Ovarian Sex Cord–Stromal Tumors in Children and Adolescents

2003 ◽  
Vol 21 (12) ◽  
pp. 2357-2363 ◽  
Author(s):  
D.T. Schneider ◽  
G. Calaminus ◽  
R. Wessalowksi ◽  
R. Pathmanathan ◽  
B. Selle ◽  
...  
Keyword(s):  
Malignant Ascites ◽  
Stage Ii ◽  
Event Free Survival ◽  
Free Survival ◽  
Stromal Tumors ◽  
Gynecology And Obstetrics ◽  
Diagnostic Standards ◽  
Stage Ia ◽  
Sex Cord Stromal Tumors

Purpose: To develop diagnostic standards and a risk-adapted therapeutic strategy for ovarian sex cord–stromal tumors (OSCST). Patients and Methods: Fifty-four patients were prospectively enrolled as follow-up patients onto the German Maligne Keimzelltumoren protocols. Surgical protocols and histopathology were reviewed centrally (53 patients with complete data). Surgery included ovariectomy in 18 patients, salpingo-ovariectomy in 34 patients, and hysterectomy in one patient. Patients with stage IA tumors were followed-up at regular intervals, whereas nine patients with stage IC and six patients with stage II to III tumors were treated with cisplatin-based chemotherapy. Results: International Federation of Gynecology and Obstetrics stage was IA in 27 patients, IC in 21 patients, II in three patients, and III in three patients. After a median follow-up of 59 months (range, 6 to 193 months), event-free survival ± SD was 0.86 ± 0.05 (47 of 54 patients) and overall survival was 0.89 ± 0.05 (49 of 54 patients). Prognosis correlated with stage (event-free survival ± SD: IA, 1.0 [27 of 27 patients]; IC, 0.76 ± 0.09 [16 of 21 patients]; and II/III, 0.67 ± 0.19 [four of six patients]; P = .02). Ten of 15 patients treated with chemotherapy, including four of six stage II to III patients, are alive after a median follow-up of 33 months. Conclusion: On the basis of a standardized clinical and histopathologic assessment, risk-adapted therapeutic strategies for OSCST can be evaluated. Considering our experience, we would recommend that stage IA tumors be followed up at regular intervals, whereas we would recommend cisplatin-based chemotherapy in stage IC tumors with preoperative rupture or malignant ascites, especially those with high mitotic activity. Finally, cisplatin-based chemotherapy also seems to be effective in advanced-stage tumors.

scholarly journals Ovarian Sex Cord-Stromal Tumors

2016 ◽  
Vol 12 (10) ◽  
pp. 940-946 ◽  
Author(s):  
Kris Ann P. Schultz ◽  
Anne K. Harris ◽  
Dominik T. Schneider ◽  
Robert H. Young ◽  
Jubilee Brown ◽  
...  
Keyword(s):  
Leydig Cell ◽  
Pelvic Mass ◽  
Signs And Symptoms ◽  
Stromal Tumors ◽  
Gynecology And Obstetrics ◽  
Stage Ia ◽  
Poorly Differentiated ◽  
Clinically Significant ◽  
The Relationship ◽  
Sex Cord Stromal Tumors

Ovarian sex cord-stromal tumors are clinically significant heterogeneous tumors that include several pathologic types. These tumors are often found in adolescents and young adults and can present with hormonal manifestations as well as signs and symptoms of a pelvic mass. Serum tumor markers may assist in preoperative diagnosis and surveillance. Several subtypes are associated with genetic predisposition, including those observed in patients with Peutz-Jegher syndrome. Recent studies have elucidated the relationship between Sertoli-Leydig cell tumors and DICER1 mutations. When classified as International Federation of Gynecology and Obstetrics stage Ia, most subtypes may be treated with surgery alone. Higher stage or recurrent tumors have variable prognoses that range from a usually rapid course in poorly differentiated Sertoli-Leydig cell tumor to an often prolonged course in adult granulosa cell tumors. New understanding of the molecular pathogenesis of these tumors may pave the way for novel therapeutics.

