scholarly journals Epithelioid hemangioendothelioma of bone: A survival analysis of 50 cases from the SEER database (1992–2016)

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110055
Author(s):  
Charles A Gusho ◽  
Sarah C Tepper ◽  
Steven Gitelis ◽  
Alan T Blank

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that may arise in bone. The purpose of this investigation was to determine the clinicopathological features and outcomes of osseous EHE in a large patient series, and to assess whether survival is impacted by demographics, tumor characteristics, or treatment factors. This was a retrospective review of the Surveillance, Epidemiology and End Results (SEER) database from 1992 to 2016. Kaplan-Meier was used to estimate overall survival (OS) and disease-specific survival (DSS). A Cox regression model was used to identify prognostic factors. Fifty patients from 1992 to 2016 with a median age of 54.5 years (IQR, 37–67) were reviewed. For location, 46% ( n = 23) of tumors arose from the appendicular skeleton while 38% ( n = 19) occurred within the axial skeleton (overlapping EHE: 16%, n = 8). Of the cases with recorded treatment factors, 54.8% ( n = 23) had surgery, 26% ( n = 13) received radiation, 22% ( n = 11) were treated with chemotherapy, and 26% ( n = 13) had surgery plus radiation. The 5-year OS probability was 49.2% (95% CI, 23.6–70.6), and the 5-year DSS probability was 63.9% (95% CI, 33.0–83.5). No surgery (surgery: HR, 0.262; 95% CI, 0.07–0.9); p = 0.041) and age older than 50 years (HR, 4.117; 95% CI, 1.1–15.4; p = 0.035) were negative prognostic factors of disease-specific mortality after controlling for confounding variables. There was no association between disease-specific mortality and adjuvant or multimodal therapy. The prognosis of EHE of bone is less than favorable, and the 5-year DSS probability of 64% emphasizes the intermediate grade nature of this tumor subtype. Surgical treatment, when feasible, is associated with a better prognosis.

BMC Cancer ◽  
2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Rei Kamitani ◽  
Kazuhiro Matsumoto ◽  
Toshikazu Takeda ◽  
Ryuichi Mizuno ◽  
Mototsugu Oya

Abstract Background Paratesticular leiomyosarcoma (LMS) is a rare tumor. Conventionally, tumor resection by high inguinal orchiectomy is performed as the preferred treatment approach for paratesticular sarcoma. On the other hand, testis-sparing surgery has recently attracted attention as a less-invasive treatment option for paratesticular sarcoma. However, the prognostic predictors and optimal treatment strategy for paratesticular LMS remain unclear because of its rarity. In this study, we systematically reviewed previously reported cases of paratesticular LMS to evaluate the prognostic factors and establish the optimal treatment strategy. Methods A systematic search of Medline, Web of Science, Embase, and Google was performed to find articles describing localized paratesticular LMS published between 1971 and 2020 in English. The final cohort included 217 patients in 167 articles. The starting point of this study was the time of definitive surgical treatment, and the end point was the time of local recurrence (LR), distant metastasis (DM), and disease-specific mortality. Results Patients with cutaneous LMS had a slightly better LR-free survival, DM-free survival, and disease-specific survival than those with subcutaneous LMS (p = 0.745, p = 0.033, and p = 0.126, respectively). Patients with higher grade tumors had a significantly higher risk of DM and disease-specific mortality (Grade 3 vs Grade 1 p < 0.001, and Grade 3 vs Grade 1 p < 0.001, respectively). In addition, those with a microscopic positive margin had a significantly higher risk of LR and DM than those with a negative margin (p < 0.001, and p = 0.018, respectively). Patients who underwent simple tumorectomy had a slightly higher risk of LR than those who underwent high inguinal orchiectomy (p = 0.067). Subgroup analysis of cutaneous LMS demonstrated that the difference in LR between simple tumorectomy and high inguinal orchiectomy was limited (p = 0.212). On the other hand, subgroup analysis of subcutaneous LMS revealed a significant difference in LR (p = 0.039). Conclusions Our study demonstrated that subcutaneous LMS and high-grade tumors are prognostic factors for paratesticular LMS. For subcutaneous LMS, tumorectomy with high inguinal orchiectomy should be the optimal treatment strategy to achieve a negative surgical margin.


