scholarly journals SURG-35. INTRACRANIAL MYXOID MESENCHYMAL TUMOR WITH EWSR1-ATF1 GENE FUSION

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii210-ii211
Author(s):  
Benjamin Ward ◽  
Christopher Wang ◽  
Rob Macaulay ◽  
James Liu

Abstract Intracranial myxoid mesenchymal tumors (IMMT) carrying an EWSR1-CREB gene family fusion are extremely rare and have only been identified in ten relatively recent reported cases. There is some question as to whether this is a novel entity, or a myxoid variant of angiomatoid fibrous histiocytoma (AFH), given certain histopathological similarities. Despite these similarities, the increasing number of cases reported appear to demonstrate clear histological differences that indicate IMMTs are a distinct and novel entity. Previous reports have focused on histological analysis but have lacked detailed long-term clinical follow-up and recommendations regarding treatment approach. In this case, we describe a 48-year-old female who presented with a left intraventricular mass that was identified histologically as an IMMT with an EWSR1-ATF1 gene fusion. Following initial resection, the tumor demonstrated local recurrence. Repeat resection was performed followed by immediate demonstration of local, as well as distant, tumor recurrence. Subsequent histological analysis of the tumor demonstrated a myxoid mesenchymal tumor clearly distinct from AFH. Fractionated stereotactic radiation therapy was administered following the second resection and tumor control was achieved at 1 year. This case is particularly remarkable as it is only the second reported IMMT case to occur in a middle-aged adult, with all other cases occurring in children or young adults. The two middle-aged adult cases share striking similarities in clinical presentation, including a history of breast cancer. Our findings indicate that an intracranial myxoid mesenchymal tumor is a novel and rare entity that may demonstrate rapid local and distant recurrence. Given the aggressive recurrence seen with the presented case, we recommend the treatment plan to be surgical resection followed by adjuvant radiation therapy to maintain tumor control.

Author(s):  
Ernest Osei ◽  
Susan Dang ◽  
Johnson Darko ◽  
Katrina Fleming ◽  
Ramana Rachakonda

Abstract Background: Breast cancer is the most commonly diagnosed cancer among women and the second leading cause of cancer-related death in Canadian women. Surgery is often the first line of treatment for low-risk early stage patients, followed by adjuvant radiation therapy to reduce the risk of local recurrence and prevent metastasis after lumpectomy or mastectomy. For high-risk patients with node positive disease or are at greater risk of nodal metastasis, radiation therapy will involve treatment of the intact breast or chest-wall as well as the regional lymph nodes. Materials and methods: We retrospectively evaluated the treatment plans of 354 patients with breast cancer with nodes positive or were at high risk of nodal involvement treated at our cancer centre. All patients were treated with a prescription dose of 50 Gy in 25 fractions to the intact breast or chest-wall and 50 Gy in 25 fractions to the supraclavicular region and, based on patient suitability and tolerance, were treated either using the deep inspiration breath hold (DIBH) or free-breathing (FB) techniques. Results: Based on patient suitability and tolerance, 130 (36·7%) patients were treated with DIBH and 224 (63·3%) with FB techniques. There were 169 (47·7%) patients treated with intact breast, whereas 185 (52·3%) were treated for post-mastectomy chest-wall. The mean PTV_eval V92%, V95%, V100% and V105% for all patients are 99·4 ± 0·7, 97·6 ± 1·6, 74·8 ± 7·9 and 1·5 ± 3·2%, respectively. The mean ipsilateral lung V10Gy, V20Gy and V30Gy are 30·0 ± 5·3, 22·4 ± 4·7 and 18·4 ± 4·3% for intact breast and 30·9 ± 5·8, 23·5 ± 5·4 and 19·4 ± 5·0% for post-mastectomy patients with FB, respectively. The corresponding values for patients treated using DIBH are 26·3 ± 5·9, 18·9 ± 5·0 and 15·6 ± 4·7% for intact breast and 27·5 ± 6·5, 20·6 ± 5·7 and 17·1 ± 5·2% for post-mastectomy patients, respectively. The mean heart V10Gy, V20Gy, is 1·8 ± 1·7, 0·9 ± 1·0 for intact breast and 3·1 ± 2·2, 1·7 ± 1·6 for post-mastectomy patients with FB, respectively. The corresponding values with the DIBH are 0·5 ± 0·7, 0·1 ± 0·4 for intact breast and 1·1 ± 1·4, 0·4 ± 0·7 for post-mastectomy patients, respectively. Conclusion: The use of 3 and/or 4 field hybrid intensity-modulated radiation therapy technique for radiation therapy of high-risk node positive breast cancer patients provides an efficient and reliable method for achieving superior dose uniformity, conformity and homogeneity in the breast or post-mastectomy chest-wall volume with minimal doses to the organs at risk. The development and implementation of a consistent treatment plan acceptability criteria in radiotherapy programmes would establish an evaluation process to define a consistent, standardised and transparent treatment path for all patients that would reduce significant variations in the acceptability of treatment plans.