Early Stage Diffuse Large B Cell Lymphoma (DLBCL) in the CHOP-R Era: Does Involved Field Radiation Therapy (IFRT) Impact Outcome?.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3433-3433
Author(s):  
Manzurul A. Sikder ◽  
Sughosh Dhakal ◽  
Jennifer Kelly ◽  
Steven H. Bernstein ◽  
Faith Young ◽  
...  
Keyword(s):  
Early Stage ◽  
B Cell Lymphoma ◽  
Stage I ◽  
Stage Ii ◽  
Disease Stage ◽  
Event Free Survival ◽  
Bulky Disease ◽  
Free Survival ◽  
The Impact

Abstract Historically, patients (pts) with early stage DLBCL treated without consolidative IFRT more commonly relapsed in sites of initial disease. While event-free survival (EFS) was superior with IFRT after CHOP in the short-term in a large randomized trial (NEJM 339:21), prolonged follow-up demonstrated equivalent overall survival due in part to late relapses (Blood 98:724a). The optimal therapy for pts with early stage DLBCL in the rituximab-CHOP (R-CHOP) era has not been established. To determine the impact of IFRT after R-CHOP treatment on outcome of early stage DLBCL, we reviewed medical records of 67 consecutive pts with stage I and II DLBCL who were primarily treated with R-CHOP with or without IFRT between February 2001 and March 2006. Three to 8 cycles of R-CHOP were administered, and 30–45 Gy IFRT was administered for those irradiated. The Kaplan-Meier method was used for the estimates of outcome. EFS was measured from the date of diagnosis until first documented disease progression or death from any cause. Comparisons were adjusted for IPI by Cox proportional hazards regressions, and all tests were two sided. Median follow-up was 2.6 years (range 0.5–5.5). 45 (67%) of the pts were men. Median age at diagnosis was 60 years (range 20–92); 18 (27%) had ‘B’ symptoms; bulky disease (defined as mass >10cm) was present in 15 (22%); 27 (40%) had stage I and 40 (60%) had stage II disease. Stage-adjusted IPI score distribution was: IPI 0, n=6 (9%); IPI 1, n=17 (25%); IPI 2, n=24 (36%); IPI 3, n=16 (24%); IPI 4, n=4 (6%). 39 (58%) pts had extranodal disease (23% soft tissue, 21% head & neck, 56% others). As expected, since the majority of pts had more than 1 IPI risk factor, the 2-year event-free survival (EFS) was 68% (median EFS not reached). 11 (16%) pts had primary refractory disease including 3 pts who died during treatment, and they were excluded from further analysis. Of the remaining pts, 34 (61%) received IFRT, and 22 were treated with R-CHOP alone. The IPI scores of the 2 groups were balanced, but as expected, stage II disease was more common in pts who received R-CHOP alone. The EFS at 2 years for pts treated with consolidative IFRT was 87%, and with chemotherapy alone was 72%. When controlling for IPI score and the presence of bulky disease, there was a significant benefit in EFS for patients treated with R-CHOP + IFRT compared with R-CHOP alone (p=0.027). Therefore, in pts with early stage DLBCL treated with R-CHOP, we find improved outcomes with a combined modality approach including consolidative IFRT compared with chemotherapy alone as was observed in the CHOP era. Our study has inherent biases due to its retrospective nature, and limited follow-up. However, to our knowledge, this is the first study that compare R-CHOP with and without IFRT in early stage DLBCL, and therefore has important implications on future clinical trial designs.

Improvement of Survival in Sex Cord Stromal Tumors: An Observational Study With More Than 25 Years Follow-Up

2009 ◽  
Vol 64 (7) ◽  
pp. 455-457
Author(s):  
Gabriele Hölscher ◽  
Christoph Anthuber ◽  
Gunther Bastert ◽  
Alexander Burges ◽  
Doris Mayr ◽  
...  
Keyword(s):  
Observational Study ◽  
Stromal Tumors ◽  
Sex Cord Stromal Tumors

Abstract 16154: Baseline Left Ventricular Diameter Predicts Recovery in New Systolic Heart Failure Syndromes

