scholarly journals Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology

Sarcoma ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-10
Author(s):  
Inna Schott ◽  
Sven-Thorsten Liffers ◽  
Farhad Farzaliyev ◽  
Johanna Falkenhorst ◽  
Hans-Ulrich Steinau ◽  
...  

Background. Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology. Methods. We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan–Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions. Results. The median age was 67 years (19–72.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size <10 cm ( p  = 0.009), negative surgical margins ( p  = 0.021), and negative lymph node status ( p  = 0.007). LRFS and MFS were longer for tumor size <10 cm ( p  = 0.012 and p  = 0.013). In multivariate analyses, age <70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; p  = 0.035). Conclusions. Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.

2020 ◽  
Vol 8 ◽  
pp. 2050313X2094431
Author(s):  
Diandra Perez ◽  
Ola El-Zammar ◽  
Brando Cobanov ◽  
Rana Naous

Low-grade fibromyxoid sarcoma, also known as Evans tumor, is a low-grade sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 10529-10529
Author(s):  
Daniel Orbach ◽  
Veronique Mosseri ◽  
Daniel Pissaloux ◽  
Bernadette Brennan ◽  
Andrea Ferrari ◽  
...  

10529 Background: A genomic index (GI) tool using array comparative genomic hybridization (aCGH) on tumor cells has recently been developed, and shown a high prognostic value in adult soft tissue sarcomas. GI correlates with genomic instability, and has emerged as independent prognostic factor associated with the risk of metastases developing in synovial sarcoma (SS). The aim, therefore, was to assess GI in pediatric patients with SS, to assess its value as a prognostic factor and its role in risk stratification. Methods: All pediatric/adolescent/young adults’ (<25 years) with localized SS prospectively included in the European EpSSG-NRSTS05 protocol with a contributive aCGH were selected. Tumors had a central pathological review or harbored the specific fusion transcript (SYT-SSX). Definition of GI was A2/C, where A is the total number of alterations (segmental gains and losses) and C is the number of involved chromosomes on aCGH results. GI1 group corresponds to cases with no or few alterations (flat profile, GI≤1) and GI2 group cases with many alterations (complex CGH profile; GI>1). Results: A total of 48 patient’s samples were available. The median age of the cohort was 13 years (range: 4-24). Patients received surgery only (19%), with adjuvant therapy (17%) or perioperative therapy (64%). GI1 group corresponded to 54.2%, and GI2 to 45.8%. After a median follow up of 58 months (range: 0.1-107), 10 tumor events occurred and 5 patients died. Patients with high GI have more axial (P<0.01), invasive (P=0.04) and higher therapeutic risk groups’ tumors (unresectable/axial tumors; P<0.015). Respectively for GI1 vs. GI2 groups, 5-year event free survival (EFS) rates were 91.8±5.6% vs. 58.9±11.2% (P<0.0084) and 5Y-Metastatic Free Survival 91.8±5.5% vs. 68.6±10.6% (P=0.055). In multivariate analysis, GI adjusted for IRS groups, site and tumor size remains prognostic for EFS (P<0.025). Conclusions: Although tumor events were rare for SS in NRSTS 2005, high GI selected patients with high risk tumor features and predicted a poorer outcome. GI may explain aggressive behavior of some pediatric SS. Founding sources: “Enfant-et-santé/SFCE,” “Info sarcome,” and “La ligue contre le cancer.”


2020 ◽  
pp. 1-8
Author(s):  
Sunyach Marie ◽  
Severine Prapan ◽  
Aurelie Bertaut ◽  
Marie Karanian ◽  
Gualter Vaz ◽  
...  

Background and Purpose: Limb sparing surgery and radiotherapy is the main treatment of patients harboring soft tissue sarcoma of the extremity. There is limited data regarding the prognostic impact of histologic response after pre-operative radiotherapy. Patients and Methods: Between 2010 and 2018, 123 patients were treated with a pre-operative radiotherapy for soft tissue sarcoma at Leon Berard Centre (Lyon, France) and were retrospectively reviewed. All patients received a dose of 50 Gy in 25 fractions. The histologic response has been analysed by considering the following factors: necrosis ≥ 90%, percentage of viable tumor cells ≤ 10% and fibrosis ≥ 10%. Overall survival (OS), local recurrence-free survival (LRFS), distant recurrence-free survival (DRFS) and event-free survival (EFS) were evaluated. Results: Median follow up was 33.2 months (range 2.3-128.1 months). Local recurrence occurred in 9 patients (7.5%) and 40 patients (33%) presented a distant recurrence. The 2 and 5-year OS was 84% and 63%. Histologic response factors (necrosis ≥ 90%, viable tumor cells ≤ 10% and fibrosis ≥ 10%) were not predictive in DRFS and EFS. In multivariate analysis, grade was the only significant prognostic factor for EFS P=0.0087. Among the 14 patients with ≤ 10% viable cells after irradiation 13 presented a metastatic evolution within 6 months. Conclusion: This study showed that current histological response evaluation based on necrosis, fibrosis and viable cells could not predict clinical outcomes after radiotherapy for extremity soft tissue sarcoma. A significant proportion of patients with a good response after pre-operative radiotherapy present a metastatic recurrence.


