bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intensive alternating 6-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma in children and adolescents: Impact on survival.
Authors: Demian Gerges Attia , Hussein Hany , Ebeid Emad Nabil , Elbadawy Samy 
Published: March 2020

9566 Background: Both 5-year overall survival (OS) and event free survival (EFS) for pediatric rhabdomyosarcoma (RMS) has increased in the last 3 decades through multimodality, risk-adapted management. The reported EFS for high risk RMS in children treated at the NCI in Egypt during the 1990s was 50%. Using an intensive 6 drug alternating chemotherapy regimen in addition to local control measures was our aim to improve the outcome for this group of patients. Methods: Forty-six previously untreated patients, younger than 21 years of age, with localized high risk RMS received this regimen. High risk criteria included: (1) Localized tumors (T1) biopsied or incompletely resected, ortumors extending beyond the tissue or organ of origin (T2) completely or incompletely resected at any site (excluding orbit, uterus, vagina, and paratestis); (2)All node positive patients with primary tumor at any site; and (3) All RMS with alveolar histology at any site. Chemotherapy regimen comprised 27 weeks of alternating 6 drugs (carboplatin, doxorubicin hydrochloride, ifosfamide, actinomycin D, etoposide, vincristine). Local therapy (surgery, radiotherapy, or both) was offered at week 9. Results: Forty-six patients meeting high-risk criteria were recruited from September 2000 to November 2005. Median follow-up of survivors was 62 months. The 5-year OS and EFS for the whole group was 64% ± 10% and 47% ± 8% respectively. The EFS was significantly affected by: the size of the tumor (≤5 cm vs. > 5 cm, p= 0.03), SIOP UICC clinical stage (p = 0.004), IRS stage (p = 0.01), lymph node status (p = 0.02), surgery vs. incisional biopsy (p=0.01) and overall duration of time in which therapy was delivered (p = 0.04). There was significant toxicity, mainly hematologic, but only one treatment related fatality. Conclusions: The use of intensified alternating 6-drug CT did not improve the EFS compared with historical control although it was feasible to be delivered safely in a variety of outpatient settings. Surgical resection of the tumor is essential. Delivering therapy in a timely fashion appears to impact outcome and future investigations will focus on impediments to administering chemotherapy as scheduled.
Long-term outcomes of children with Xp11.2 translocation renal cell carcinoma.
Authors: Asanuma Hiroshi , Mizuno Ryuichi , Masuda Aya , Satoh Hiroyuki , Matsui Zenichi , Kono Hidaka , Shinoda Kazunobu , Kikuchi Eiji , Nagata Hirohiko , Miyajima Akira , Nakagawa Ken , Oya Mototsugu 
Published: March 2020

9569 Background: We aimed to assess the clinico-pathological characteristics and the long-term prognosis of Xp11.2 translocation renal cell carcinoma (Xp-RCC) in children. Methods: A total of 52 Japanese children with renal tumors were presented to our institutions. Of these, 5 (10%) had RCC, and all RCCs were Xp11.2 translocation subtype with positive nuclear transcription factor E3 immunostaining. We retrospectively reviewed the pathological and hospital records of these 5 patients. Results: In the 1 boy and 4 girls with an average age of 9 years 7 months at diagnosis, the most common presenting complains were gross hematuria (60%) and palpable abdominal mass (40%). All patients had unilateral disease and there was no special family or medical history in any children. Computerized tomography revealed characteristic calcification within the tumor in 4 of the 5 patients (80%). In the remaining case, the lesion had high density areas with microcalcification, as confirmed by histopathological study. In 3 patients with regional lymph node metastasis, calcification was also observed in the metastatic lesions. Stage of the disease were stage I in 1 patient, II in 1, IV without and with distant metastasis in 2 and 1, respectively. All patients underwent transabdominal nephrectomy with regional lymphadenectomy. One patient with stage I disease had multiple metastases 15 months after surgery and died of disease in spite of interferon and chemotherapy 4 years postoperatively. Three patients received adjuvant interferon therapy and 2 of them are without evidence of recurrence postoperative 19 and 14 years, respectively. One of them had lymph node and lung metastases 12 years postoperatively. The remaining one patient had multiple lymph node, lung, bone, and dura mater metastases at diagnosis. The latter 2 patients have received targeted therapy (sunitinib, temsirolimus) without their progression for 6 and 12 months, respectively. Conclusions: Calcification within the tumor and/or metastatic lesions is characteristic findings suggestive of Xp-RCC in children. Regional lymphadenectomy or targeted therapy may provide some benefit in selected pediatric patients. Long-term follow-up is essential in pediatric patients with RCC.
Ifomide/pirarubicin/etoposide/carboplatin (ITEC) as second-line chemotherapy for hepatoblastoma.
Authors: Hiyama Eiso , Kamimatsuse Arata , Ogura Kaoru , Hishiki Tomoro , Watanabe Kenichiro , Yano Michihiro , Kondo Satoshi , Tajiri Tatsuro , Ida Kohmei , Sasaki Fumiaki 
Published: March 2020

