scholarly journals Clinical Sequencing of High-Grade Undifferentiated Sarcomas: A Case Series and Report of an Aggressive Primary Cardiac Tumor With Multiple Oncogenic Drivers

2020 ◽  
pp. 1061-1069
Author(s):  
Chandan Kumar-Sinha ◽  
Bailey Anderson ◽  
Amer Heider ◽  
Josh N. Vo ◽  
Dan R. Robinson ◽  
...  
Author(s):  
Juan C. Espinoza-Cobos ◽  
Sergio Ortiz-Obregón ◽  
Enrique Blanco-Lemus ◽  
Gerardo Gutiérrez-Tovar ◽  
Luis E. Santos-Martínez ◽  
...  

2019 ◽  
Vol 12 (3) ◽  
pp. 220-228 ◽  
Author(s):  
Laura Evangelista ◽  
Lea Cuppari ◽  
Luisa Bellu ◽  
Daniele Bertin ◽  
Mario Caccese ◽  
...  

Purpose: The aims of the present study were to: 1- critically assess the utility of L-3,4- dihydroxy-6-18Ffluoro-phenyl-alanine (18F-DOPA) and O-(2-18F-fluoroethyl)-L-tyrosine (18F-FET) Positron Emission Tomography (PET)/Computed Tomography (CT) in patients with high grade glioma (HGG) and 2- describe the results of 18F-DOPA and 18F-FET PET/CT in a case series of patients with recurrent HGG. Methods: We searched for studies using the following databases: PubMed, Web of Science and Scopus. The search terms were: glioma OR brain neoplasm and DOPA OR DOPA PET OR DOPA PET/CT and FET OR FET PET OR FET PET/CT. From a mono-institutional database, we retrospectively analyzed the 18F-DOPA and 18F-FET PET/CT of 29 patients (age: 56 ± 12 years) with suspicious for recurrent HGG. All patients underwent 18F-DOPA or 18F-FET PET/CT for a multidisciplinary decision. The final definition of recurrence was made by magnetic resonance imaging (MRI) and/or multidisciplinary decision, mainly based on the clinical data. Results: Fifty-one articles were found, of which 49 were discarded, therefore 2 studies were finally selected. In both the studies, 18F-DOPA and 18F-FET as exchangeable in clinical practice particularly for HGG patients. From our institutional experience, in 29 patients, we found that sensitivity, specificity and accuracy of 18F-DOPA PET/CT in HGG were 100% (95% confidence interval- 95%CI - 81-100%), 63% (95%CI: 39-82%) and 62% (95%CI: 39-81%), respectively. 18F-FET PET/CT was true positive in 4 and true negative in 4 patients. Sensitivity, specificity and accuracy for 18F-FET PET/CT in HGG were 100%. Conclusion: 18F-DOPA and 18F-FET PET/CT have a similar diagnostic accuracy in patients with recurrent HGG. However, 18F-DOPA PET/CT could be affected by inflammation conditions (false positive) that can alter the final results. Large comparative trials are warranted in order to better understand the utility of 18F-DOPA or 18F-FET PET/CT in patients with HGG.


Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1453
Author(s):  
Chiara Fabbroni ◽  
Giovanni Fucà ◽  
Francesca Ligorio ◽  
Elena Fumagalli ◽  
Marta Barisella ◽  
...  