2008 ◽  
Vol 24 (5) ◽  
pp. E4 ◽  
Author(s):  
Marco Lee ◽  
M. Yashar S. Kalani ◽  
Samuel Cheshier ◽  
Iris C. Gibbs ◽  
John R. Adler ◽  
...  

Object Many benign intracranial tumors are amenable to radiotherapy treatment including meningiomas, schwannomas, pituitary tumors, and craniopharyngiomas. The authors present their experience in the treatment of craniopharyngiomas in 16 patients using frameless CyberKnife stereotactic radiosurgery (SRS). The authors discuss the role of radiation therapy in the management of these tumors, and more specifically, the role of CyberKnife SRS. Methods Sixteen patients were treated for residual or recurrent craniopharyngioma between 2000 and 2007 with CyberKnife SRS at Stanford University Medical Center. All patients underwent magnetic resonance imaging and visual and neuroendocrine evaluations before and at regular intervals after SRS. A multisession treatment regimen and a nonisocentric treatment plan for each patient were used with a mean marginal dose of 21.6 Gy and a mean maximal dose of 29.9 Gy. Results There were adequate clinical data to assess outcomes in 11 of 16 patients. Evaluation of patients between 13 and 71 years of age (mean 34.5 years) with a mean follow-up period of 15.4 months revealed no deterioration in visual or neuroendocrine function. Tumor shrinkage was achieved in 7 of these 11 patients, and tumor control in another 3. One patient had cystic enlargement of the residual tumor. Conclusions The authors' early experience with the application of CyberKnife SRS to residual or recurrent craniopharyngiomas has been positive; control or shrinkage of the tumor was achieved in 91% of patients, with no visual or neuroendocrine complications. Longer-term follow-up with a larger group of patients is required to fully evaluate the safety and effectiveness of this treatment modality.


2018 ◽  
Vol 19 (1) ◽  
pp. 64
Author(s):  
Sadiq R Malik ◽  
Shohel Reza ◽  
MM Shakhawat Hossain

<p><span>Advancement in Cancer Therapy Technology (CTT) due to Software, Hardware and precise delivery of radiation dose has enhanced the quality of life of cancer patients. This report aims at the application of 3-D CRT (Three Dimensional Conformal Radiation Therapy) and IMRT (Intensity Modulated Radiation Therapy) for a quality of treatment. Other anatomical sites viz. Prostate, Lung, etc. may also be treated provided a better tool is applied for target delineation for which FUSION of CT and MRI images are used to ascertain differences in tissue density. This Fusion image of 3 mm slices offer accurate contouring of the tumor. The oncologist and/or physicist perform delineation of (I) GTV (Gross Tumor Volume), (II) CTV (Clinical Target Volume), (III) PTV (Planning Target Volume), (IV) TV (Treated Volume) and (V) OARs (Organs at Risk). This is done to secure conformal dose distribution and justify the clinical objectives of Tumor Control Probability (TCP) by reducing the normal tissue complication probability (NTCP). <span> </span><span> </span>The implication of this study outlines the fundamental goal of effective treatment procedures by comparing treatment plans of 3-D CRT and IMRT. Tolerance levels of dose to different organs are optimized by the analysis of random and systemic geometrical deviations, margin on target volumes, conformity index (CI), patient selection process and, of course, the shape and stage of target. The comparative parameters of treatment plans are segmented and tabulated to implicate the application of necessary tools to decide on a treatment plan for similar patients.</span></p><p><span>Bangladesh J. Nuclear Med. 19(1): 64-67, January 2016</span></p>


2021 ◽  
Vol 11 ◽  
Author(s):  
Huzaifa Piperdi ◽  
Daniella Portal ◽  
Shane S. Neibart ◽  
Ning J. Yue ◽  
Salma K. Jabbour ◽  
...  

