Prognostic Factors for Children and Adolescents With Surgically Resected Nonrhabdomyosarcoma Soft Tissue Sarcoma: An Analysis of 121 Patients Treated at St Jude Children's Research Hospital

1999 ◽  
Vol 17 (12) ◽  
pp. 3697-3705 ◽  
Author(s):  
Sheri L. Spunt ◽  
Catherine A. Poquette ◽  
Yasmeen S. Hurt ◽  
Alvida M. Cain ◽  
Bhaskar N. Rao ◽  
...  

PURPOSE: The rarity and heterogeneity of pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) has precluded meaningful analysis of prognostic factors associated with surgically resected disease. To define a population of patients at high risk of treatment failure who might benefit from adjuvant therapies, we evaluated the relationship between various clinicopathologic factors and clinical outcome of children and adolescents with resected NRSTS over a 27-year period at our institution. PATIENTS AND METHODS: We analyzed the records of 121 consecutive patients with NRSTS who underwent surgical resection between August 1969 and December 1996. Demographic data, tumor characteristics, treatment, and outcomes were recorded. Univariate and multivariate analyses of prognostic factors for survival, event-free survival (EFS), and local and distant recurrence were performed. RESULTS: At a median follow-up of 9.2 years, 5-year survival and EFS rates for the entire cohort were 89% ± 3% and 77% ± 4%, respectively. In univariate models, positive surgical margins (P = .004), tumor size ≥ 5 cm (P < .001), invasiveness (P = .002), high grade (P = .028), and intra-abdominal primary tumor site (P = .055) adversely affected EFS. All of these factors except invasiveness remained prognostic of EFS and survival in multivariate models. Positive surgical margins (P = .003), intra-abdominal primary tumor site (P = .028), and the omission of radiation therapy (P = .043) predicted local recurrence, whereas tumor size ≥ 5 cm (P < .001), invasiveness (P < .001), and high grade (P = .004) predicted distant recurrence. CONCLUSION: In this largest single-institution analysis of pediatric patients with surgically resected NRSTS, we identified clinicopathologic features predictive of poor outcome. These variables should be prospectively evaluated as risk-adapted therapies are developed.

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.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 11040-11040
Author(s):  
Giuseppe Badalamenti ◽  
Lorena Incorvaia ◽  
Antonio Galvano ◽  
Giovanni Manfredi Assanto ◽  
Emmanuela Musso ◽  
...  

11040 Background: The majority of GIST patients with advanced disease initially achieves disease control from imatinib treatment. Approximately 10% of patients progresses within 6 months of starting therapy (primary resistance) and also 50-60% of the responding patients develops progression disease within two years (secondary resistance). Progression disease (PD) can be numerical, dimensional or mixed. The known prognostic factors of risk stratification in local disease are tumor size, mitotic activity and anatomic site. In this retrospective analysis we explore several clinical factors affecting survival in metastatic setting. Methods: The population included in this large database of 128 patients with metastatic GIST was obtained examining data collected from four Oncologic Centres with expertise for the GIST management. The clinical factors analyzed were sex, tumor size, mitotic activity, anatomic site, KIT and PDGFRa mutational status, site of metastasis, FDG-PET status at progressing disease and pattern of tumor progression to I line imatinib 400, II line Imatinib 800 or Sunitinib, evaluated with CT scan or MRI: PD with dimensional growth (dimensional, dPD), with new lesions appearance (numerical, nPD) and with both numerical and dimensional growth (Mixed, mPD). Every factor has been correlated with Overall Survival (OS) measured in months. Survival analyses were performed by using the Kaplan-Meier method. Univariate and multivariate Cox proportional hazard regression models were executed to search for association with the outcome. Results: Univariate analysis showed significant value for primary tumor site (p < 0.0001), mitotic activity (p = 0.02), tumor size (p = 0.05) and PD pattern (p = 0.008): OS nPD group was 102.7 months, in dPD group 87 and in mPD group 70. The multivariate analysis confirm significant prognostic factors for OS tumor site (p = 0.0004) and PD pattern (p = 0.02). Conclusions: with the limitations of a retrospective analisys, this study shows for the first time the impact of pattern of progression on OS: patients with dPD have a worse prognosis than those with nPD or mPD, suggesting type of PD as an independent prognostic factor for OS in advanced GISTs.


