scholarly journals The importance of surgery as part of multimodal therapy in rapid progressive primary extraosseous Ewing sarcoma of the cervical intra- and epidural space

2016 ◽  
Vol 6 (4) ◽  
Author(s):  
Richard Bostelmann ◽  
Mario Leimert ◽  
Hans Jakob Steiger ◽  
Kirstin Gierga ◽  
Athanasios K. Petridis

Primary extraosseous Ewing sarcomas (EESs) are an extremely rare pathological entity. Less than 32 cases have been reported in the literature. Here we report an uncommon case with very rapid progression in the cervical region with extra- and intradural involvement. We present a thorough review of the literature and discuss possible treatment modalities. The Medline database was searched using the search terms: <em>Ewing sarcoma</em>, <em>extraosseus tumour</em>, <em>treatment</em>, <em>management</em>, <em>cervical spine</em>. A previously healthy 29-year-old man complained of right-sided radiculopathy (C7). Magnetic resonance imaging showed an enhancing foraminal, sandglass shaped neurinoma- like lesion. Surgery revealed an intraand extra-dural lesion, which was histologically diagnosed as Ewing sarcoma. Despite gross total resection, there was a massive symptomatic tumor recurrence within 6 weeks. A second gross total resection was realized. The patient was treated according to the EURO E.W.I.N.G.-Protocol (VIDE) and recovered very well (progression-free interval during therapy). Several decompressive re-surgeries were realized with adjuvant radio-chemotherapy. At the last follow-up (17 months after initial surgery) the patient was in remission with a good quality of live. This case is to illustrate that despite extensive therapeutic efforts, the progression- free survival in case of primary EES may be very short. To maintain neurological function and good quality of live as long as possible, a multimodal strategy seems to be adequate. Like in the present case this implies several surgeries and adjuvant chemo-and radiotherapy. Whether this improves overall survival remains unclear.

2021 ◽  
Vol 10 ◽  
Author(s):  
Lingzhe He ◽  
Buyi Zhang ◽  
Jianmin Zhang ◽  
Zhige Guo ◽  
Feina Shi ◽  
...  

BackgroundIt still remains unclear whether patients with atypical meningioma (AM) could benefit from postoperative adjuvant radiotherapy (PORT) after gross-total resection (GTR).ObjectiveExploring the effectiveness of PORT on AM patients after GTR.MethodsLiteratures on PubMed, Embase, Web of science, and Scopus databases published between January 2000 and January 2019 were searched. After the selection based on the certain exclusion criteria, the Newcastle-Ottawa evaluation scale was used to evaluate the quality of the included literatures. Finally, a meta-analysis was conducted to analyze the effectiveness of PORT on local control (LC), progression-free survival (PFS) and overall survival (OS) in atypical meningioma patients after GTR.ResultsA total of 17 articles with 2,008 AM patients were included in the meta-analysis. The 5-year LC, 5-year PFS, and 5-year OS rates were 82.2, 84.1, and 79.0%, respectively, for AM patients receiving PORT after GTR, and they were 71.0, 71.9, and 81.5%, respectively, for those not receiving PORT after GTR. PORT could significantly improve 5-year LC rate (OR [95% Cl] = 2.59 [1.40–4.81], P = 0.002) and 5-year PFS rate (OR [95% Cl] = 1.99 [1.35–2.95], P = 0.001), but did not significantly improve 5-year OS rate (OR [95% Cl] = 1.07 [0.60–1.91], P = 0.828).ConclusionPORT could improve the 5-year LC rate and 5-year PFS rate in AM patients after GTR. AM patients might benefit from PORT after GTR.


2020 ◽  
Vol 16 (35) ◽  
pp. 2997-3013
Author(s):  
Kentaro Kogushi ◽  
Michael LoPresti ◽  
Shunya Ikeda

Background: Synovial sarcoma (SS) is a rare, aggressive soft tissue sarcoma with a poor prognosis after metastasis. The objective of this study was to conduct a systematic review of the clinical evidence for therapeutic options for adults with metastatic or advanced SS. Materials & methods: Relevant databases were searched with predefined keywords. Results: Thirty-nine publications reported clinical data for systemic treatment and other interventions. Data on survival outcomes varied but were generally poor (progression-free survival: 1.0–7.7 months; overall survival: 6.7–29.2 months) for adults with metastatic and advanced SS. A high frequency of neutropenia with systemic treatment and low quality of life post-progression were reported. Conclusion: Reported evidence suggests poor outcomes in adults with metastatic and advanced SS and the need for the development of new treatment modalities.


