Clinical features and prognosis of patients with liposarcoma: Single-center experience.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e22557-e22557
Author(s):  
Metin Demir ◽  
Deniz Can Guven ◽  
Burak Yasin Aktas ◽  
Gürkan Güner ◽  
Oktay Halit Aktepe ◽  
...  

e22557 Background: Liposarcomas constitute 15% of all soft tissue sarcomas.They consist of four subtypes;well-differentiated,dedifferentiated,myxoid and pleomorphic.In this trial we assessed demographic and clinical features of patients with liposarcoma who were treated in our hospital.Methods: Patients who were diagnosed with liposarcoma in Hacettepe University Medical Oncology Department between 2005and2015(n = 119) were included. Patients’ data were collected from hospital registration system.Survival analyses were performed with Kaplan Meier analyses.Results: At the time of diagnosis, the vast majority of patients had localized and/or node positive disease(n = 98),8 patients had metastatic disease.Further analyses hold for only early stage disease:Median age was 52(min:18-max:81).105 patients(94.6%) had upfront surgery.26(23.4%) and 30 patients(27%) received perioperative chemotherapy(CT) and radiotherapy(RT),respectively.The most commonly used CT agents were ifosfamide, anthracyclines, etoposide,taxanes and gemcitabine.At a median follow-up of 45months, relapses were observed in 34 patients(30.6%) and 35 patients(31.5%) died.Median overall survival(OS) was 113.1 months and median relapse free survival (RFS) was 23.8 months.Perioperative CT and RT were not associated with improved RFS and OS.Among patients with early stage disease; median RFS of patients with well-differentiated, dedifferentiated, myxoid and pleomorphic subtypes were 31.8,34.3,28 and 5.2months,respectively(p = 0.013).Well-differentiated group had significantly higher RFS than those with pleomorphic liposarcomas(p = 0.003).The mostly used CT drugs in recurrent setting were ifosfamide+doxorubicine(42.9%) and ifosfamide+etoposide(21.4%).Conclusions: Liposarcoma prognosis varies significantly with histological subtype and the efficacy of systemic chemotherapy is limited both in early stage and advanced disease.[Table: see text]

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Judith E Heida ◽  
Ronald Gansevoort ◽  
Lianne Messchendorp ◽  
Esther Meijer ◽  
Niek F Casteleijn ◽  
...  

