21 results on '"Winston W. Huh"'
Search Results
2. Prognostic Value of Cell-Surface Vimentin-Positive CTCs in Pediatric Sarcomas.
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Dao L, Ragoonanan D, Batth I, Satelli A, Foglesong J, Wang J, Zaky W, Gill JB, Liu D, Albert A, Gordon N, Huh W, Harrison D, Herzog C, Kleinerman E, Gorlick R, Daw N, and Li S
- Abstract
Background: Despite advances in care, the 5 year overall survival for patients with relapsed and or metastatic sarcoma remains as low as < 35%. Currently, there are no biomarkers available to assess disease status in patients with sarcomas and as such, disease surveillance remains reliant on serial imaging which increases the risk of secondary malignancies and heightens patient anxiety., Methods: Here, for the first time reported in the literature, we have enumerated the cell surface vimentin (CSV+) CTCs in the blood of 92 sarcoma pediatric and adolescent and young adult (AYA) patients as a possible marker of disease., Results: We constructed a ROC with an AUC of 0.831 resulting in a sensitivity of 85.3% and a specificity of 75%. Additionally, patients who were deemed to be CSV+ CTC positive were found to have a worse overall survival compared to those who were CSV+ CTC negative. We additionally found the use of available molecular testing increased the accuracy of our diagnostic and prognostic tests., Conclusions: Our findings indicate that CSV+ CTCs have prognostic value and can possibly serve as a measure of disease burden., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Dao, Ragoonanan, Batth, Satelli, Foglesong, Wang, Zaky, Gill, Liu, Albert, Gordon, Huh, Harrison, Herzog, Kleinerman, Gorlick, Daw and Li.)
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- 2021
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3. Association of body mass index with toxicity and survival in pediatric patients treated with cisplatin-containing regimens.
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Bhandari R, Scott E, Yeh MY, Wong K, Rushing T, Huh W, and Orgel E
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- Adolescent, Antineoplastic Agents therapeutic use, Antineoplastic Agents toxicity, Body Mass Index, Child, Cisplatin therapeutic use, Cisplatin toxicity, Female, Humans, Male, Progression-Free Survival, Proportional Hazards Models, Retrospective Studies, Survival Analysis, Antineoplastic Agents adverse effects, Cisplatin adverse effects, Malnutrition complications, Neoplasms complications, Neoplasms drug therapy, Obesity complications
- Abstract
Malnutrition is associated with treatment-related toxicities (TRT) in adults with solid tumors and in children with leukemia. Few studies have assessed whether malnutrition in pediatric patients treated for solid tumors impacts risk for TRT, relapse, and/or survival. To address this knowledge gap, this retrospective study evaluated the association between body mass index (BMI) at diagnosis, and imputed BMI during therapy, on the prevalence of TRT, specific toxicities, relapse, and survival in pediatric patients with solid tumors treated with cisplatin-containing regimens. Kaplan-Meier curves and regression models evaluated the association between patient-specific characteristics (including BMI) and TRT, relapse, and survival. The cohort included 221 patients, of whom 22% were malnourished at diagnosis (10% were underweight and 12% were obese). Most patients (60%) experienced at least one severe TRT, and 30% developed more than one severe TRT. Most patients with obesity at diagnosis remained obese during therapy (62%). In multivariable analysis, obesity at diagnosis was significantly associated with a more than threefold greater risk for developing severe TRT (p = 0.037), specifically for acute or chronic kidney injury ( p = 0.014). Obesity at diagnosis and adolescent and young adult age (≥15 years at diagnosis) were associated with worse event-free survival (hazard ratio [HR] 2.32, p = 0.024 and HR 2.28, p = 0.010, respectively) and overall survival (HR 3.69, p = 0.006 and HR 2.6, p = 0.012, respectively). Obese and older patients therefore constitute populations at risk for poorer outcomes. Prospective studies are warranted to gain further insight into the mechanism and role of obesity and adolescence in developing TRT and/or treatment failure.
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- 2021
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4. Primary Knee Intra-articular Synovial Sarcoma in Pediatric and Adolescent Patients.