Lung cancer treatment is constantly evolving due to technological advances in the delivery of radiation therapy. Adaptive radiation therapy (ART) allows for modification of a treatment plan with the goal of improving the dose distribution to the patient due to anatomic or physiologic deviations from the initial simulation. The implementation of ART for lung cancer is widely varied with limited consensus on who to adapt, when to adapt, how to adapt, and what the actual benefits of adaptation are. ART for lung cancer presents significant challenges due to the nature of the moving target, tumor shrinkage, and complex dose accumulation because of plan adaptation. This article presents an overview of the current state of the field in ART for lung cancer, specifically, probing topics of: patient selection for the greatest benefit from adaptation, models which predict who and when to adapt plans, best timing for plan adaptation, optimized workflows for implementing ART including alternatives to re-simulation, the best radiation techniques for ART including magnetic resonance guided treatment, algorithms and quality assurance, and challenges and techniques for dose reconstruction. To date, the clinical workflow burden of ART is one of the major reasons limiting its widespread acceptance. However, the growing body of evidence demonstrates overwhelming support for reduced toxicity while improving tumor dose coverage by adapting plans mid-treatment, but this is offset by the limited knowledge about tumor control. Progress made in predictive modeling of on-treatment tumor shrinkage and toxicity, optimizing the timing of adaptation of the plan during the course of treatment, creating optimal workflows to minimize staffing burden, and utilizing deformable image registration represent ways the field is moving toward a more uniform implementation of ART.


2010 ◽  
Vol 113 (2) ◽  
pp. 319-324 ◽  
Author(s):  
Aaron J. Clark ◽  
Michael E. Ivan ◽  
Michael E. Sughrue ◽  
Isaac Yang ◽  
Derick Aranda ◽  
...  

Object Pineocytoma is a rare tumor, and the current literature on these tumors is primarily composed of case reports and small case series. Thus, recommendations on appropriate treatment of these tumors are highly varied. Therefore, the authors performed a systematic review of the literature on tumor control after surgery for pineocytoma to determine the relative benefits of aggressive resection and postoperative adjuvant radiotherapy. Methods A comprehensive search of the published English-language literature was performed to identify studies citing outcome data of patients undergoing surgery for pineocytoma. Determination of rates of progression-free survival (PFS) was performed using Kaplan-Meier analysis. Results Sixty-four articles met the criteria of the established search protocol, which combined for a total of 166 patients. Twenty-one percent of these patients had undergone a biopsy procedure, 38% had undergone subtotal resection (STR), 42% had undergone gross-total resection, and 28% were treated with radiation therapy. The 1- and 5-year PFS rates for the resection group versus the biopsy group were 97 and 90% (1 year), and 89 and 75% (5 years), respectively (p < 0.05, log-rank test). The 1- and 5-year PFS rates for the GTR group versus the group undergoing STR combined with radiation therapy were 100 and 94% (1 year), and 100 and 84% (5 years), respectively (p < 0.05, log-rank test). There was no significant difference in PFS for STR only compared with STR in addition to radiation therapy. Conclusions Gross-total resection is the ideal treatment for pineocytoma and might represent a cure for these lesions. When gross-total resection is not possible, adjuvant radiation therapy after STR is of questionable benefit for these patients.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii190-ii190
Author(s):  
Shearwood McClelland ◽  
Ulysses Gardner ◽  
Mitesh Shah ◽  
Gordon Watson ◽  
Kevin Shiue

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 &lt; 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 (&lt; 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.


2004 ◽  
Vol 171 (4S) ◽  
pp. 279-280
Author(s):  
Jonathan Rubenstein ◽  
Misop Han ◽  
Sheila A. Hawkins ◽  
William J. Catalona

2020 ◽  
Vol 133 (5) ◽  
pp. 1355-1359
Author(s):  
Maria Peris-Celda ◽  
Laura Salgado-Lopez ◽  
Carrie Y. Inwards ◽  
Aditya Raghunathan ◽  
Carrie M. Carr ◽  
...  

Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors’ knowledge, have not been previously documented at this anatomical site.An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.


2004 ◽  
Vol 130 (6) ◽  
pp. 327-333 ◽  
Author(s):  
Hans Geinitz ◽  
Frank B. Zimmermann ◽  
Reinhard Thamm ◽  
Monika Keller ◽  
Raymonde Busch ◽  
...  

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