9577 Background: The Japanese Study Group for Pediatric Liver Tumor is running cooperative treatment studies on hepatoblastoma (HBL) since 1991. The main aim in JPLT-1 study was to evaluate the efficacy of cisplatin/pirarubicin (CITA). A total of 145 cases were registered and their 5-year overall survival (OS) and event-free survival (EFS) were 73.4% and 66%, respectively (J Pediatr Surg 37: 851-6, 2002). Then, JPLT-2 protocol, in which CITA is kept as the first-line treatment, and ifomide, etoposide, pirarubicin, and carboplatin (ITEC) is the second-line regimen for CITA-resistant cases, was launched to evaluate the cure rate of risk-stratified HBL: standard risk HBL (a tumor involving three or fewer sectors of the liver) and high risk HBL (a tumor involving all sectors of the liver or with metastasis). Methods: In JPLT-2, 281 HBL cases were registered in JPLT-2 between 1999 and 2010, and 69 cases underwent ITEC protocol due to poor response to the CITA regimen. To evaluate the efficacy of the ITEC regimen, surgical resectability and outcome of these 69 patients who underwent this treatment. Results: These 69 cases were divided into 53 high-risk (metastatic, involvement of all sectors of the liver, vascular invasion and/or extra-hepatic intra-abdominal disease) and 16 standard-risk HBL (all others). All cases were initially treated with the CITA regimen and then underwent the ITEC regimen due to poor response to CITA. Complete resection of the liver tumor could be achieved in 48 patients (69.6%) consisting of 13 (81%) standard, 35 (66%) high- risk cases. The 5-year OS and EFS of the cases with standard risk HB were 96% and 76%, while that of the cases with high risk HB was 54% and 34%. The late phase complications in 281 cases were 3 cases with maldevelopment, 13 with cardiac complications, 20 with ototoxicity and 5 with second malignancies. Conclusions: As compared with the CITA-sensitive cases, ITEC regimen achieved similar rates of survival for CITA-resistant cases both in standard and high-risk HBL cases, indicating ITEC is effective as a second-line chemotherapeutic regimen for HBL.
Proton radiotherapy for rhabomyosarcoma: Preliminary results from a multicenter prospective study.
Authors: Yock Torunn I. , Szymonifka Jackie , Friedmann Alison M. , Mahajan Anita , Yeap Beow Yong , MacDonald Shannon M. , Kasper Hallie Bieber , Grosshans David , Marcus Karen Chayt , Tarbell Nancy 
Published: March 2020

9585 Background: Pediatric rhabdomyosarcoma (RMS) is commonly cured with chemotherapy and radiation. However, late effects of radiotherapy (RT) can be disabling. Proton RT irradiates less normal tissue, which should result in fewer late side effects of treatment. The purpose of this study was to describe the disease control and side effect profile of proton RT in RMS patients (pts). Methods: Eligible pts included those with localized disease and metastatic embryonal RMS if age 2-10. All pts were treated with VAC (vincristine, actinomycin, cyclophosphamide) based chemotherapy and proton RT, median dose 50.4 Gy (36-50.4 GyRBE). Concurrent enrollment in COG protocols was allowed. All pathology/imaging was reviewed at the treating institution. Results: 47 pts with RMS were prospectively enrolled from January 2005 to June 2011 and evaluable for analysis. Median age was 3.1 yrs, (range 0.6-15.6 years; M/F ratio 23:24). There were 1, 7, 37, and 2 Group I, II, III and IV and 14, 12, 19 and 2, Stage I-IV pts respectively, and 33 with embryonal 14 with alveolar/other. Most common sites included PM (48.9%), orbital (23.4%) bladder/prostate (6.4%), H&N non-PM (6.4%), extremities (4.3%), trunk/abdomen 2.1%), perineal/anal region (2.1%) and other (6.4%). Median follow-up is 15.2 months. One/two-year overall survival (OS) and progression-free survival (PFS) for the entire group is 94/81% (OS) and 79/73% (PFS). 2-year OS for stage I,II/III,IV pts is 91/66% (OS, p=0.114) and two-year PFS for these pts is 86/57%, respectively, (p=0.083. 16 (34%) had grade 3/4 acute toxicities attributable to the radiation during treatment, the most common of which was mucositis/oral pain (12.8%), anorexia (4.3%), and erythema (4.3%). Among the 24 pts analyzable for late toxicities with at least 2 yrs of follow up, there were no grade 3 or 4 late toxicities attributable to radiation. Conclusions: Early results of this prospective trial demonstrate comparable disease outcomes and thus far limited late effects in a young pediatric RMS population. However, additional follow up is needed to determine if protons truly reduce rates and severity of late effects compared with photon cohorts published in the literature.
Effectiveness of using cardiac biomarkers to detect late anthracycline cardiotoxicity in childhood leukemia survivors.
Authors: Mladosievicova Beata , Urbanova Dagmar , Radvanska Eva , Mikuskova Eva , Simkova Iveta 
Published: March 2020