2020 ◽  
Author(s):  
muyuan liu ◽  
Litian Tong ◽  
Manbin Xu ◽  
Xiang Xu ◽  
Bin Liang ◽  
...  

Abstract Background: Due to the low incidence of mucoepidermoid carcinoma, there lacks sufficient studies for determining optimal treatment and predicting prognosis. The purpose of this study was to develop prognostic nomograms, to predict overall survival and disease-specific survival (DSS) of oral and oropharyngeal mucoepidermoid carcinoma patients, using the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database. Methods: Clinicopathological and follow-up data of patients diagnosed with oral and oropharyngeal mucoepidermoid carcinoma between 2004 and 2017 were collected from the SEER database. The Kaplan-Meier method with the log-rank test was employed to identify single prognostic factors. Multivariate Cox regression was utilized to identify independent prognostic factors. C-index, area under the ROC curve (AUC) and calibration curves were used to assess performance of the prognostic nomograms. Results: A total of 1230 patients with oral and oropharyngeal mucoepidermoid carcinoma were enrolled in the present study. After multivariate Cox regression analysis, age, sex, tumor subsite, T stage, N stage, M stage, grade and surgery were identified as independent prognostic factors for overall survival. T stage, N stage, M stage, grade and surgery were identified as independent prognostic factors for disease-specific survival. Nomograms were constructed to predict the overall survival and disease-specific survival based on the independent prognostic factors. The fitted nomograms possessed excellent prediction accuracy, with a C-index of 0.899 for OS prediction and 0.893 for DSS prediction. Internal validation by computing the bootstrap calibration plots, using the validation set, indicated excellent performance by the nomograms. Conclusion: The prognostic nomograms developed, based on individual clinicopathological characteristics, in the present study, accurately predicted the overall survival and disease-specific survival of patients with oral and oropharyngeal mucoepidermoid carcinoma.


2021 ◽  
Vol 9 (3) ◽  
pp. e001259
Author(s):  
Shanying Gui ◽  
W Quinn O'Neill ◽  
Theodoros N Teknos ◽  
Quintin Pan

BackgroundPatients with human papillomavirus (HPV+) head and neck squamous cell carcinoma (HNSCC) have superior prognoses compared with patients with HPV− HNSCC and strategies for treatment de-escalation are under investigation for the HPV+ setting. However, the survival advantage associated with HPV is not universal, and a subset of patients with HPV+ HNSCC fail definitive treatment and progress with metastatic/recurrent disease. Currently, no biomarker is available to distinguish aggressive from indolent HPV+ HNSCC. Immune dysfunction facilitates tumorigenesis and is associated with poor treatment response; therefore, we hypothesized that diminished intratumoral immune cell functionality may be attractive biomarkers to identify patients with HPV+ HNSCC at risk for early disease-specific mortality.MethodsThis is a retrospective analysis of The Cancer Genome Atlas (TCGA) HPV+ HNSCC cohort.ResultsImmunoglobulin J polypeptide (IGJ), uniquely expressed in plasma cells, showed a broad expression range in HPV+ HNSCC. Cox regression model, adjusting for clinical covariates, indicated that IGJ is an independent prognostic biomarker for disease-specific survival (DSS) and overall survival (OS). Patients with low IGJ had a 7.2-fold (p<0.001) increase in risk of disease-specific death with a median DSS of 13 months. Low IGJ showed an area under curve (AUC) of 0.89 with 91.0% sensitivity and 87.6% specificity to identify early disease-specific mortality (defined as DSS ≤12 months). Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis revealed a global dampening of immune pathways in low IGJ tumors.ConclusionsOur work showed that IGJ is a robust and independent prognostic biomarker for disease-specific mortality in HPV+ HNSCC. Patient with HPV+ HNSCC with limited adaptive immune functionality should not be candidates for treatment de-escalation modalities.