2008 ◽  
Vol 26 (18) ◽  
pp. 3063-3072 ◽  
Author(s):  
James C. Yao ◽  
Manal Hassan ◽  
Alexandria Phan ◽  
Cecile Dagohoy ◽  
Colleen Leary ◽  
...  

Purpose Neuroendocrine tumors (NETs) are considered rare tumors and can produce a variety of hormones. In this study, we examined the epidemiology of and prognostic factors for NETs, because a thorough examination of neither had previously been performed. Methods The Surveillance, Epidemiology, and End Results (SEER) Program registries were searched to identify NET cases from 1973 to 2004. Associated population data were used for incidence and prevalence analyses. Results We identified 35,618 patients with NETs. We observed a significant increase in the reported annual age-adjusted incidence of NETs from 1973 (1.09/100,000) to 2004 (5.25/100,000). Using the SEER 9 registry data, we estimated the 29-year limited-duration prevalence of NETs on January 1, 2004, to be 9,263. Also, the estimated 29-year limited-duration prevalence in the United States on that date was 103,312 cases (35/100,000). The most common primary tumor site varied by race, with the lung being the most common in white patients, and the rectum being the most common in Asian/Pacific Islander, American Indian/Alaskan Native, and African American patients. Additionally, survival duration varied by histologic grade. In multivariate analysis of patients with well-differentiated to moderately differentiated NETs, disease stage, primary tumor site, histologic grade, sex, race, age, and year of diagnosis were predictors of outcome (P < .001). Conclusion We observed increased reported incidence of NETs and increased survival durations over time, suggesting that NETs are more prevalent than previously reported. Clinicians need to be become familiar with the natural history and patterns of disease progression, which are characteristic of these tumors.


2008 ◽  
Vol 109 (Supplement) ◽  
pp. 77-86 ◽  
Author(s):  
Daniel W. Golden ◽  
Kathleen R. Lamborn ◽  
Michael W. McDermott ◽  
Sandeep Kunwar ◽  
William M. Wara ◽  
...  

Object The authors conducted a study to determine whether prognostic factors and the applicability of prognostic systems vary by primary tumor site in patients treated with radiosurgery for brain metastases. Methods The authors evaluated data obtained in patients who underwent radiosurgery with or without whole-brain radiotherapy (WBRT) from 1991 to 2005 for newly diagnosed brain metastases. Four groups were analyzed: 1) all primary sites combined, 2) breast, 3) lung, and 4) melanoma primary sites. Kaplan–Meier, log-rank, Cox proportional hazard uni- and multivariate analysis, and recursive partitioning analysis (RPA) were used to assess prognostic factors and 4 prognostic systems: Radiation Therapy Oncology Group (RTOG) RPA, Graded Prognostic Assessment (GPA), basic score for brain metastases (BSBM), and the newly proposed Golden grading system (GGS). The GGS divides patients into 4 prognostic groups by age ≥ 65 years, Karnofsky Performance Scale (KPS) score < 70, and known presence of extracranial metastases. Results Data acquired in 479 newly diagnosed patients with 1664 lesions were analyzed. The median survival time from diagnosis of brain metastases was 12.1 months; the median follow-up was 25.4 months in 73 patients who were censored. Survival and prognostic factors were equivalent for 369 patients treated with radiosurgery compared with 110 patients treated with radiosurgery and WBRT, so these subsets were combined. Multivariate analysis of all primary sites combined demonstrated age < 65 years, KPS score ≥ 70, no known extracranial metastases, and ≤ 3 brain metastases were associated with longer survival, and primary tumor control was not. In subgroup multivariate analysis of patients with breast, lung, or melanoma primaries, favorable factors included only primary tumor control in 87 patients with breast primary; age < 65 years, no known extracranial metastases, and ≤ 3 brain metastases in 169 patients with lung primary; and KPS ≥ 70 years, primary tumor control, and ≤ 3 brain metastases in 137 patients with melanoma primary. The median survival for ≤ 3 versus > 3 metastases was 15.6 and 16.9 months, respectively, for breast, 16.5 and 11.3 months for lung, and 9.0 and 5.7 months for melanoma. Analysis of the 4 prognostic systems (RTOG RPA, BSBM, GPA, and GGS) showed that each prognostic system's clinical applicability varied depending on primary tumor site. The RPA confirmed that GGS and primary tumor site are significant variables for prognosis. Conclusions Favorable prognostic factors for patients with newly diagnosed brain metastases treated with radiosurgery vary by primary site. The 4 prognostic grading systems analyzed were applicable to different primary sites depending on which prognostic factors each individual system incorporated. Therefore, the authors recommend further development and use of primary-specific prognostic systems.