2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e12023-e12023
Author(s):  
Mohamed Salah Fayaz ◽  
Gerges Attia Demian ◽  
Mustafa El-Sherify ◽  
Sadeq Abuzalouf ◽  
Thomas George ◽  
...  

e12023 Background: Young age is a known independent poor prognostic factor for breast cancer. Few data exist about validating such prognostic factor in triple negative subtype of breast cancer. In this study, we evaluate the prognostic value of young age presentation in triple negative breast cancer (TNBC) patients who were diagnosed in Kuwait Cancer Control Center. Methods: This is a retrospective analysis of 363 patients diagnosed with TNBC between July 1999 and June 2009. Of these, 27% were diagnosed at or below the age of 40. Chi-square test was used to correlate the age with other prognostic factors. Survival measurements were estimated using Kaplan-Meier analysis. Statistical significance was calculated using the log-rank test. Results: There was no correlation between young age at presentation and other prognostic factors including grade, T stage, lymph node status, lymphovascular invasion, and Ki67 positivity. Similarly, young age was not statistically associated with poorer 5-years overall survival (78% for patients < 40 years compared to 72% for those > 40 years; p = 0.13), disease free survival (66% vs. 61%; p = 0.5) or locoregional recurrence free survival (81% vs. 83%; p = 0.7). Conclusions: Young age does not seem to negatively impact the survival of TNBC patients nor associated with poor prognostic factors in our study population. Further studies are needed to define new prognostic factors, e.g. molecular markers, in this subtype of patients rather than the conventional clinicopathologic prognostic factors.


1988 ◽  
Vol 24 (10) ◽  
pp. 1583-1588 ◽  
Author(s):  
Wlodzimierz Ruka ◽  
Lawrence J. Emrich ◽  
Deborah L. Driscoll ◽  
Constantine P. Karakousis

2002 ◽  
Vol 12 (1) ◽  
pp. 32-41 ◽  
Author(s):  
M Graflund ◽  
B Sorbe ◽  
A Hussein ◽  
M Bryne ◽  
M Karlsson

Abstract.Graflund M, Sorbe B, Hussein A, Bryne M, Karlsson M.The purpose of this study was to investigate the prognostic importance of clinical and histopathologic factors, including malignancy grading systems (MGS), partial index (PI), invasive front grading (IFG), and microvessel density. A complete geographic series of 172 early stage (FIGO I–II) cervical carcinomas treated by Wertheim-Meigs surgery during the period 1965–1990 was studied. The patients were followed up for at least 10 years. Significant prognostic factors for disease-free survival were lymph node status (P < 0.0000001), radical surgical margins (P = 0.00003), and tumor size (P = 0.008). In a multivariate Cox analysis it was shown that lymph node status was the single most important prognostic factor with regard to disease-free survival. The total MGS and the PI scores were highly significantly (P = 0.0001) associated with pelvic lymph node metastases and disease-free survival rate in squamous cell carcinomas. The MGS and the PI systems were superior to the IFG system in predicting lymph node metastases. The total IFG score was also a statistically highly significant (P = 0.003) prognostic factor with regard to disease-free survival in both univariate and multivariate analyses. Microvessel density was a nonsignificant prognostic factor. There was a highly significant (P = 0.002) association between vascular space invasion of tumor cells and the presence of lymph node metastases. In conclusion, histopathologic malignancy grading systems provide valuable prognostic information in patients with early stage squamous cell carcinomas of the uterine cervix.


2014 ◽  
Vol 47 (02) ◽  
pp. 259-262 ◽  
Author(s):  
Shalaka Indap ◽  
Manjiri Dasgupta ◽  
Nilay Chakrabarti ◽  
Abhishek Agarwal

ABSTRACTA low-grade fibromyxoid sarcoma is a rare soft tissue tumour that has a tendency to develop in the deep soft tissue of young adults with potential for local recurrence or distant metastasis. We describe a 40-year-old female patient with a low-grade fibromyxoid sarcoma of the shoulder that had been excised twice in the past and then had recurred after a few months. A wide resection of this tumour with flap reconstruction was performed followed by radiation to the area. The patient had no evidence of local recurrence or distant metastasis at 2 years after surgery.


1996 ◽  
Vol 14 (5) ◽  
pp. 1679-1689 ◽  
Author(s):  
P W Pisters ◽  
D H Leung ◽  
J Woodruff ◽  
W Shi ◽  
M F Brennan