Abstract Introduction Predicting disease progression in autosomal dominant polycystic kidney disease (ADPKD) patients poses a challenge, especially in early stage disease when renal function is not yet affected. The ongoing formation and growth of cysts causes the urine concentrating capacity to decrease from early on in the disease. We therefore hypothesized that the easy and inexpensive to measure urine-to-plasma urea ratio (UPU ratio), which is assumed to be surrogate for maximal urine concentrating capacity, can be used as marker to predict disease progression in ADPKD. Methods The UPU ratio was calculated by dividing urea concentration in a fasting morning spot urine sample by plasma urea concentration adjusted for plasma creatinine concentration. First, we validated the UPU ratio in 30 ADPKD patients who underwent a prolonged water deprivation test to measure the maximal urine concentrating capacity. Thereafter, the association of the UPU ratio with renal outcome was evaluated in 583 ADPKD patients participating in the DIPAK observational cohort (inclusion criteria: age>18 years, eGFR >15 mL/min/1.73m2, no concomitant diseases affecting eGFR, without V2 receptor antagonist prescription). Kidney function was assessed as eGFR by the creatinine based CKD-EPI formula, height adjusted total kidney volume (htTKV) by MRI and copeptin (surrogate for vasopressin) by ELISA. Results In the water deprivation test participants (n=30), the UPU ratio was strongly correlated with maximal urine concentrating capacity (R = 0.67, p<0.001). This association remained significant after correcting for sex, age, htTKV and eGFR (st. β = 0.53, p = 0.007). In these subjects maximal urine concentrating capacity as well as UPU ratio were associated with the rate of eGFR decline during a median follow-up of 6.3 yr (12 eGFR assessments per patient) assessed using linear mixed modeling, also when corrected for sex, baseline age and eGFR (β = 0.009, p = 0.04, and β = 5.56, p<0.001, resp.). We subsequently corroborated in the larger DIPAK observational cohort (n=583, 58% female, mean age 47 yr median eGFR 60 mL/min/1.73m2 and htTKV 898 ml/m), that the UPU ratio was significantly associated with rate of eGFR decline during a median follow-up of 4.0 yr (6 eGFR assessments per patient): β = 0.23, p = 0.005. This association remained significant when corrected for sex, baseline age and eGFR (β = 0.32, p<0.001) and even when additionally corrected for Mayo class, PDK mutation and copeptin (β = 0.40, p <0.001). Stepwise backward multivariate regression analysis resulted in a final model including the UPU ratio, PKD mutation, Mayo Class and copeptin. Cox survival analysis showed that a lower baseline UPU ratio (indicating less urine concentrating capacity) was significantly associated with a higher risk to develop the combined renal endpoint of incidence of start of kidney replacement therapy, eGFR <15 mL/min/1.73m2 or eGFR decrease >40% during follow-up (adjusted Hazard Ratio per SD = 1.39, p = 0.007). Limiting the aforementioned analyses to the subgroup of patients with relative early stage disease (n=122, age <40 yr and eGFR >60 mL/min/1.73m2) rendered essentially similar results, with an adjusted β for UPU ratio in the final model of 0.33 (p = 0.04). In this subgroup with a limited number of events (n=10), Cox survival analysis did not reach formal significance (adjusted HR per SD: 2.67, p = 0.055). Conclusion The UPU ratio, which is calculated from routine laboratory measurements, predicts renal prognosis in ADPKD in addition to other, more laborious to measure and expensive risk markers. Notably, this marker of urine concentrating capacity also shows promise in early-stage disease.


2014 ◽  
Vol 96 (5) ◽  
pp. e18-e20 ◽  
Author(s):  
J Ahmad ◽  
AIW Mayne ◽  
Y Zen ◽  
MB Loughrey ◽  
P Kelly ◽  
...  

Introduction Incidental gallbladder cancer is found in 0.6–2.1% of patients undergoing laparoscopic cholecystectomy for symptomatic gallstones. Patients with Tis or T1a tumours generally undergo no further intervention. However, spilled stones during surgery may have catastrophic consequences. We present a case and suggest aggressive management in patients with incidental gallbladder cancer who had spilled gallstones at surgery. Case History A 37-year-old woman underwent a laparoscopic cholecystectomy for symptomatic gallstones, during which some stones were spilled into the peritoneal cavity. Subsequent histological examination confirmed incidental pT1a gallbladder cancer. Hepatopancreatobiliary multidisciplinary team discussion agreed on regular six-monthly follow-up. The patient developed recurrent pain two years after surgery. Computed tomography revealed a lesion in segment 6 of the liver. At laparotomy, multiple tumour embedded gallstones were found on the diaphragm. Histological examination showed features (akin to the original pathology) consistent with a metastatic gallbladder tumour. Conclusions This case highlights the potential for recurrence of early stage disease resulting from implantation of dysplastic or malignant cells carried through spilled gallstones. It is therefore important to know if stones were spilled during original surgery in patients with incidental gallbladder cancer following a laparoscopic cholecystectomy. Aggressive and early surgical management should be considered for these patients.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e18237-e18237
Author(s):  
Antoine Harb ◽  
Adam Curtis ◽  
Laura Skacel ◽  
Michael Babcock ◽  
Marek Skacel