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Daniel Gold ◽  
Nathan Kong ◽  
Matthew Gold ◽  
Tess Allan ◽  
Anand Shah ◽  
...  
Keyword(s):  
Left Ventricular ◽  
Medical Decision ◽  
Left Ventricular Ejection ◽  
Internal Diameter ◽  
Event Free Survival ◽  
Short Term Treatment ◽  
Ventricular Ejection Fraction ◽  
Free Survival ◽  
Advanced Therapies

Introduction: It is unknown how often patients with very advanced left ventricular (LV) dilation at initial presentation demonstrate meaningful recovery with medical therapy. Understanding short term treatment outcomes may impact medical decision making and counseling. Hypothesis: Patients with left ventricular end diastolic internal diameter (LVEDD) > 6.5cm will be less likely to recover left ventricular ejection fraction (LVEF) as compared to patients with LVEDD < 6.5cm. Methods: Patients were retrospectively identified by a database search of echocardiogram studies obtained at the University of Chicago between 2008-2018. Manual review was performed to ensure new diagnosis of systolic dysfunction with LVEF ≤ 35% and follow up echocardiogram study within 3 to 9 months of index study. LVEDD was determined from parasternal long axis views per routine. LVEF recovery was specified as LVEF > 35%. Chart review was done to assess for composite death, hospice, transplant, left ventricular assist device, and sustained ventricular tachycardia. Chi-square, multivariable logistic regression, and Kaplan-Meier survival were used for analysis. Results: Out of 100 patients included for analysis, mean age was 59.7 years, 41 were female and 82 were African American. 17.7% of patients’ with LVEDD > 6.5 cm had LVEF recovery compared to 53.0% of patients’ with LVEDD ≤ 6.5 cm (p = 0.008). LVEDD > 6.5 cm was associated with less LVEF recovery even when adjusted for age, gender, hypertension, and diabetes (adjusted odds ratio 0.18, 95% CI 0.04 to 0.65). LVEDD > 6.5cm was associated with worse event free survival (p = 0.004) with a median follow-up time of 2.4 years. Conclusions: An LVEDD of > 6.5cm is associated with diminished LVEF recovery and event free survival when compared to those patients with an LVEDD ≤ 6.5cm. Delaying consideration for advanced therapies and device based therapies in hopes of recovery may be inappropriate for many such patients.

scholarly journals CRT-720 Mitral Balloon Valvuloplasty Long-Term Follow-Up of Single Balloon Versus Inoue Balloon Techniques. Independent Predictors of Survival and Event Free Survival

2014 ◽  
Vol 7 (2) ◽  
pp. S57
Author(s):  
Edison Sandoval Peixoto Peixoto ◽  
Ivana Picone Borges ◽  
Rodrigo Trajano Sandoval Peixoto ◽  
Ricardo Trajano Sandoval Peixoto
Keyword(s):  
Balloon Valvuloplasty ◽  
Event Free Survival ◽  
Free Survival ◽  
Long Term Follow Up ◽  
Single Balloon ◽  
Inoue Balloon

Event free survival in adults with relapsed ALL who underwent front-line therapy with CALGB 10403.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e19005-e19005
Author(s):  
Suravi Raychaudhuri ◽  
Ilana Yurkiewicz ◽  
Gabriel N. Mannis ◽  
Bruno C. Medeiros ◽  
Steve E. Coutre ◽  
...  
Keyword(s):  
Overall Survival ◽  
Cohort Study ◽  
Survival Time ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Event Free Survival ◽  
Front Line ◽  
Free Survival ◽  
First Relapse