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Cao Q, Shillingford N, Huh W, VandenBerg C, Raca G, Allison DC, Wang L, and Zhou S
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- Adolescent, Diagnosis, Differential, Female, Humans, Male, Sarcoma, Synovial pathology, Soft Tissue Neoplasms pathology, Knee Joint pathology, Sarcoma, Synovial diagnosis, Soft Tissue Neoplasms diagnosis
- Abstract
Synovial sarcoma (SS) arising within a knee joint is extremely rare, with 10 reported cases in pediatric and adolescent patients in English literature. Its rarity and nonspecific clinical and radiological features pose a diagnostic challenge. We present two cases of primary intra-articular SS of left knee to enhance awareness of this entity. One patient is a 17-year-old male complained of left knee pain and gait abnormality for 9 years. The other one is a 13-year-old female presented with left knee pain for one year. Both cases were clinically diagnosed as benign joint lesion and underwent biopsies. Histological examination, immunohistochemical staining and molecular study confirmed that both patients had primary intra-articular SS, monophasic spindle cell type. Intraarticular SS should be considered as a potential diagnosis with unexplained long-standing knee pain.
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- 2021
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5. Clinical and molecular characterization of early-onset colorectal cancer.
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Willauer AN, Liu Y, Pereira AAL, Lam M, Morris JS, Raghav KPS, Morris VK, Menter D, Broaddus R, Meric-Bernstam F, Hayes-Jordan A, Huh W, Overman MJ, Kopetz S, and Loree JM
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- Adenocarcinoma genetics, Adenocarcinoma pathology, Adolescent, Adult, Age of Onset, Aged, Colorectal Neoplasms genetics, Colorectal Neoplasms pathology, DNA Mutational Analysis, Female, Follow-Up Studies, Humans, Incidence, Male, Microsatellite Instability, Middle Aged, Prognosis, Retrospective Studies, United States epidemiology, Young Adult, Adenocarcinoma epidemiology, Biomarkers, Tumor genetics, Colorectal Neoplasms epidemiology, Mutation
- Abstract
Background: Colorectal cancer (CRC) incidence is increasing in adults younger than 50 years. This study evaluated clinical and molecular features to identify those features unique to early-onset CRC that differentiate these patients from patients 50 years old or older., Methods: Baseline characteristics were evaluated according to the CRC onset age with 3 independent cohorts. A fourth cohort was used to describe the impact of age on the consensus molecular subtype (CMS) prevalence., Results: This retrospective review of more than 36,000 patients with CRC showed that early-onset patients were more likely to have microsatellite instability (P = .038), synchronous metastatic disease (P = .009), primary tumors in the distal colon or rectum (P < .0001), and fewer BRAF V600 mutations (P < .001) in comparison with patients 50 years old or older. Patients aged 18 to 29 years had fewer adenomatous polyposis coli (APC) mutations (odds ratio [OR], 0.56; 95% confidence interval [CI], 0.35-0.90; P = .015) and an increased prevalence of signet ring histology (OR, 4.89; 95% CI, 3.23-7.39; P < .0001) in comparison with other patients younger than 50 years. In patients younger than 40 years, CMS1 was the most common subtype, whereas CMS3 and CMS4 were uncommon (P = .003). CMS2 was relatively stable across age groups. Early-onset patients with inflammatory bowel disease were more likely to have mucinous or signet ring histology (OR, 5.54; 95% CI, 2.24-13.74; P = .0004) and less likely to have APC mutations (OR, 0.24; 95% CI, 0.07-0.75; P = .019) in comparison with early-onset patients without predisposing conditions., Conclusions: Early-onset CRC is not only distinct from traditional CRC: special consideration should be given to and further investigations should be performed for both very young patients with CRC (18-29 years) and those with predisposing conditions. The etiology of the high rate of CMS1 in patients younger than 40 years deserves further exploration., (© 2019 American Cancer Society.)
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- 2019
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6. Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival.