e18237 Background: Non-Small Cell Lung Cancer (NSCLC) is the most common malignancy worldwide and the leading cause of malignancy-related mortality in the United States. The state of Maine in particular, has one of the highest rates of lung cancer in the country. Methods: We reviewed all NSCLC patients (adenocarcinoma (AC) and squamous cell (SC) histology) diagnosed between January 2017 and June 2018 at Northern Light Cancer Institute. 261 patients with clinical follow-up were identified. We correlated their clinical characteristics with molecular abnormalities identified by Next Generation Sequencing (NGS) and Fluorescence in situ hybridization, PD-L1 status by immunohistochemistry, disease-free and overall survival. Results: 210 patients had AC and 51 SC. They were evenly split between men and women. The median age at diagnosis was 68 years. 99% of patients were Caucasian. 15 patients were never smokers, the rest were equally divided between active and previous smokers. 44% had early stage disease (I/II) and 56% had late stage disease (III/IV) on presentation. 36.4% had a PD-L1 high status. The frequencies of the molecular aberrations identified in AC and SC are listed in the table below: Treatment differed by stage, including surgery/Radiation +/- adjuvant chemotherapy for early stage disease, definitive chemo-radiation followed by immunotherapy for stage III disease. Stage IV patients were treated with immunotherapy, combination chemo-immunotherapy, targeted therapy, palliative radiation and hospice referral. After a median follow-up of 10.6 months, overall survival (OS) was 66%. Disease free survival (DFS) was 33%. Using univariate (chi-square), multivariate (logistic regression) and Kaplan-Meier (log rank) analyses, we identified that in addition to a high clinical stage, which was associated with shorter OS and DFS, high PD-L1 status, and the presence of p53 mutation, were independent predictors of shorter OS, and p53 mutation of shorter DFS. Conclusions: NGS-based molecular testing deployed in real-time non-academic setting proved to be a valuable tool to identify therapeutic and prognostic targets in NSCLC. Besides those endorsed by the NCCN guidelines, p53 mutation is a common abnormality associated with adverse outcomes. While high PD-L1 expression is a desirable immunotherapy marker, its presence also predicted adverse overall outcomes in our patients.[Table: see text]


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4634-4634
Author(s):  
Amory V. Novoselac ◽  
Raghu K. Kunamneni ◽  
Malgorzata McMasters ◽  
Miroslav R. Radevic ◽  
Randy L. Levine

Abstract Background: PMBCL is a recognized separate entity within the group of diffuse large B-cell lymphoma (DLBCL) (Harris et al, Blood, 1994) with clinical, pathological and recently described molecular distinctiveness. It typically presents as a bulky mediastinal mass with the SVC syndrome, early stage disease, occurring predominantly in young females. The therapeutic approach has paralleled that of DLBCL consisting of anthracycline based regimens with or without the addition of radiation therapy (RT). In recent years, the use of rituximab has become part of the treatment of DLBCL, with outcome improvement demonstrated in patients >60 years of age (Coiffier et al, NEJM 2002) and recently <60 years of age (Sehn et al, ASH 2003, and Pfreundschuh et al, ASCO 2004). Though commonly used as part of the initial treatment, outcome benefit has not as of yet been established in PMBCL. Methods: Retrospective analysis of 10 patients with PMBCL diagnosed and/or treated in our institution from September 2000 to October 2003 with R-CHOP. Results: The median age was 30 (range 22–56). There were 7 female and 3 male patients. Eight patients had a low/low intermediate IPI, and all but one had an elevated LDH. Eight patients had early stage disease (I and II), two had stage III. Beta2 microglobulin was not elevated in the 6 patients for whom results were available. Two patients had B symptoms. Bulky disease (> 10 cm or > 1/3 of the thoracic diameter) was present in 7 patients; SVC syndrome was seen in 3. None of the patients had marrow involvement. Median follow up was 13 months (range 9–47). All patients received 4–6 cycles of R-CHOP. Two patients received maintenance rituximab (one received 4 weekly treatments at 6 months and the other received 4 weekly treatments at 3 and 6 months). RT was given to 6 patients. Eight patients had a complete remission (CR) following R-CHOP and two patients had near CR (> 90% reduction); they subsequently achieved CR after the RT. Conclusions: Great variations of CR rates in the treatment of PMBCL, ranging from 10 to 95% for anthracycline containing regimens have been reported in the literature. Reported CR rates for CHOP alone range from 45–80% and optimal anthracycline based therapies are still being debated. Regardless of the initial regimen, failure patterns most commonly described in PMBCL were either no response to initial treatment or early progression, typically seen within 6 to 12 months. All of our patients achieved and remain in CR with a median follow up of 13 months. We recognize the limitations of our study (i.e. short median follow up, absence of control group, heterogeneous therapeutic approaches). However, this retrospective analysis demonstrates that the addition of rituximab could potentially contribute to the initial response to CHOP in comparison to traditionally reported CR rates found in the literature. Long term follow up in a larger series is necessary for the assessment of outcome improvement with the addition of rituximab in PMBCL patients. Charcteristics of 10 PMBCL patients treated with R-CHOP Median age (yrs) (range) 30 (22–56) Female/Male 7/3 Low IPI Score (total) (%) 8 (80%) High LDH (total) (%) 9 (90%) B Symptoms (total) (%) 2 (20%) Stage I and II (total) (%) 8 (80%) Bulky Disease (total) (%) 7 (70%) SVC Syndrome (total) (%) 3 (30%) High Beta2 microglobulin (total) (%) 0 (0%) Bone Marrow Involvement (total) (%) 0 (0%) RT (total) (%) 6 (60%) CR (total) (%) 10 (100%) Median Follow up (mts) (range) 13 (9–47)