9592 Background: Cardiotoxicity is usually detected only when clinical symptoms or progressive cardiac dysfunction has already occurred. Cardiac biomarkers (troponin T and N-terminal fragment brain natriuretic peptide precursor) have been hypothesized to reflect subclinical anthracycline cardiotoxicity earlier than echocardiography. This study aims to assess the effectiveness of using cTNT and NTproBNP in asymptomatic long-term survivors of childhood leukemia treated with and without antracyclines (ANT). Methods: Sixty-nine childhood leukemia survivors 5 - 25 years after completion of therapy were evaluated with immunochemical analysis of cTnT and NT-proBNP and echocardiography. Patients from group I (n = 36) received combined therapy with anthracyclines (ANT) with total cumulative dose 95-600 (median 221) mg/m2, patients from group II (n = 33) received therapy without anthracyclines (nonANT). Control group consisted from 44 age- and gender-matched apparently healthy subjects. Results: Levels of NTproBNP were significantly higher in ANT group than in controls (median 51,52 vs 17,37 pg/ml; p=0.0026). Patients treated with ANT had significantly increased median values of NTproBNP compared with patients in non ANT group (51,52 vs 12,24 pg/ml; p=0.0002). CTnT levels remained below the diagnostic cut-off levels in all groups. No patient had echocardiographic abnormalities, but significant differences were found in mean values of left ventricular ejection fraction and deceleration time between patients treated with and without ANT. Conclusions: Assessment of plasma NTproBNP concentrations may be an effective tool for detection of late subclinical cardiac damage in survivors of childhood leukemia previously treated with low ANT doses. Higher NTproBNP levels in patients after ANT therapy might reflect an initial stage of cardiotoxicity before the development of echocardiographic abnormalities. This study was supported by a grant from Ministry of Health 2007/42-UK-18, Slovakia.
Can Oncotype DX recurrence score (RS) be used in luminal A and luminal B breast cancer patients (pts) to predict the likely benefit of chemotherapy? A retrospective study in the Spanish population.
Authors: García-Estevez Laura , Contreras Ana C , Calvo Isabel , Fernandez Abad Maria , de la Cruz Juan J , Perea Sofia , Suárez Ana , López-Ríos Fernando , Hidalgo Manuel 
Published: March 2020