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Subbiah V, Lamhamedi-Cherradi SE, Cuglievan B, Menegaz BA, Camacho P, Huh W, Ramamoorthy V, Anderson PM, Pollock RE, Lev DC, Qiao W, McAleer MF, Benjamin RS, Patel S, Herzog CE, Daw NC, Feig BW, Lazar AJ, Hayes-Jordan A, and Ludwig JA
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- Adult, Combined Modality Therapy, Desmoplastic Small Round Cell Tumor genetics, Desmoplastic Small Round Cell Tumor pathology, Desmoplastic Small Round Cell Tumor surgery, Disease-Free Survival, Female, Humans, Male, RNA-Binding Protein EWS genetics, Sarcoma, Ewing genetics, Sarcoma, Ewing pathology, Sarcoma, Ewing surgery, Young Adult, Cytoreduction Surgical Procedures, Desmoplastic Small Round Cell Tumor drug therapy, Sarcoma, Ewing drug therapy
- Abstract
Purpose: Desmoplastic small round cell tumor (DSRCT), which harbors EWSR1-WT1 t(11;22)(p13:q12) chromosomal translocation, is an aggressive malignancy that typically presents as intra-abdominal sarcomatosis in young males. Given its rarity, optimal treatment has not been defined. Experimental Design: We conducted a retrospective study of 187 patients with DSRCT treated at MD Anderson Cancer Center over 2 decades. Univariate and multivariate regression analyses were performed. We determined whether chemotherapy, complete cytoreductive surgery (CCS), hyperthermic intraperitoneal cisplatin (HIPEC), and/or whole abdominal radiation (WART) improve overall survival (OS) in patients with DSRCT. Critically, because our institutional practice limits HIPEC and WART to patients with less extensive, potentially resectable disease that had benefited from neoadjuvant chemotherapy, a time-variant analysis was performed to evaluate those adjunct treatment modalities. Results: The pre-2003 5-year OS rate of 5% has substantially improved to 25% with the advent of newer chemotherapies and better surgical and radiotherapy techniques (HR, 0.47; 95% CI, 0.29-0.75). Chemotherapy response (log rank P = 0.004) and CCS (log rank P < 0.0001) were associated with improved survival. Although WART and HIPEC lacked statistical significance, our study was not powered to detect their potential impact upon OS. Conclusions: Improved 3- and 5-year OS were observed following multidisciplinary treatment that includes Ewing sarcoma (ES)-based chemotherapy and complete tumor cytoreductive surgery, but few if any patients are cured. Prospective randomized studies will be required to prove whether HIPEC or WART are important. In the meantime, chemotherapy and CCS remain the cornerstone of treatment and provide a solid foundation to evaluate new biologically targeted therapies. Clin Cancer Res; 24(19); 4865-73. ©2018 AACR ., (©2018 American Association for Cancer Research.)
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- 2018
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7. Ewing's Sarcoma of the Cervical Spine.
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Dahm PH, Bhattacharjee M, Huh W, and Thapar V
- Abstract
In this article, we present the case of a 6-year-old female presented to the emergency department with progressive ascending motor weakness leading to cardiac arrest. The recent medical history included neck trauma 1 month prior to admission, 2 weeks of subjective fevers, and 1 day of urinary incontinence. After stabilization, and a review of the recent signs and symptoms, a magnetic resonance imaging of the neck revealed a posterior neck mass from C2 to T2. Neurosurgical removal of the mass was consistent with Ewing's sarcoma. Neck pain is a common presentation in the pediatric population, with the most common cause being traumatic. When coupled with neurological deficits, further studies are warranted to evaluate for organic causes.
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- 2018
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8. Clinical Activity of Pazopanib in Patients with Advanced Desmoplastic Small Round Cell Tumor.