Background. We previously showed that grading can prognosticate the outcome of retroperitoneal liposarcoma (LPS). In the present study, we aimed to explore the impact of pathological stratification using grading on the clinical outcomes of patients with advanced well-differentiated LPS (WDLPS) and dedifferentiated LPS (DDLPS) treated with trabectedin. Patients: We included patients with advanced WDLPS and DDLPS treated with trabectedin at the Fondazione IRCCS Istituto Nazionale dei Tumori between April 2003 and November 2019. Tumors were categorized in WDLPS, low-grade DDLPS, and high-grade DDLPS according to the 2020 WHO classification. Patients were divided in two cohorts: Low-grade (WDLPS/low-grade DDLPS) and high-grade (high-grade DDLPS). Results: A total of 49 patients were included: 17 (35%) in the low-grade cohort and 32 (65%) in the high-grade cohort. Response rate was 47% in the low-grade cohort versus 9.4% in the high-grade cohort (logistic regression p = 0.006). Median progression-free survival (PFS) was 13.7 months in the low-grade cohort and 3.2 months in the high-grade cohort. Grading was confirmed as an independent predictor of PFS in the Cox proportional-hazards regression multivariable model (adjusted hazard ratio low-grade vs. high-grade: 0.45, 95% confidence interval: 0.22–0.94; adjusted p = 0.035). Conclusions: In this retrospective case series, sensitivity to trabectedin was higher in WDLPS/low-grade DDLPS than in high-grade DDLPS. If confirmed in larger series, grading could represent an effective tool to personalize the treatment with trabectedin in patients with advanced LPS.


2004 ◽  
Vol 68 (10) ◽  
pp. 961-963 ◽  
Author(s):  
Yoshikazu Ohara ◽  
Yoshikazu Hiasa ◽  
Shinobu Hosokawa ◽  
Koji Yamaguchi ◽  
Riyo Ogura ◽  
...  

2022 ◽  
pp. 000348942110694
Author(s):  
Holden W. Richards ◽  
Caitlin Bertelsen ◽  
Bronwyn Hamilton ◽  
David Sauer ◽  
Joshua Schindler

Objectives: Discussions regarding the specific management and outcomes for laryngeal MEC are limited to very small, single-institution case series. To look further into the diagnosis and management of these uncommon non-squamous cell carcinomas of the larynx, we present 3 recent cases of laryngeal MEC treated at our institution. Methods: Patients at a tertiary hospital treated for MEC between October 2019 and December 2020 were retrospectively identified. Chart review, imaging analysis, and histologic slide creation were completed for all patients. Results: We identified and treated 2 patients with high-grade supraglottic and 1 patient with intermediate-grade glottic MEC. These patients presented to our clinic with a primary complaint of either gradual, worsening dysphonia, dysphagia, or both. All patients underwent laryngovideostroboscopy as well as panendoscopy with directed submucosal biopsy, which was consistent with MEC. MRI was performed in 2 of the cases further elucidating the extent of submucosal spread. PET-CT was performed in all 3 cases, and none demonstrated evidence of regional or distal metastases. Surgically, high-grade MEC lesions were treated with a total laryngectomy. The intermediate MEC lesion was managed with a supracricoid partial laryngectomy (SCPL). Surgical margins were free of tumor in all cases with no nodal metastases by modified radical neck dissection. Radiation therapy was offered to both high-grade MEC patients and declined by one. Radiation was not recommended to the patient with intermediate-grade MEC as we believed that the risk of additional treatment outweighed the benefit. Conclusion: We believe that MEC of the larynx should be considered in patients with atypical submucosal laryngeal masses. Laryngovideostroboscopy, MRI, and PET imaging may be valuable in determining the extent of the lesions and planning appropriate surgery. Postoperative radiation therapy should be considered a per tumor grade in other more studied sites, as there is no data on efficacy in laryngeal MEC.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Hiroshige Murata ◽  
Yasushi Miyauchi ◽  
Takashi Nitta ◽  
Kenta Takahashi ◽  
Ippei Tsuboi ◽  
...  