2016 ◽  
Vol 115 (5) ◽  
pp. 860-867 ◽  
Author(s):  
Jonathan M. Hodgson ◽  
Richard L. Prince ◽  
Richard J. Woodman ◽  
Catherine P. Bondonno ◽  
Kerry L. Ivey ◽  
...  

AbstractHigher fruit intake is associated with lower risk of all-cause and disease-specific mortality. However, data on individual fruits are limited, and the generalisability of these findings to the elderly remains uncertain. The objective of this study was to examine the association of apple intake with all-cause and disease-specific mortality over 15 years in a cohort of women aged over 70 years. Secondary analyses explored relationships of other fruits with mortality outcomes. Usual fruit intake was assessed in 1456 women using a FFQ. Incidence of all-cause and disease-specific mortality over 15 years was determined through the Western Australian Hospital Morbidity Data system. Cox regression was used to determine the hazard ratios (HR) for mortality. During 15 years of follow-up, 607 (41·7 %) women died from any cause. In the multivariable-adjusted analysis, the HR for all-cause mortality was 0·89 (95 % CI 0·81, 0·97) per sd (53 g/d) increase in apple intake, HR 0·80 (95 % CI 0·65, 0·98) for consumption of 5–100 g/d and HR 0·65 (95 % CI 0·48, 0·89) for consumption of >100 g/d (an apple a day), compared with apple intake of <5 g/d (Pfor trend=0·03). Our analysis also found that higher apple intake was associated with lower risk for cancer mortality, and that higher total fruit and banana intakes were associated lower risk of CVD mortality (P<0·05). Our results support the view that regular apple consumption may contribute to lower risk of mortality.


2021 ◽  
Vol 34 (Supplement_1) ◽  
Author(s):  
Arasteh Reyhani ◽  
Jason M Dunn ◽  
Aida Santaolalla ◽  
Janine Zylstra ◽  
Eliza Gimson ◽  
...  

Abstract   To assess the outcomes of patients with early esophageal cancer (EEC) and high-grade dysplasia (HGD) comparing esophagectomy, the historical treatment of choice, to endoscopic eradication therapy (EET). Methods Retrospective cohort study of consecutive patients with EEC/HGD, treated between 2000 and 2018 at a tertiary referral centre. Primary outcomes were all-cause and disease-specific mortality assessed by multivariable Cox regression and a propensity score matching sub analysis, providing hazard ratios (HR) with 95% confidence intervals (CIs), adjusted for age, tumor grade (G1/2 vs G3), tumor stage and lymphovascular invasion(LVI). Secondary outcomes included complications, hospital stay, and overall costs. Results Among 269 patients, 133 underwent esophagectomy and 136 received endoscopic mucosal resection (EMR)+/−further EET. Adjusted survival analysis showed no difference between groups regarding all-cause mortality (HR 1.85,95%CI 0.73,4.72) and disease-specific mortality (HR 1.10,95%CI 0.26,4.65). In-hospital and 30-day mortality was 0% in both groups. The surgical group had a significantly higher rate of complications (Clavien-Dindo ≥3 26.3% vs EMR 0.74%), longer in-patient stay (median 14 vs 0 days EMR) and higher hospital costs ($20.426 vs $10.988 per patient). Conclusion This series of patients with EEC/HGD treated during a transition period from surgery to EET, demonstrates a primary endoscopic approach does not compromise oncological outcomes with the benefit of fewer complications, shorter hospital stays, and lower costs compared to surgery. EET should be available as the gold standard treatment for patients with early esophageal cancer. Those with adverse prognostic features may still benefit from esophagectomy.


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