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Menegaz BA, Cuglievan B, Benson J, Camacho P, Lamhamedi-Cherradi SE, Leung CH, Warneke CL, Huh W, Subbiah V, Benjamin RS, Patel S, Daw N, Hayes-Jordan A, and Ludwig JA
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- Adolescent, Adult, Antineoplastic Agents adverse effects, Child, Desmoplastic Small Round Cell Tumor pathology, Female, Humans, Indazoles, Male, Middle Aged, Pyrimidines adverse effects, Retrospective Studies, Salvage Therapy, Sulfonamides adverse effects, Survival Analysis, Treatment Outcome, Young Adult, Antineoplastic Agents therapeutic use, Desmoplastic Small Round Cell Tumor drug therapy, Pyrimidines therapeutic use, Sulfonamides therapeutic use
- Abstract
Background: Desmoplastic small round cell tumor (DSRCT) is an aggressive, often fatal soft tissue sarcoma that lacks an optimal salvage regimen. We retrospectively reviewed data from 29 pretreated DSRCT patients who received pazopanib at MD Anderson Cancer Center after failure of standard chemotherapies., Subjects, Materials, and Methods: Medical records of patients treated from January 2012 to December 2016 were reviewed and regression analyses were performed. Median progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method and differences in survival were assessed by a log-rank test. A landmark statistical analysis was used to assess OS at a predefined 12-week time point following pazopanib initiation., Results: The mean age at pazopanib treatment was 27.5 years (range, 6.3-50.1 years). According to RECIST 1.1 criteria, 16 patients (55%) had stable disease, 1 patient (3%) had partial response, 1 patient (3%) had complete response, and 11 patients (38%) had progressive disease. Estimated median PFS was 5.63 months (95% confidence interval [CI]: 3.23-7.47). Median OS was 15.7 months (95% CI: 10.3-32.4). As of December 2016, 11 patients (38%) were still alive, with a median follow-up time of 16.8 (range 3.8-30.1) months. Doses between 400 and 800 mg were included. Pazopanib was well tolerated and 23 (79%) of the patients continued it until progression or death, 4 discontinued because of side effects, and 2 were still on pazopanib at the time of data analysis., Conclusion: In the largest study conducted to date in DSRCT, pazopanib was well tolerated and clinically active in heavily pretreated patients who otherwise lack good treatment options., Implications for Practice: Desmoplastic small round cell tumor (DSRCT) is a rare, extremely aggressive soft tissue sarcoma subtype that most commonly occurs in adolescent and young adult males. No DSRCT-specific therapies exist, and for lack of a better treatment approach, current therapies have relied upon U.S. Food and Drug Administration-approved drugs like pazopanib that exhibit clinical activity in other sarcoma subtypes. This article describes the largest experience to date using pazopanib as salvage treatment in heavily pretreated DSRCT patients. Pazopanib was well tolerated and clinically active, surpassing predefined metrics proposed by the European Organization for Research and Treatment of Cancer indicative of "active" sarcoma drugs (5.63 months progression-free survival [PSF], with 62% of the study population achieving progression-free survival at 12 weeks)., Competing Interests: Disclosures of potential conflicts of interest may be found at the end of this article., (© AlphaMed Press 2017.)
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- 2018
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9. Treatment of carcinomatosis using cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in adolescents and young adults.
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Oyeniyi J, Wu J, Liu D, Yao JC, Green H, Albritton K, Huh W, and Hayes-Jordan A
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- Adolescent, Adult, Combined Modality Therapy, Humans, Retrospective Studies, Young Adult, Carcinoma therapy, Chemotherapy, Cancer, Regional Perfusion, Colorectal Neoplasms therapy, Cytoreduction Surgical Procedures, Hyperthermia, Induced
- Abstract
Background: Among colorectal carcinoma patients, approximately 150 patients/year are age 25 years old or younger according to Surveillance, Epidemiology, and End Results Program statistics. Because of lack of screening in their age group, they are at risk to have more advanced disease and have been largely unstudied., Objective: To determine outcome of colon cancer adolescent and young adult patients., Methods: Patients under the age of 26 were retrospectively reviewed from a single institution., Results: The 5-year overall survival rate from the time of the first surgery was .45 (95% confidence interval .17 to .70). The median overall survival for the cohort was 2.98 years. Patients aged 15 to 21 years had a poorer overall survival than patients aged 22 to 25 years (82% survival vs 100% at 2 years and zero vs 56% at 5 years). Five patients underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Three are alive at 82, 36, and 16 weeks after hyperthermic intraperitoneal chemotherapy., Conclusions: Patients less than 21 years with nonsyndromic colorectal carcinoma may have a poorer outcome. Novel, more aggressive therapy may be necessary in these patients., (Copyright © 2015 Elsevier Inc. All rights reserved.)
