scholarly journals Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Andreas M. Stark ◽  
Ivo Leuschner ◽  
H. Maximilian Mehdorn ◽  
Alexander Claviez

Medulloblastoma, astrocytoma, and ependymoma represent the most common infratentorial tumors in childhood, while Ewing sarcomas in that localization are extremely rare. A large left infratentorial space-occupying lesion was diagnosed in a 12-year-old girl with signs of increased intracranial pressure. Following total tumor resection, histological and molecular examination revealed Ewing sarcoma with rearrangedEWSR-1gene. The patient achieved complete remission following adjuvant chemotherapy and radiotherapy according to Euro-EWING 2008 treatment protocol. Intracranial Ewing sarcoma, although rare, should be an important differential diagnosis of intracranial tumors in childhood which requires aggressive multimodal treatment.

Author(s):  
Sandeep Mohindra ◽  
Manjul Tripathi ◽  
Aman Batish ◽  
Ankur Kapoor ◽  
Ninad Ramesh Patil ◽  
...  

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.


2020 ◽  
Vol 2 (Supplement_3) ◽  
pp. ii12-ii13
Author(s):  
Shinichiro Koizumi ◽  
Kazuhiko Kurozumi

Abstract Introduction: The elasticity of intracranial tumors is difficult to assess non-invasively because the lesion is surrounded by the skull. Therefore, intracranial tumors have not been verified before surgery in terms of elastic modulus. Magnetic resonance elastography (MRE) is an epoch-making method capable of non-invasively imaging the elasticity of internal organs. We have examined the elasticity of meningiomas and pituitary adenomas and reported their usefulness. This time, we measured the glioma elasticity and verified usefulness of MRE. Method: Twenty-four gliomas (mean age 51.8±15.7 years, male: female = 17: 7) who underwent tumor resection after MRE imaging from July 2017 to May 2020 were targeted. The average elasticity was measured as an evaluation of tumor elastic modulus by MRE. Gliomas were divided into a low-grade glioma group (LGG: Grade 1, 2) and a high-grade glioma group (HGG: Grade 3, 4). Then, a comparative statistical study was conducted. Results: The average values of the average elasticity of LGG group (9 cases) and HGG group (15 cases) were 1.8±0.8 kPa and 2.5±0.8 kPa, respectively. The average elasticity was significantly higher in the HGG group (p=0.023). In the ROC analysis, the cutoff value was 2.1 kPa (sensitivity 70%, specificity 70%). Therefore, it was suggested that the tumor is likely to be HGG when the average elasticity is 2.1 kPa or more. Discussion: The glioma elasticity by preoperative MRE was significantly higher in the HGG group. Based on actual surgical experience, the tumor seems to be hard in the HGG group, and it was judged to be consistent with this our MRE research. The preoperative evaluation of glioma elasticity by MRE was considered useful, and it might help in planning a surgical strategy considering malignant grade.


2016 ◽  
Vol 17 (4) ◽  
pp. 434-438 ◽  
Author(s):  
Ritchell van Dams ◽  
Henry S. Park ◽  
Ahmed K. Alomari ◽  
Adele S. Ricciardi ◽  
Harini Rao ◽  
...  

This case report demonstrates that hypofractionated partial-brain radiation therapy with limited margins is a reasonable approach following gross tumor resection of Ewing sarcoma metastases to the brain. The patient presented with 2 intracranial metastases treated with gross-total resection followed by radiation therapy to 30 Gy in 5 fractions. The patient experienced symptomatic treatment-related inflammatory changes with resolution after receiving dexamethasone. He remains alive at 21 months of follow-up with no evidence of disease.


2020 ◽  
Vol 11 ◽  
pp. 186
Author(s):  
Mohamed Elnokaly ◽  
Gordon Mao ◽  
Khaled A. Aziz