2016 ◽  
Vol 34 (33) ◽  
pp. 3984-3991 ◽  
Author(s):  
Donald R. Sullivan ◽  
Christopher W. Forsberg ◽  
Linda Ganzini ◽  
David H. Au ◽  
Michael K. Gould ◽  
...  

Purpose Depression symptoms are common among patients with lung cancer; however, longitudinal changes and their impact on survival are understudied. Methods This was a prospective, observational study from the Cancer Care Outcomes Research and Surveillance Consortium from five US geographically defined regions from September 2003 through December 2005. Patients enrolled within 3 months of their lung cancer diagnosis were eligible. The eight-item Center for Epidemiologic Studies Depression scale was administered at diagnosis and 12 months’ follow-up. The main outcome was survival, which was evaluated using Kaplan-Meyer curves and adjusted Cox proportional hazards modeling. Results Among 1,790 participants, 681 (38%) had depression symptoms at baseline and an additional 105 (14%) developed new-onset depression symptoms during treatment. At baseline, depression symptoms were associated with increased mortality (hazard ratio [HR], 1.17; 95% CI, 1.03 to 1.32; P = .01). Participants were classified into the following four groups based on longitudinal changes in depression symptoms from baseline to follow-up: never depression symptoms (n = 640), new-onset depression symptoms (n = 105), depression symptom remission (n = 156), and persistent depression symptoms (n = 254) and HRs were calculated. Using the never-depression symptoms group as a reference group, HRs were as follows: new-onset depression symptoms, 1.50 (95% CI, 1.12 to 2.01; P = .006); depression symptom remission, 1.02 (95% CI, 0.79 to 1.31; P = .89), and persistent depression symptoms, 1.42 (95% CI, 1.15 to 1.75; P = .001). At baseline, depression symptoms were associated with increased mortality among participants with early-stage disease (stages I and II; HR, 1.61; 95% CI, 1.26 to 2.04), but not late-stage disease (stages III and IV; HR, 1.05; 95% CI, 0.91 to 1.22). At follow-up, depression symptoms were associated with increased mortality among participants with early-stage disease (HR, 1.71; 95% CI, 1.27 to 2.31) and those with late-stage disease (HR, 1.32; 95% CI, 1.04 to 1.69). Conclusion Among patients with lung cancer, longitudinal changes in depression symptoms are associated with differences in mortality, particularly among patients with early-stage disease. Symptom remission is associated with a similar mortality rate as never having had depression.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2789-2789 ◽  
Author(s):  
Clive S. Zent ◽  
Susan L. Slager ◽  
Wei Ding ◽  
Karin F. Giordano ◽  
Susan M. Schwager ◽  
...  