e19005 Background: CALGB 10403 is a pediatric-inspired ALL regimen that has recently been shown to have improved survival rates in adolescents and young adults with ALL when compared to historical outcomes with traditional adult ALL regimens (Stock et. al, 2019). Methods: This is a retrospective cohort study of ALL patients who received induction CALGB 10403 at Stanford University (both on and off trial), achieved CR1, and subsequently relapsed. Primary outcome of interest was event free survival from time of diagnosis. Events were defined as relapse or death. Secondary outcomes were overall survival and event free survival from first relapse. Patients were censored at time of last clinical follow up. Results: 25 patients met inclusion criteria and received front-line CALGB 10403 from April 2010 to September 2018. At the time of initial diagnosis median age was 30 years (range 18 – 39 years). 68% of patients were male. 48% of patients were overweight and 40% were obese. 76% of patients had precursor B cell ALL while 24% had T cell ALL. 12% had CNS disease at diagnosis. 36% of patients had WBC greater than 30k. 12% of patients had CRLF2 rearrangement. 12% of patients were MRD positive after first induction. 20% of patients received rituximab. Median event free survival time from diagnosis was 20 months (range 3 – 79 months) and median overall survival time was 53 months. Blinatumomab was the most common salvage therapy after 1st relapse, followed by inotuzumab. 15 patients (60%) achieved CR2, of which 4 (27%) were MRD positive after 2nd induction. 15 patients (60%) went to HSCT. Of the patients who achieved CR2, 8 relapsed for a second time. Median event free survival time after first relapse was 23 months. Survival 1 year after relapse was 60%. 11 of the 25 patients were alive at last follow up. Median follow up time of survivors was 6 years. Conclusions: This is a descriptive retrospective cohort study of adult patients in a real world setting who received CALGB 10403 induction and subsequently relapsed. Compared to other studies of relapsed ALL patients who were induced with traditional chemotherapy (Fielding et. al, 2007), survival 1 year after relapse was much higher (60% vs. 22%). As CALGB 10403 becomes an increasingly common induction regimen for AYA and adults with ALL, further outcomes study is required.[Table: see text]

Adjuvant therapy with alpha-interferon in radically resected stage Il and III renal cell carcinoma

1992 ◽  
Vol 59 (1_suppl) ◽  
pp. 160-162
Author(s):  
◽  
Urologico Lombardo
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Randomized Study ◽  
Disease Free Survival ◽  
Stage Ii ◽  
Alpha Interferon ◽  
Free Survival ◽  
Resected Stage

In February 1990, a multicentric randomized study was started to verify the therapeutic efficacy of adjuvant alpha-interferon in Robson's stage II and III renal cell carcinoma. Up to April 1992, 749 radical nephrectomies have been recorded by the 25 participating centers. Stage II and III patients fullfilling selection criteria were 124: 64 were assigned to Interferon therapy and 60 to control. The short follow-up does not allow any evaluation of the relapse rate. Therapy related toxicity was represented by mild fever and flu-like syndrome in 50% of cases and slight leucopenia in 5%. The recruitment of the first 200 randomized patients will end approximately within 1 year. While evaluating the hypothesis of a 20% difference in the 5 year disease-free survival between the two groups recruitment will continue up to a total of 350 cases, in order to verify the 15% hypothesis, too.

Lumpectomy and radiation therapy for the treatment of intraductal breast cancer: findings from National Surgical Adjuvant Breast and Bowel Project B-17.

1998 ◽  
Vol 16 (2) ◽  
pp. 441-452 ◽  
Author(s):  
B Fisher ◽  
J Dignam ◽  
N Wolmark ◽  
E Mamounas ◽  
J Costantino ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Radiation Therapy ◽  
Breast Cancer Incidence ◽  
Resected Specimen ◽  
Cumulative Probability ◽  
Event Free Survival ◽  
Free Survival ◽  
National Surgical Adjuvant Breast ◽  
Bowel Project