Background: The minimally invasive approaches to the anterior skull base region through fronto-orbital craniotomy remain a highly accepted option that gains countenance and predilection over time. The transpalpebral “eyelid” incision is an under-utilized and more recent technique that offers a safe efficient corridor to manage a wide variety of lesions. Methods: We carried a retrospective study of 44 patients operated on by the fronto-orbital craniotomy through transpalpebral “eyelid” incision for intracranial tumors, in the time period from March 2007 to July 2016. The results from surgeries were analyzed; extent of tumor resection, length of hospital stay, cosmetic outcome, and complications. Results: Out of the 44 intracranial tumor cases, we had 16 male and 28 female patients with median age 54 years. We had 19 anterior skull base lesions, 8 middle skull base lesions and 8 parasellar lesions. We also operated on four frontal intraparenchymal lesions and four other various lesions. Total resection was achieved in 32 cases (72.7%), with excellent cosmetic outcome in 43 cases (97.7%). Average hospital stay was 6 days. No major complications recorded. Three cases (6.8%) had complications that varied between pseudomeningocele, wound infections, and facial pain. Follow-up average period was 23.6 months. Conclusion: The fronto-orbital approach through eyelid incision remains a reliable approach to the skull base. It provides natural anatomical dissection planes through the eyelid incision and a fronto-orbital craniotomy, creating a wide surgical corridor to manage specific lesions with consistent surgical and cosmetic outcome.


2020 ◽  
Vol 55 (5) ◽  
pp. 289-294
Author(s):  
Kadir Oktay ◽  
Ebru Guzel ◽  
Okay Baykara ◽  
Mevlana Akbaba ◽  
Ibrahim Sari ◽  
...  

<b><i>Introduction:</i></b> Thymic carcinoma metastases of the spinal column are very rare, especially in pediatric patients. To our knowledge, this is the first such pediatric case in the literature. <b><i>Case Presentation:</i></b> We report the case of a 14-year-old male patient with T12 and L1 metastases of thymic carcinoma. He had history of thymectomy and intrathoracic tumor resection 7 months previously. The patient’s neurological condition deteriorated; therefore, tumor resection and decompression of the spinal canal were performed. He underwent instrumentation and fusion procedures to prevent spinal instability. <b><i>Conclusion:</i></b> The main purpose of the treatment is gross total resection of the thymic carcinoma. However, adjuvant methods such as radiotherapy and chemotherapy should be added to the treatment protocol in patients who have higher stage diseases or those in whom total tumor resection cannot be achieved.


2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Ashley Albert ◽  
Robert Allbright ◽  
Todd Nichols ◽  
Edward Farley ◽  
Srinivasan Vijayakumar

Background. Pneumocephalus is a rare phenomenon that can occur as a complication after operations involving the thoracic discs, following thoracotomy for tumor resection, and after an intracranial operation or cranial trauma. This complication frequently occurs when a tumor is located in the costovertebral angle and an operative intervention creates a tear in the dura resulting in a pleural-dural fistula. Case Presentation. We describe the case of a 58-year-old man with an inoperable superior sulcus tumor who developed pneumocephalus after the initiation of chemoradiation secondary to a pleural-dural fistula. Conclusions. Although a rare occurrence, pneumocephalus should be considered when patients with tumors in the superior sulcus treated with radiation develop neurologic symptoms characteristic of increased intracranial pressure.


2013 ◽  
Vol 119 (1) ◽  
pp. 106-112 ◽  
Author(s):  
Ibrahim Hussain ◽  
Jean Anderson Eloy ◽  
Peter W. Carmel ◽  
James K. Liu

Craniopharyngiomas are benign intracranial tumors that arise in the suprasellar and intrasellar region in children and adults. They are associated with calcification on neuroimaging, endocrinopathies, vision problems, and recurrence following subtotal resection. Molecular studies into their genetic basis have been limited, and therefore targeted medical therapies for this tumor have eluded physicians. With the discovery of aberrant Wnt/β-catenin pathway signaling in the pathogenesis of the most common subtype of craniopharyngioma (adamantinomatous), the identification of candidate genes and proteins implicated in this cascade provide attractive targets for future therapies. The recent development of a genetically engineered animal model of this tumor may also serve as a platform for evaluating potential therapies prior to clinical trials in humans. Advances in understanding the molecular pathogenesis of tumor recurrence have also been made, providing clues to develop adjuvant and neoadjuvant therapies to couple with tumor resection for optimal response rates. Finally, advances in genomic technologies and next-generation sequencing will underlie the translation of these genetic and molecular studies from the bench to clinical practice. In this review, the authors present an analysis of the molecular oncogenesis of craniopharyngioma and current directions in the development of novel therapies for these morbid, yet poorly understood brain tumors.