Abstract Patients with CLL complicated by autoimmune diseases causing cytopenias (AID) are often considered to have clinically advanced disease (Rai stage III - IV, Binet C). With the use of automatic differential counts and flow cytometric immunophenotyping of peripheral blood, CLL is now most often diagnosed in asymptomatic patients with early stage disease. These changes in clinical practice could have an impact on clinical presentation and prognosis of CLL patients with AID. To more fully define AID complicating CLL, we conducted an observational study of a large cohort of CLL patients. Methods All 1737 patients with chronic B cell lymphoproliferative diseases seen in the Division of Hematology at Mayo Clinic Rochester from January 1, 1995 to December 30, 2004 were included. Lymphoid malignancies were classified using WHO and modified NC-WG 1996 criteria (CLL = 1649, “atypical” CLL = 4, leukemic phase of lymphoma = 9, chronic B cell lymphoproliferative disorders (not otherwise classified) = 75). Autoimmune hemolytic anemia (AIHA) was defined as a Hgb ≤ 10 g/dL with at least one appropriate marker of hemolysis (elevated indirect bilirubin, LDH, or reticulocyte count; or increased bone marrow erythropoiesis without bleeding) and evidence of an autoimmune mechanism (positive DAT, cold agglutinins), or at least 2 markers of hemolysis in absence of evidence of bleeding or hypersplenism. Immune thrombocytopenia (ITP) was defined as a platelet count of ≤100 x 109/L with normal/ increased bone marrow (BM) megakaryocytes or normal/increased absolute reticulocyte count. Pure red blood cell aplasia (PRBCA) was defined as a Hgb ≤ 10 g/dL, reticulocytopenia, and an isolated absence of BM erythrocyte precursors. Autoimmune granulocytopenia (AIG) was defined as sustained neutropenia (< 0.5 x 109/l) in the absence of chemotherapy for at least 8 weeks and with decreased/absent BM granulocyte precursors. Results Seventy four (4.5%) CLL patients had AID (78% male, median age at diagnosis 67 yr). The diagnosis of AID preceded CLL in 12% and was concomitant with the diagnosis of CLL in 15% of patients. Of the CLL patients with AID, 55% had AIHA, 47% ITP, 10% PRBCA and 4% AIG; 10% had coincident AIHA and ITP (Evans syndrome). Most AIHA (74%) patients presented with symptomatic anemia but 68% of the ITP patients were asymptomatic at diagnosis. Forty one patients (55%) developed AID prior to receiving any treatment for CLL. Only 9 of the 33 patients who developed AID after treatment for CLL had received purine analogues. Eighteen (24%) patients with CLL and AID died; the estimated median survival was 12.4 yr. Discussion In this CLL population with predominantly early stage disease at diagnosis (> 60% Rai stage 0), AID is clinically distinct from that previously reported in patients with more advanced disease. A higher proportion of patients had ITP which was usually asymptomatic. Most patients with AID had not previously required therapy for CLL. Purine analogue therapy did not appear to be a major cause of AID in this population. Overall survival was considerably more favorable than that previously reported with CLL induced bone marrow failure (Rai stage III-IV, Binet C). We conclude that the earlier diagnosis and subsequently longer follow up of patients with CLL has altered the clinical presentation and prognostic consequences of AID. A more detailed analysis of the correlation between novel prognostic risk factors and the risk of AID in CLL patients is planned.