2021 ◽  
Vol 8 ◽  
Author(s):  
Shuo Liu ◽  
Xiaoqiang Liu ◽  
Weiduan Zhuang

Background: Diffuse astrocytoma (DA) is a rare disease with inadequately understood epidemiological characteristics and prognosis. Identification of the factors associated with the survival in DA patients is therefore necessary. In this study, we aim to investigate the clinicopathological characteristics of DA to delineate factors influencing the survival of DA.Methods: A population-based cohort study was conducted, utilizing prospectively extracted data from the Surveillance, Epidemiology and End Results (SEER) database. Patients with histological diagnosis of DA in the SEER database from 1973 to 2017 were included.Results: A total of 799 participants with DA were included, consisting of 95.9% fibrillary astrocytoma and 4.1% protoplasmic variants. The average age of participants was 41.9 years, with 57.2% being male. The majority of the population was white (87.5%). More than half (53.9%) of the patients were married. DA arose mostly in the cerebrum (63.8%). Around 71.6% of the population had received surgical treatment. The overall 1-, 3-, 5-, and 10-year survival rate were 73.7, 55.2, 49.4, and 37.6%, respectively. Kaplan–Meier analysis showed that age at diagnosis, marital status, primary tumor site, tumor size, and surgery was possibly associated with cancer-specific survival (CSS) (p &lt; 0.05). Multivariate Cox proportional hazard analysis indicated that surgery was a protective factor whereas older age, larger tumor size, and tumor in the brainstem were harmful factors for patients with DA. Moreover, a nomogram predicting 5- and 10-year survival probability for DA was developed.Conclusions: Age, primary tumor site, tumor size, and surgery were associated with the survival of patients with DA.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10040-10040
Author(s):  
Gerlinde Egerer ◽  
Patrick Wuchter ◽  
Sascha Dietrich ◽  
Johann Wilhelm Schmier ◽  
Anthony D Ho ◽  
...  

