
Stage Ii ◽  
External Beam Radiation ◽  
Adjuvant Radiation ◽  
Beam Radiation ◽  
Time To Death

e17118 Background: Endometrial cancer (EC) is the most common gynecologic malignancy. Stage II EC - involving the cervix - is ideally treated with primary surgical staging; however, adjuvant treatment is not well defined. Methods: The population included 8,506 patients with 2009 FIGO Stage II endometrioid-type EC treated surgically within the National Cancer Database. Patients were categorized into six treatment groups: 1. hysterectomy (HYS) alone; 2. HYS with external beam radiation therapy (EBRT); 3. HYS with chemotherapy (CT); 4. HYS with CT and EBRT; 5. HYS with vaginal brachytherapy (VBT); and 6. HYS with CT and VBT. Univariable frailty survival analysis (UVA) that allowed for clustering of patients was used to determine the effect of sociodemographic factors and comorbidities on time to death; a generalized linear mixed effects allowing random intercepts for each treatment facility type was also used to estimate the odds of receiving any treatment beyond HYS. Results: 3,571 (42%) underwent HYS alone, while 4,312 (51%) received HYS with adjuvant radiation therapy (ART), 251 (3%) received HYS with CT, and 372 (4%) received all three modalities. Of those that received ART, 2,951 (68%) had EBRT while 1,361 (32%) had VBT. On UVA, black race, years of education, government insurance or uninsured status, higher tumor grade, greater Charlson-Deyo comorbidity count (CDCC), increased age, tumor grade, and tumor size all predicted poor survival. On MVA, patients receiving ART were only 0.747 (95% CI: 0.641 – 0.871) times as likely to die at any given time when compared with HYS alone ( p = .0002). There was no difference in survival between radiation modalities. Further, compared to patients who only received HYS, those who received HYS+CT had comparable survival estimates ( p = .20) as did patients who received HYS+ART+CT ( p = .24). When controlling for all other factors, higher tumor grade, increased CDCC, age, or tumor size, and Medicaid insurance were hazardous predictors of time to death ( p< .05). Conclusions: In stage II EC patients, adjuvant EBRT and VBT improved survival. Adjuvant CT did not improve survival though this should be further studied given the infrequency it was delivered in this population.

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