Introduction: Ventricular tachycardia (VT) associated with a primary cardiac tumor is extremely rare. Complete resection of a tumor was reported to be effective in a treatment of this VT. However, the mechanism of the cardiac tumor-related VT (CT-VT) is still unknown, and the therapeutic strategy of the VT in patients with unresectable tumor is not determined. Methods and Results: Four patients (20 ± 15 years, 3 males) with CT-VT (fibroma in 2, lipoma in 1, and hemangioma in 1 patient) were investigated. All four patients developed repetitive forms of monomorphic VTs, which were reproducibly induced by programmed ventricular stimulation and terminated by burst pacing. These VTs exhibited a right bundle branch block QRS morphology (QRS duration, 160 ± 28 ms) with a pseudo-delta wave (75 ± 10 ms) at a cycle length of 330 ± 86 ms. Intraoperative electroanatomical mapping showed a radially spreading activation pattern originating from the epicardial border of the tumor, where fractionated and late potentials were detected during sinus rhythm. Histopathological studies of the sections from this border area revealed tumor infiltration to the surrounding myocardium and myocardial cell disorganization exhibiting myocardial disarray. In 2 patients in whom the cardiac tumor was completely resected, cryoablation was added to the resection line. In the remaining 2 patients in whom complete resection of the tumor was unfeasible, encircling cryoablation to entirely isolate the unresectable tumor was effective in suppressing their VTs. Conclusions: The mechanism of CT-VT is reentry localized at the epicardial border of the tumor. Myocardial disarray associated with infiltration of the cardiac tumor may be a substrate of this VT. Encircling cryoablation along the border of the tumor may be a therapeutic option for an unresectable CT-VT.


Author(s):  
Aikeremujiang Muheremu ◽  
Tianlin Wen ◽  
Xiaohui Niu

Objective: The current study was carried out to assess the value of positron emission tomography (PET)/CT on the diagnosis and staging of primary musculoskeletal tumors. Methods: PET–CT test results and histopathological study reports of all the patients with primary musculoskeletal tumors in our department from January 2006 to July 2015 were retrospectively reviewed. Maximum standardized uptake value (SUVmax) in these PET–CT reports were recorded and analyzed respectively for each type of sarcoma. Results: A total of 255 patients were included in the final analysis. Sensitivity of SUVmax based diagnosis was 96.6% for primary malignant osseous sarcomas and 91.2% for soft tissue sarcomas. SUVmax of high-grade osseous sarcomas (average 8.4 ± 5.5) was significantly higher (p < 0.001) than low-grade osseous sarcomas (average 3.9 ± 1.8); based on current case series, SUVmax of high-grade soft tissue sarcomas (7.5 ± 5.1) was not significantly different (p = 0.229) from that of low-grade soft tissue sarcomas (5.3 ± 3.7). Significant decrease of SUVmax value after chemotherapy was associated with favorable prognosis in patients with osteosarcoma. Conclusion: Results of the current study indicate that, the SUVmax based application of PET–CT can be a valuable supplementary method to histopathological tests regarding the diagnosis and staging of primary musculoskeletal sarcomas. Advances in knowledge: SUVmax based application of PET–CT is a highly sensitive method in diagnosis of primary osseous and soft tissue sarcomas in Chinese patients.


2016 ◽  
Vol 53 (4) ◽  
pp. 558 ◽  
Author(s):  
VM Patil ◽  
R Abhinav ◽  
R Tonse ◽  
S Epari ◽  
T Gupta ◽  
...  

2019 ◽  
Vol 13 ◽  
pp. 117955651987052 ◽  
Author(s):  
Sara Sinno ◽  
Adel M Assaad ◽  
Nina Salem Shabb

Oropharyngeal small cell carcinomas (OPSmCC) are rare with only few case reports and case series published in the literature. More recently, an association of these tumors with human papillomavirus (HPV) infection has been detected. However, unlike oropharyngeal squamous cell carcinomas which have a better outcome when associated with HPV, OPSmCC exhibit an aggressive behavior. In this article, we report a case of tonsillar carcinoma arising in a 14-year-old boy that was associated with HPV infection. The tumor exhibited morphologic features of small cell carcinoma with no overt squamous differentiation. Yet, by immunohistochemistry, it showed diffuse and strong co-expression of both squamous and neuroendocrine markers. In addition, we present the clinicopathologic features of all the cases of OPSmCC reported in the literature for which p16 and/or HPV testing have been done.


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