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- 2015
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10. Outcome of patients with localized orbital sarcoma who relapsed following treatment on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III and -IV, 1984-1997: a report from the Children's Oncology Group.
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Raney B, Huh W, Hawkins D, Hayes-Jordan A, Million L, Rodeberg D, Teot L, and Anderson J
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- Adolescent, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Child, Child, Preschool, Clinical Trials, Phase III as Topic, Clinical Trials, Phase IV as Topic, Combined Modality Therapy, Female, History, Ancient, Humans, Male, Neoplasm Recurrence, Local pathology, Orbital Neoplasms pathology, Orbital Neoplasms therapy, Radiotherapy, Research Report, Retrospective Studies, Sarcoma pathology, Sarcoma therapy, Treatment Outcome, Neoplasm Recurrence, Local mortality, Orbital Neoplasms mortality, Sarcoma mortality
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Background: We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997., Procedure: Retrospective chart review., Results: Twenty-four of 188 patients (12.8%) developed local (n = 22) or distant relapse (n = 2) at 0.057-7.05 years (median, 1.58) after enrollment. Ages at study entry were 0.14-17 years (median, 5 years). Initial tumor operations included biopsy (n = 20) or gross resection with microscopic residual (n = 4). Initial tumor diameters were 0.5-7 cm (median, 3). Pathologic subtypes were embryonal rhabdomyosarcoma (ERMS, n = 19), sarcoma not otherwise specified (n = 2), and alveolar RMS, botryoid ERMS, or undifferentiated sarcoma (n = 1 each). Initial treatment included vincristine/dactinomycin (n = 24) including an alkylator (n = 4) and radiotherapy (RT, n = 21). Twenty patients responded, 14 completely, 6 partially. After recurrence, patients underwent orbital exenteration (n = 10), enucleation (2), tumor excision (3), or biopsy (1); 7 had no operation, and 1 had no data. Post-relapse chemotherapy included combinations of etoposide (n = 14 patients), doxorubicin (14), ifosfamide (12), cyclophosphamide (7), and dacarbazine (n = 1). Six patients received RT, including four previously treated and two not irradiated initially. Two patients died; one at 1.79 years after contralateral brain metastasis followed by local recurrence, and another at 2.49 years after multiple local recurrences. Twenty-two patients (91.7%) survived sarcoma-free for 0.04-17 years (median, 6.9) after relapse, and 18 of 22 (82%) were alive ≥5 years after relapse., Conclusion: Survival following recurrent localized orbital sarcoma appears likely after vigorous re-treatment given with curative intent., (Copyright © 2012 Wiley Periodicals, Inc.)
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- 2013
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11. Malnutrition and obesity in pediatric oncology patients: causes, consequences, and interventions.
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Co-Reyes E, Li R, Huh W, and Chandra J
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- Behavior Therapy, Child, Child Nutrition Disorders diagnosis, Child Nutrition Disorders therapy, Humans, Nutritional Support, Obesity complications, Obesity therapy, Risk Factors, Child Nutrition Disorders etiology, Neoplasms complications
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In children with cancer, suboptimal nutrition states are common consequences of the disease and its treatment. These nutrition states have been attributed to a number of etiologies dependent on the patient's tumor type and treatment, and are associated with increased morbidity and mortality. Interventions vary from psychosocial to pharmacological and surgical management. Further research is necessary to understand the epidemiology and etiology of these nutrition states. Of great importance is the development and implementation of effective interventions to optimize nutritional status among children with cancer during and after therapy., (Copyright © 2012 Wiley Periodicals, Inc.)
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- 2012
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12. Minor salivary gland malignancies in the pediatric population.