PURPOSE To identify specific independent adverse clinicopathologic factors for event-free survival in a cohort of consecutively treated patients with extremity soft tissue sarcomas. PATIENTS AND METHODS Prospectively collected data from a population of 1,041 adult patients with localized (American Joint Committee on Cancer [AJCC] stage IA to IIIB) extremity soft tissue sarcomas were analyzed. Patients were treated at a single institution between 1982 and 1994. Patient, tumor, and pathologic factors were analyzed by univariate and multivariate techniques to identify independent prognostic factors for the end points of local recurrence, distant recurrence, disease-specific survival, and post-metastasis survival. RESULTS The 5-year survival rate for this cohort of patients was 76%, with a median follow-up time of 3.95 years. Significant independent adverse prognostic factors for local recurrence were age greater than 50 years, recurrent disease at presentation, microscopically positive surgical margins, and the histologic subtypes fibrosarcoma and malignant peripheral-nerve tumor. For distant recurrence, intermediate tumor size, high histologic grade, deep location, recurrent disease at presentation, leiomyosarcoma, and nonliposarcoma histology were independent adverse prognostic factors. For disease-specific survival, large tumor size, high grade, deep location, recurrent disease at presentation, the histologic subtypes leiomyosarcoma and malignant peripheral-nerve tumor, microscopically positive surgical margins, and lower extremity site were adverse factors. For post-metastasis survival, only large tumor size ( > 10 cm) was an adverse prognostic factor. CONCLUSION The independent adverse prognostic factors for distant recurrence and disease specific survival differ from those identified for subsequent local recurrence. Patients with microscopically positive surgical margins or patients who present with locally recurrent disease are at increased risk for subsequent local recurrence and tumor-related mortality. Specific histopathologic subtypes are associated with increased risks for local failure and tumor-related mortality.


2021 ◽  
Vol 11 ◽  
Author(s):  
Zhenhua Lu ◽  
Rui Li ◽  
Xianglong Cao ◽  
Chengyu Liu ◽  
Zhen Sun ◽  
...  

BackgroundRecent studies have shown that the systemic inflammation and nutritional indicators are prognostic for a variety of malignancies. However, only limited data have so far demonstrated their usefulness in gastrointestinal mesenchymal tumors (GIST).MethodsWe retrospectively analyzed the data of GIST patients who underwent radical surgery in Beijing hospital from October 2004 to July 2018. The area under the receiver operating characteristic curve (AUC) was used to compare several commonly used inflammatory and nutritional indicators. The indicators with largest AUC were further analysis. Optimal cut-off values of those indicators in predicting recurrence-free survival (RFS) were determined. Kaplan-Meier curve and the time-dependent receiver operating characteristic (ROC) curve were used to assess the prognostic values. We then used univariate and multivariate Cox regression analyses to identify prognostic factors that were associated with RFS.ResultsIn total, 160 patients who underwent surgery for GIST were included in the study. The median survival time was 34.5 months, with 1-, 3-, and 5-year RFS rates of 96.1%, 84.7%, and 80.8%, respectively. The inflammatory and nutritional indicators with largest AUC were Systemic immunoinflammatory Index (SII) and Geriatric Nutrition Risk Index (GNRI), reached 0.650 and 0.713, respectively. The optimal cutoff of GNRI and SII were 98.3, and 820.0, respectively. Univariate analysis showed that GNRI, SII, KI67, surgery method, tumor location, tumor size, and mitotic index were all significant prognostic indicators of RFS. After multivariate Cox analysis, independent prognostic factors for RFS in GIST included tumor location, mitotic index, tumor size, and GNRI (HR=2.802,95% CI: 1.045 to 7.515, p = 0.041). Besides, SII also tended to be associated with RFS (HR = 2.970, 95% CI: 0.946 to 9.326, p = 0.062).ConclusionsHigh GNRI is an independent prognostic factor for RFS in GIST, while SII can be considered as a prognostic factor. GNRI and SII can be used as tools to evaluate the prognosis of patients before surgery, helping doctors to better treat high-risk patients.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 19513-19513
Author(s):  
A. Aykanat ◽  
A. Yoney ◽  
M. Unsal ◽  
O. Kandemir ◽  
E. Berberoglu ◽  
...  

19513 Background: Postoperative radiotherapy results with higher local rates than surgery alone. In this trial we have evaluated and studied factors that affects local recurrence rates. Methods: A retrospective clinical study of 125 eligible patients with soft tissue sarcoma diagnosis from SB Okmeydani Educational Hospital-Oncology Department treated between 1998–2002 years was performed. Tumor size, surgery margin histopathologic grade and type were evaluated. At subgroup analysis local recurrense rates were studied in groups with or without postoperative radiotherapy. Results: In 125 patients, 70 were tumor free, 45 were tumor positive or with suspicious margin after the surgery. Subgroup of 25 patients which have tumor size smaller than 5 cm and negative margin had no radiotherapy and had no local recurrens. In an other group of 45 patients which have tumor size larger than 5 cm and tumor negative after excision 18 patients did not have any postoperative radiotherapy and 4 of them (% 22) had local failure. The rest of the 27 patients of this group who had postoperatif radiotherapy had 2 (% 7) local failure. Among 55 patients with positive or suspicious margins; 18 had no postoperative radiotherapy and there were 6 (% 33) local recurrens while the 37 patients with radiotherapy had 7 (% 18) local recurrens rates. Conclusions: In operable soft tissue sarcomas tumor size, hystopathological grade, postoperatif surgery margins are the most important factors which affects local recurrens rates. [Table: see text] No significant financial relationships to disclose.


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