e11006 Background: The 2011 St Gallen guidelines recommend the use of Ki67 as a surrogate marker for luminal A and Luminal B breast cancer subtypes. Pts with luminal B subtypes should be considered for adjuvant chemotherapy. The guidelines also recognize the Oncotype DX RS as a predictor of chemotherapy benefit. The aim of this study is to assess the distribution of the RS in luminal A and luminal B breast cancer subtypes as defined by Ki67. Methods: Data from 91 pts with invasive breast cancer for which Oncotype DX results and pathology data were available. Pathological assessment was evaluated by the same pathologist. Estrogen (ER) and progesterone (PR) receptor was assessed by immunostaining (cut-off 10% nuclear staining). Ki67 was assessed by IHC [high (≥14%) and low (< 14%)]. Grading was performed by using Nottingham grading system. Results: Median age: 50 years (range: 35-78); premenopausal status: 49 pts (53.8%). Median tumor size: 1.5 cm (0, 3-6); Median of Ki 67 index: 15. 5 (range: 3-63); Median ER: 93 (35-100) and PR: 85(0-100). Sixty nine pts (75.8%) had negative-lymph nodes. RS was low in 56 (61. 5%) cases, intermediate in 24 (26. 4%), and high in 11 (12. 1%). The median RS was 16 (range 3-55). Forty six (51%) tumors were classified as luminal B (according to high Ki 67) and 38 (41.7%) as Luminal A. In the Luminal B group RS was low in 28 (61%) pts, intermediate in 10 (22%) and high in 8 (17%) while in the Luminal A group 23 (61%) had low RS; 13 (34%) intermediate and 2 (5%) high RS. RS changed the first treatment recommendation in 23 (50%) cases of Luminal B subtype vs. 10 (26%) cases in Luminal A subtype (p=0.013). Conclusions: Although based in a small case series, the results show that a substantial number (61%) pts with Luminal B breast cancer subtype had a low RS, therefore preserving them from adjuvant chemotherapy treatment. In addition, 5% of patients with Luminal A breast cancer had a high RS and thereby likely to benefit from chemotherapy. The wide range of RS in both Luminal B and Luminal A breast cancer subtypes confirm the important role of Oncotype DX in treatment decision- making.
Does obesity change the effect of aromatase inhibitors (AIs) on estradiol, leptin, insulin, and IGF-1 serum levels in breast cancer patients?
Authors: Artac Mehmet , Askin Dudu , Kiyici Aysel , Eren Onder Orhan , Oncel Mufide , Boruban Melih Cem , Bozcuk Hakan Sat 
Published: March 2020

e11008 Background: The complexity of the relationship between obesity and breast cancer reflects important contributions of obesity-related factors in the adjuvant therapy. The efficacy of adjuvant AIs compared to tamoxifen is lower in obese postmenopausal women. Therefore, we aimed to identify whether the effect of AIs on estradiol, leptin, insulin, and IGF-1 serum levels with respect to the status of the obesity in breast cancer patients. Methods: A total of 34 postmenopausal breast cancer patients treated with adjuvant AIs were enrolled in the study. Pretreatment and 3 months after treatment body weight, height, waist-to-hip (WHR) ratio and body mass index (BMI) were recorded. Blood samples for serum estradiol, leptin, insulin and IGF-1 were drawn after 12 hour starvation and stored in – 70o before and 3 months after the onset of AI treatment. Results: Median age and BMI were 61 years (range 49-84) and 31 (range 22-43), respectively. 18 (%52.9) patients were treated with letrozole and 16 (%47.1) patients were treated with anastrazole. There was no change in the 3 months period in weight, BMI and WHR. Estradiol levels were decreased significantly between the baseline and the 3rd month (p<0.01). There was a slight increase in the leptin levels between the basal and 3rd month (p>0.05). No significant change was detected in insulin and IGF-1 levels in the follow-up period. In the multivariate regression model, there was no difference between the effect of letrozole and anastrazole on estradiol levels. Pretreatment BMI did not alter the effect of AIs on estradiol levels. The only factor related with the effect of the AIs is the pretreatment estradiol levels. Conclusions: This study shows that the suppression of estrogen in obese breast cancer patients with AIs at the standard doses may not be different from non-obese patients. Further researches may show us other mechanisms for the obesity-related factors to improve outcome with aromatase inhibition in obese breast cancer patients.
Advanced breast cancer (ABC): Clinical-pathologic characteristics of long-term survivors after dissemination of the tumor.
Authors: Nacul Mario Javier , Gil Deza Ernesto , Morgenfeld Eduardo L. , Rivarola Edgardo G. J. , Gercovich Felipe G. 
Published: March 2020

e11037 Background: ABC is currently an incurable disease but some patients achieve a longer survival. The following case-control study compared patients suffering from ABC that survived 50 months or more after the onset of metastasis (case) with a group of patients similarly diagnosed treated alongside the case group, whose survival after metastasis was less than 50 months (control). Methods: A search of the IOHM breast cancer tumors registry between September 1997 and April 2010 was carried out. Only those with ABC were included in the paper. The population was divided between cases and controls and both groups were compared. Results: 227 patients meeting the required criteria were found. The population characteristics are detailed in the table below. In 161 pts (70%), a molecular subtype was established: Luminal Type A or B (Cases: 49 pts; Controls: 84 pts), Her2 (Cases: 5 pts; Controls: 9 pts), Triple Negative (Cases: 0 pts; Controls: 14 pts). Systemic and local treatments were similar in both groups, depending on the disease’s stage. Conclusions: 1 – Sixty seven patients (cases) survived 50 or more months after the onset of metastasis. 2 – The Luminal Subtype, long DFS of early stages, compromise of non-visceral organs and the compromise of only one organ were indicative of extended survival of the patients. 3 – No patient with Triple Negative tumors was able to achieve prolonged survival, and so we must continue to research better treatments for these patients. [Table: see text]
Use of platinum-based neoadjuvant chemotherapy (NACT) in patients (pts) with hormone receptor-positive breast cancer.
Authors: Kyriakopoulos Christos , Ambros Tadeu Frantz , Montero Alberto J. , Hurley Judith 
Published: March 2020