10040 Background: Soft tissue sarcoma (STS) represent a group of rare malignant tumors. Survival for patients with disseminated disease is dismal. Gemcitabine/docetaxel (GD) is a commonly used systemic 2°-line regime in this setting. Methods: Here we report on our single center experience of GD (G 900 mg/m² or 675 mg/m² d1+8 and D 100 mg/m² d8) in STS. Patients were retrospectively analysed. Restaging according to RECIST criteria of primary tumor site and metastases was performed every 3 cycles or on clinical progression. Progression-free (PFS) and overall survival (OS) were estimated using the method of Kaplan and Meier. Results: Between 2005 and 2011, 34 STS patients (male=20, female=14, median age 59 years [range 32-75]) were treated with GD at our institution. Histological subtypes comprised leiomyo- (n=13), lipo- (n=7), pleomorphic- (n=6), rhabdomyo- (n=3), synovial sarcoma (n=2), and other subtypes (n=3) with tumor grades being G1 (n=1), G2 (n=9) and G3 (n=24). Primary tumor sites included extremities (n=19), abdomen/retroperitoneum (n=10), trunk (n=3), and others (n=2). Metastases were found in lung (n=26), soft tissue/solid organs (n=12), bone (n=8) and lymph nodes (n=5). Patients had received no (n=2), 1 (n=22), 2 (n=9) or 3 (n=1) previous lines of CTX. A total number of 158 cycles was administered with a median number of 6 cycles (range 1-6) per patient. Best response by RECIST criteria after 3 cycles of treatment was PR (n=2, 6%). Disease stabilisation (SD) was achieved in further 22 subjects, resulting in a clinical benefit rate (CBR) of 70%. Median PFS and OS for the whole population were 7.7 and 15.3 months, respectively. PFS at 6 months was 62%. Patients responding to therapy had a significantly longer median PFS with 8.6 months (p <0.0001; HR 33.1) and OS with 22.5 months (p<0.0001; HR 20.2). Major toxicities included hand-foot syndrome (n=10), febrile neutropenia (n=7), mucositis (n=7), oedema (n=5), hematological toxicity (n=4) and polyneuropathy (n=3), leading to dose reductions in 10 patients. Conclusions: GD is an active regimen in STS with tolerable side effects. CBR was 70% after 3 cycles. Patients achieving at least SD had a significantly prolonged PFS and OS.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 11054-11054
Author(s):  
Mudit Chowdhary ◽  
Akansha Chowdhary ◽  
Neilayan Sen ◽  
Nicholas George Zaorsky ◽  
Kirtesh R. Patel ◽  
...  

11054 Background: Large, high-grade extremity/trunk (ET) non-rhabdomyosarcoma soft-tissue sarcoma (STS) is at high risk for distant recurrence and death. The integration of chemotherapy (C) to standard of care neoadjuvant radiotherapy (RT) remains controversial, even for these patients. This study examines the impact of adding C to neoadjuvant RT on overall survival (OS) in high risk ET-STS. Methods: The National Cancer Data Base (NCDB) was queried for patients ≥18 years with high risk (≥5 cm + high grade) non-rhabdomyosarcoma ET-STS (WHO histology) who received neoadjuvant RT and limb sparing surgery from 2006-2014. Patients were next stratified based upon receipt of C (RT and CRT cohorts). Overall survival (OS) for RT vs CRT cohorts was analyzed using the Kaplan-Meier (KM) method, log-rank test, and Cox proportional hazards models. Propensity score-matched analysis (PSM) was employed to account for potential treatment selection bias between cohorts. Results: A total of 848 (71.1%) and 344 (28.9%) patients received RT and CRT, respectively. Patient cohorts were well-balanced except for the CRT cohort having higher rates of treatment in the West (22.1% vs 10.6%) & Midwest (28.3% vs 22.7%), Charlson-Deyo [CD] score 0 vs ≥1 (85.5% vs 79.4%), younger age (≤50) (45.9% vs 21.7%), synovial sarcoma histology (18.9% vs 3.2%), earlier year of diagnosis (2006-2010) (39.5% vs 32.3%), and positive lymphovascular invasion (2.0 vs 1.51%), (p < 0.05 each). The KM 5-year OS was significantly higher in the CRT vs RT cohort: 69.2% vs 58.1% on univariate (p < 0.0001) and multivariate analysis (Hazard Ratio [HR]: 0.66; 95% Confidence Interval [CI]: 0.52-0.85; p = 0.001) even after adjusting for age, race, income, CD score, histology, tumor size, tumor grade, and primary site (lower extremity; upper extremity; trunk). PSM identified evenly matched cohorts of 300 patients each with respect to age, income, CD score, histology, grade, tumor size, and primary site. The addition of neoadjuvant C remained prognostic for OS on PSM (HR: 0.74 [0.56-0.99], p = 0.042). Conclusions: The addition of C to neoadjuvant RT was associated with improved OS in patients with high risk non-rhabdomyosarcoma ET-STS in the NCDB. These hypothesis generating results support prospective evaluation.


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