2020 ◽  
Vol 9 (02) ◽  
pp. 090-092
Author(s):  
Gaurav Das ◽  
V. Sridevi ◽  
Mohanaraj Natarajan

Abstract Background Primary mucinous epithelial ovarian adenocarcinoma (mEOC) constitutes a small percentage (2–5%) of ovarian cancer. Our aim is to understand the clinicopathological characteristics and survival results of patients with mEOC after a long-term follow-up. Materials and Methods This is a retrospective study of primary mEOC cases treated at a tertiary cancer center in India, from January 1, 2005, to December 31, 2012. Results Out of 958 malignant ovarian tumors, 52 (5.43%) were mucinous adenocarcinoma. Nearly 71.2% of cases were of early-stage disease, and the remaining were of advanced-stage disease. After a follow-up period of 63 months (range: 1–138 months), the 5-year actuarial overall survival for stages I, II, III, and IV was 92.5, 70, 38.5, and 0%, respectively. Among advanced-stage tumors, half of them progressed without undergoing cytoreductive surgery and died. Conclusion Most of the mEOC cases present in early stages and have good clinical outcome. Patients with advanced-stage disease do not respond well to standard chemotherapy regimens in use and have poor survival figures. The use of primary cytoreduction should be considered in the place of interval cytoreduction for advanced mEOC.


Hand ◽  
2021 ◽  
pp. 155894472110172
Author(s):  
Logan R. Koehler ◽  
Ghazi M. Rayan

Background: Thumb trapeziometacarpal (TM) joint arthrosis is a common cause of thumb pain, which adversely affects hand function. Early arthrosis is characterized by capsular laxity, painful pinch and grip, and physical findings of joint tenderness and laxity. Dorsoradial capsulodesis (DRC) is a surgical technique used to stabilize the TM joint and treat early-stage arthrosis. We aim to evaluate the clinical outcomes of DRC for treating trapeziometacarpal instability in early-stage disease. Methods: Between 2003 and 2019, 23 patients underwent DRC. Patients with stage I TM arthritis and more than 6-month postoperative follow-up were included. Pain and disability scores were calculated along with physical examination and radiographic evaluation at the final follow-up. Results: At mean postoperative follow-up of 43.5 months, 13 patients with a mean age of 39.1 years were examined. The mean Disabilities of the Arm, Shoulder, and Hand score was 5.7, and visual analog pain score was 0.5. Patients had no significant difference in strength or range of motion in the ipsilateral versus contralateral hand. Follow-up radiographs did not demonstrate arthritic changes. Conclusions: Dorsoradial capsulodesis is a technically simple and reasonable option for stabilizing the TM joint in patients with early-stage arthrosis. This intervention showed no midterm progression to advanced arthritis in this cohort.


2021 ◽  
pp. 019459982199664
Author(s):  
Ryan S. Jackson ◽  
Katelyn Stepan ◽  
Craig Bollig ◽  
Rahul K. Sharma ◽  
Mihir Patel ◽  
...  

Objective The incidence of oropharyngeal squamous cell carcinoma (OPSCC) is increasing secondary to human papillomavirus (HPV)–related disease. Despite this, outcomes of patients with HPV-negative OPSCC undergoing transoral robotic surgery (TORS) are largely unknown. Study Design Analysis of the National Cancer Database (NCDB). Setting Not applicable. Methods The 2015 participant user file from the NCDB was analyzed between 2010 and 2015 for patients with OPSCC who underwent TORS and neck dissection. Kaplan-Meier survival analysis was used to estimate overall survival of the study population. Univariable Cox survival analyses was used to determine significant associations between demographic, tumor, and treatment characteristics and overall survival (OS). Results There were 164 patients (124 male and 40 female) with a mean age of 58 years (30-89 years). Median follow-up was 34 months. Five-year OS was 78% (95% CI, 70%-86%). Patients with early stage disease (pT1-2, N0-1) had significantly improved OS compared to patients with advanced T- or N-stage disease (log-rank 0.011; 5-year OS: 88% [95% CI, 78%-98%] vs 66% [95% CI, 50%-82%]). Conclusion Very few patients in the NCDB underwent TORS for HPV-negative OPSCC, but those who did had favorable outcomes, especially in early stage disease. Based on these findings, TORS may be considered in the treatment algorithm for patients with HPV-negative OPSCC. Level of Evidence Level IV.


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