2020 ◽  
Vol 137 ◽  
pp. e176-e182 ◽  
Author(s):  
Anil K. Mahavadi ◽  
Priyen M. Patel ◽  
Manish Kuchakulla ◽  
Ashish H. Shah ◽  
Dan Eichberg ◽  
...  

2007 ◽  
Vol 7 (2) ◽  
pp. 264-269 ◽  
Author(s):  
Mirza N. Baig ◽  
Syed Saquib ◽  
Greg Christoforidis ◽  
Louis P. Caragine

✓Spinal hemangiomas can be categorized into three different groups based on location. Vertebral body (VB) heman-giomas are frequent incidental findings on magnetic resonance (MR) imaging. There is a subdivision of these with spinal epidural extension that have been reported in the literature. Spinal hemangiomas can also be epidural without VB involvement; these are extremely rare with few reported cases in the thoracic epidural spinal column. The diagnosis and imaging characteristics as well as the surgical tools used in gross-total resection of spinal epidural hemangioma are not well understood. The authors present a detailed characterization of a spinal epidural hemangioma in a 30-year-old woman who presented with complaints of gradual onset of low-back pain that worsened over 1 year. The MR imaging findings indicated a large L2–S1 epidural spinal mass causing thecal sac compression. The patient underwent an L2–S1 laminectomy, and a vascular extradural mass was noted on the posterior aspect of the dura mater. Preoperative spinal angiography as well as intraoperative angiography was performed. Total resection of the tumor was achieved using intraoperative embolization with sodium tetradecyl sulfate and microscopic dissection. The postoperative MR imaging findings and clinical outcome were excellent. The findings and use of sodium tetradecyl sulfate in gross-total resection are discussed. The authors also review treatment modalities and demonstrate the utility and effectiveness of intraoperative sodium tetradecyl sulfate in gross-total resection of large difficult spinal epidural hemangiomas.


2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi17-vi17
Author(s):  
Davy Deng ◽  
Lubna Hammoudeh ◽  
Daniel Cagney ◽  
J Ricardo McFaline-Figueroa ◽  
Ugonma Chukwueke ◽  
...  

Abstract BACKGROUND Glioblastoma (GBM) patients are treated with radiation therapy (RT), temozolomide, and corticosteroids which can affect hematologic and immunologic parameters. We examined lymphocytes, neutrophil-to-lymphocyte ratio and platelet measurements and their association with progression-free survival (PFS) overall survival (OS). METHODS We identified 759 newly diagnosed adult GBM patients treated at our institution in the temozolomide (TMZ) era with blood counts that could be automatically extracted from the electronic medical record during chemoradiation (CRT, defined as within 42 days of RT) and at first recurrence. Linear regression and Cox modeling were used to evaluate outcomes. RESULTS Median age was 60.3 years; 87% had KPS ≥ 70, 37.5% had gross total resection, and 90% received TMZ. Prior to RT, 56.4% (375/665) patients had a lymphocyte measurement &lt; 1.0 × 1000 cells [K]/μL. Within 42 days of CRT, 81.7% (536/656) had a lymphocyte measurement &lt; 1.0 K/μL, 37.8% (248/656) &lt; 0.5 K/μL. 10.7% (58/544) patients developed grade 2 or higher neutropenia, 9.1% (50/547) patients developed grade 2 or higher thrombocytopenia. On multivariable analysis (MVA), older age (AHR1.03, p&lt; 0.001), unmethylated MGMT status (AHR2.56,p&lt; 0.001), lower RT dose (&lt;54Gy, AHR 3.45, p&lt; 0.001), male sex (AHR1.45, p=0.02), non-gross total resection (AHR1.63, p&lt; 0.001), lymphopenia during CRT (AHR0.63, p=0.008) and higher NLR during CRT (AHR1.02, p=0.001) were significantly associated with worse OS. Older age (AHR1.01, p=0.02), unmethylated MGMT status (AHR2.44, p&lt; 0.001), lower RT dose (AHR1.82, p=0.02), higher NLR during CRT (AHR1.03, p &lt; 0.001) were significantly associated with worse PFS on MVA. At first recurrence, median lymphocyte count was 0.7 K/μL with 74% (348/468) patients &lt; 1.0 K/μL and 27% &lt; 0.5 K/μL. CONCLUSION Lymphopenia and higher neutrophil-to-lymphocyte ratio are associated with inferior outcomes. Persistent lymphopenia at time of first recurrence may have implications for clinical trial eligibility and immunotherapy approaches in recurrent GBM.


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