e11038 Background: NACT is used for the treatment of locally advanced breast cancer. Evaluation of breast cancer subtypes and their responses to specific chemotherapy regimens is important. Methods: Retrospective review of medical records from University of Miami/Sylvester Comprehensive Cancer Center/Jackson Memorial Hospital from 1998-2011. Complete pathologic response (pCR) was classified as absence of malignant tissue both in the breast and the lymph nodes in the surgical specimen. Results: One hundred ninety-one women with ER-positive breast cancer received platinum based NACT. Mean age was 52 years (range 31-83). One hundred eleven (58.1%) women were premenopausal and 80 (41.9%) postmenopausal. The clinical stage at presentation: IIA (6.3%), IIB (26.7%), IIIA (31.9%), IIIB (30.9%), IIIC (4.2%). Sixty one (31.9%) pts had her-2/neu positive tumors. pCR occurred in 17 patients (8.9%), premenopausal vs. postmenopausal (9.9% vs. 7.5%, p=0.56), white race vs. black (9.9% vs. 4.9%, p=0.32), Hispanic vs. non-Hispanic (9.4% vs. 6.8%, p=0.6), tumor size <=4 cm vs. >4 cm (14.7% vs. 7.6%, p=0.19), her-2/neu positive vs. her-2/neu negative (13.1% vs. 6.9%, p=0.16), T4 vs. T1-T3 (4.8% vs. 10.9%, p=0.19). Conclusions: pCR rates with platinum based NACT in pts with hormone receptor-positive tumors were low. The pCR rate did not appear to be influenced by her2/neu, tumor size, race, ethnicity or menopausal status.
Compliance and arthralgias in clinical therapy (COMPACT): Assessment of the incidence of arthralgia, therapy costs, and compliance in the first year of adjuvant anastrozole.
Authors: Hadji Peyman , Blettner Maria , Bolten Wolfgang W. , Harbeck Nadia , Hindenburg Hans-Joachim , Jackisch Christian , Klein Peter , König Klaus , Rief Winfried , Wallwiener Diethelm , Zaun Silke , Kreienberg Rolf 
Published: March 2020

e11040^ Background: Aromatase Inhibitors (AI) are well established as adjuvant endocrine treatment for postmenopausal women with HR+ early breast cancer (EBC). However, clinical trial data show higher frequently of arthralgia with AI than tamoxifen. As arthralgia may be greatly influencing compliance to adjuvant therapy, we designed a prospective trial to collect real world data on the effects of AI-associated arthralgia on patient compliance, patient outcomes and treatment costs of arthralgia. Methods: COMPACT is an open, prospective non-interventional study (NCT00857012) assessing the incidence of arthralgia, therapy costs, and compliance within the first year of adjuvant anastrozole (ANA) therapy in postmenopausal patients with HR+ EBC. Patients on adjuvant ANA for 3-6 months were enrolled and stratified by initial adjuvant ANA or switch from tamoxifen. All patients receive regular standardized information about breast cancer from baseline to week 20 to support treatment compliance. Data on demographics, arthralgia, therapy thereof and QoL are collected at baseline, 3, 6 and 9 months. Primary endpoints are scaled data on arthralgia and compliance within the first year of ANA therapy. Secondary endpoints include the incidence of arthralgia, therapy costs, reasons for non-compliance, and influence of arthralgia on clinical outcome. Results: From Apr 2009 to Mar 2011, 2313 patients were enrolled, 2007 on upfront ANA and 306 patients on switch therapy. The mean age was 64.5 years, mean BMI 27.7. Only 16.8% had received HRT prior to their EBC. 41.5% had symptoms relating to skeleton or musculature, and 11.9% stated arthralgia existing prior to ANA. 13.1% reported a worsening of pre-existing arthralgias or new arthralgia after starting ANA. Predictors for non-adherence to AI and scaled data on arthralgia are under analysis. Conclusions: COMPACT aims to provide valid data on AI-associated arthralgia, treatment, treatment costs and compliance to AI. This may help to better inform patients and health care providers about these clinically important issues with the aim to improve adherence to adjuvant ANA, breast cancer outcomes, and therapy costs.