PURPOSE In 1993, findings from a National Surgical Adjuvant Breast and Bowel Project (NSABP) trial to evaluate the worth of radiation therapy after lumpectomy concluded that the combination was more beneficial than lumpectomy alone for localized intraductal carcinoma-in-situ (DCIS). This report extends those findings. PATIENTS AND METHODS Women (N = 818) with localized DCIS were randomly assigned to lumpectomy or lumpectomy plus radiation (50 Gy). Tissue was removed so that resected specimen margins were histologically tumor-free. Mean follow-up time was 90 months (range, 67 to 130). Size and method of tumor detection were determined by central clinical, mammographic, and pathologic assessment. Life-table estimates of event-free survival and survival, average annual rates of occurrence for specific events, relative risks for event-specific end points, and cumulative probability of specific events comprising event-free survival are presented. RESULTS The benefit of lumpectomy plus radiation was virtually unchanged between 5 and 8 years of follow-up and was due to a reduction in invasive and noninvasive ipsilateral breast tumors (IBTs). Incidence of locoregional and distant events remained similar in both treatment groups; deaths were only infrequently related to breast cancer. Incidence of noninvasive IBT was reduced from 13.4% to 8.2% (P = .007), and of invasive IBT, from 13.4% to 3.9% (P < .0001). All cohorts benefited from radiation regardless of clinical or mammographic tumor characteristics. CONCLUSION Through 8 years of follow-up, our findings continue to indicate that lumpectomy plus radiation is more beneficial than lumpectomy alone for women with localized, mammographically detected DCIS. When evaluated according to the mammographic characteristics of their DCIS, all groups benefited from radiation.

scholarly journals Chemotherapy in 998 unselected childhood acute lymphoblastic leukemia patients. Results and conclusions of the multicenter trial ALL-BFM 86

Blood ◽  
1994 ◽  
Vol 84 (9) ◽  
pp. 3122-3133 ◽  
Author(s):  
A Reiter ◽  
M Schrappe ◽  
WD Ludwig ◽  
W Hiddemann ◽  
S Sauter ◽  
...  
Keyword(s):  
Acute Lymphoblastic Leukemia ◽  
Risk Group ◽  
Lymphoblastic Leukemia ◽  
Multicenter Trial ◽  
Low Risk ◽  
Event Free Survival ◽  
Free Survival ◽  
Risk Patients ◽  
To Receive

Abstract In trial ALL-BFM 86, the largest multicenter trial of the Berlin- Frankfurt-Munster (BFM) study group for childhood acute lymphoblastic leukemia (ALL), treatment response was used as an overriding stratification factor for the first time. In the previous trial ALL-BFM 83, the in vivo response to initial prednisone treatment was evaluated prospectively. A blast cell count of > or = 1,000/microL peripheral blood after a 7-day exposure to prednisone and one intrathecal dose of methotrexate (MTX) identified 10% of the patients as having a significantly worse prognosis. In trial ALL-BFM 86 patients with > or = 1,000/microL blood blasts on day 8 were included in an experimental branch EG. Patients with < 1,000/microL blood blasts on day 8 were stratified by their leukemic cell burden into two branches, Standard Risk Group (SRG) and Risk Group (RG). SRG patients received an eight- drug induction followed by consolidation protocol M (6-mercaptopurine, high-dose [HD] MTX 4 x 5 g/m2) and maintenance. RG patients were treated with an additional eight-drug reinduction element. For EG patients protocol M was replaced by protocol E (prednisone, HD-MTX, HD- cytarabine, ifosfamide, mitoxantrone). All patients received intrathecal MTX therapy; only those of branches RG and EG received cranial irradiation. In branch RG, patients were randomized to receive or not to receive late intensification (prednisone, vindesine, teniposide, ifosfamide, HD-cytarabine) in the 13th month. During the trial reinduction therapy was introduced in branch SRG, because in the follow-up of trial ALL-BFM 83 the randomized low-risk patients receiving reinduction did significantly better. Nine hundred ninety- eight evaluable patients were enrolled, 28.6% in SRG, 61.1% in RG, 10.3% in EG. At a median follow-up of 5.0 (range 3.4 to 6.9) years, the estimated 6-year event-free survival was 72% +/- 2% for the study population, 58% +/- 5% in branch SRG for the first 110 patients without reinduction therapy, 87% +/- 3% for the next 175 patients with reinduction therapy, 75% +/- 2% in branch RG, and 48% +/- 5% in branch EG. Late intensification did not significantly affect treatment outcome of RG patients; however, only 23% of the eligible patients were randomized. Prednisone poor response remained a negative prognostic parameter despite intensified therapy. The results confirmed the benefit of intensive reinduction therapy even for low-risk patients. The strategy of induction, consolidation, and intensive reinduction may offer roughly 75% of unselected childhood ALL patients the chance for an event-free survival.

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