2015 ◽  
Vol 14 (4) ◽  
pp. 399-401 ◽  
Author(s):  
Khairi Che Mat ◽  
Rohayah Husain ◽  
Nasir Mohamad

This case report presented a rare cause of severe headache in a patient who developed episodes of acute severe headache for the first time. The earliest accurate provisional diagnosis is crucial for a new onset severe headache in adult. A good history taking and high index of suspicious are remained the most important clinical component in managing severe first time headache in adult. The differential diagnosis of subarachnoid hemorrhage, space-occupying lesion with increased intracranial pressure, acute intoxication, meningo-encephalitis and others must be ruled out as these conditions are benefited in early management. In this patient, progressive development of headache is masked by severe intense headache, which later will be described as acute headache. The causes of newly onset severe intense headache will be discussed in the discussion.Bangladesh Journal of Medical Science Vol.14(4) 2015 p.399-401


1989 ◽  
Vol 71 (4) ◽  
pp. 506-511 ◽  
Author(s):  
Toshihiro Yasui ◽  
Akira Hakuba ◽  
Soo Han Kim ◽  
Shuro Nishimura

✓ The authors report eight cases of trigeminal neurinoma managed over the past 13 years with radical resection at a single-stage operation. Three patients were male and five were female, ranging in age from 25 to 56 years (mean 41.5 years). One had von Recklinghausen's disease. The tumors were located mainly within the middle fossa in two cases and within the posterior fossa in two, and extended both supra- and infratentorially in four cases. Facial pain and hearing disturbance were the main symptoms, with various other symptoms such as focal seizures, hemiparesis, gait disturbance, increased intracranial pressure, and visual disturbance also being noted. All patients underwent radical tumor resection with either a transpetrosal transtentorial or orbitozygomatic infratemporal surgical approach; the approach depended on the topography of the tumor. Total removal was performed in all cases. Only one patient, treated early in the series, required a second operation to remove the tumor completely. In another case the tumor recurred 5 years after the operation. There has been no operative mortality, but injury or permanent damage to the trigeminal branches was inevitable in many cases. The surgical results were excellent in three patients and good in five.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e22504-e22504
Author(s):  
Bivas Biswas ◽  
Akash Jha ◽  
Atul Batra ◽  
Moushumi Sengupta ◽  
Sameer Bakhshi

e22504 Background: Ewing sarcoma family tumor (ESFT) is a systemic disease and inflammatory in nature. Inflammatory biomarkers have been identified as prognostic marker in various malignancies including sarcoma. Here we have evaluated the prognostic implication of pretreatment neutrophil to lymphocyte ratio (NLR) in ESFT. Methods: We have calculated baseline NLR from peripheral blood parameters in 570 patients of ESFT treated with uniform chemotherapy protocol in our institution from June’2003 to Feb’2015. Treatment protocol consists of neoadjuvant chemotherapy followed by surgery and/or radiotherapy as local treatment modality and adjuvant chemotherapy. NLR was dichotomized as high and low with high NLR defined as value over the median. Data was censored on 30th Jan’2016. Results: Median age was 15 years (range: 0.1-55) with male: female ratio of 40:17 and 41% (n = 236) patients had metastasis at presentation. Most common sites of tumor were long bones in 209 (37%), thorax in 108 (19%) and pelvis in 90 (16%) patients. Hundred-one (18%) had a soft tissue primary. Twenty-five percent (n = 144) patients had systemic symptoms at presentation. Median symptom duration was 4 months (range: 0.5-36) with median tumor size 8.8 cm (range: 1.6-26). Median NLR was 1.55 (range: 0.08-12.87). High NLR was associated with older age (p = 0.002), female sex (p = 0.02), low serum albumin (p = 0.003) and high lactate dehydrogenase level (p = 0.03). After median follow-up of 32 months (range: 0.8-152), 5-year event-free survival (EFS) was 25.3% & 35.8% and overall survival (OS) was 38% & 48.7%, respectively in whole cohort and in those with localized disease. In the whole cohort, high NLR ( > 1.55) emerged as independent prognostic factor predicting inferior EFS (Hazard ratio-1.36, p = 0.03) along with metastasis at presentation (p < 0.001) and tumor diameter > 8 cm (p = 0.005), in multivariate analysis. NLR couldn’t predict OS. Conclusions: High pretreatment NLR emerged as independent prognostic factor predicting inferior EFS in patients with ESFT. It is an easily available and cost effective biomarker. Prognostic nature of NLR should be evaluated in a prospective study and if proven, should be included in prognostic model to tailor therapy.


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