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Galer C, Santillan AA, Chelius D, Huh W, El-Naggar A, Hanna E, Weber RS, and Kupferman ME
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- Adolescent, Child, Disease-Free Survival, Female, Humans, Male, Prognosis, Retrospective Studies, Salivary Gland Neoplasms mortality, Salivary Gland Neoplasms therapy, Survival Analysis, Treatment Outcome, Salivary Gland Neoplasms epidemiology, Salivary Glands, Minor pathology
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Background: Minor salivary gland malignancies in children are rare and data on treatment and outcomes are limited., Methods: A retrospective chart review of all pediatric patients with the pathologic diagnosis of minor salivary gland malignancy at a tertiary care cancer hospital was used to conduct this review., Results: From 1952 to 2006, 35 children with minor salivary gland cancers were treated at The University of Texas MD Anderson Cancer Center. Mean age was 15.2 +/- 2.9 years with a slight female predominance. Recurrence occurred in 4 patients and was significantly associated with positive margins, advanced stage, and high histologic grade. Overall survival (OS) and disease-specific survival (DSS) were 89.3% and 88.4%, respectively, at 5 years. Advanced stage, positive margins, and high grade were associated with adverse survival., Conclusion: Minor salivary gland malignancies in children are rare. Surgical resection with clear margins yields excellent outcomes in patients with low-intermediate grade and early stage tumors. Patients with high-grade malignancies do poorly despite multimodality therapy., (Copyright © 2012 Wiley Periodicals, Inc.)
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- 2012
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13. Primacy of surgery in the management of mucoepidermoid carcinoma in children.
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Ryan JT, El-Naggar AK, Huh W, Hanna EY, Weber RS, and Kupferman ME
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- Adolescent, Carcinoma, Mucoepidermoid pathology, Child, Disease-Free Survival, Female, Humans, Male, Parotid Neoplasms pathology, Parotid Neoplasms surgery, Salivary Gland Neoplasms pathology, Carcinoma, Mucoepidermoid surgery, Salivary Gland Neoplasms surgery
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Background: Epithelial salivary gland neoplasms are rare in children. Malignant tumors account for 30% to 50% of cases in the pediatric age group, with mucoepidermoid carcinoma as the most common histology., Methods: A retrospective medical record review was conducted from 1953 to 2007 to identify patients with mucoepidermoid carcinoma at the age of 18 years or younger at the time of diagnosis. Forty-nine patients were identified. Their medical records were examined for presentation, treatment, pathologic features, and outcomes., Results: Forty-nine pediatric patients with mucoepidermoid carcinoma were identified. The parotid gland (49%) and oral cavity (35%) were the most common subsites. Nodal metastasis was seen in 24% of patients. All patients underwent surgery, and 11 patients (22%) were treated with radiation therapy. The 5-year overall survival was 98%, the 10-year overall survival was 94%, and 10% of patients developed recurrence., Conclusion: Mucoepidermoid carcinoma in children carries a favorable prognosis and can be successfully treated with surgery alone in most cases., (Copyright © 2011 Wiley Periodicals, Inc.)
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- 2011
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14. Outcomes of pediatric patients with malignancies of the major salivary glands.
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Kupferman ME, de la Garza GO, Santillan AA, Williams MD, Varghese BT, Huh W, Roberts D, and Weber RS
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- Adenocarcinoma pathology, Adolescent, Adult, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Carcinoma, Adenoid Cystic pathology, Carcinoma, Mucoepidermoid pathology, Carcinoma, Squamous Cell pathology, Child, Child, Preschool, Combined Modality Therapy, Female, Follow-Up Studies, Humans, Male, Neoplasm Staging, Radiotherapy Dosage, Retrospective Studies, Salivary Gland Neoplasms pathology, Survival Rate, Treatment Outcome, Young Adult, Adenocarcinoma therapy, Carcinoma, Adenoid Cystic therapy, Carcinoma, Mucoepidermoid therapy, Carcinoma, Squamous Cell therapy, Salivary Gland Neoplasms therapy
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Background: To report the outcomes and early to long term treatment complications among pediatric patients with major salivary gland malignancies treated at a single institution., Materials and Method: This study was a retrospective case review set at a tertiary referral cancer center. Patients less than 19 years of age with a diagnosis of a major salivary gland malignancy were identified at the M. D. Anderson tumor database between 1953 and 2006., Results: A total of 61 patients were identified, with equal gender distribution. The majority of tumors arose in the parotid gland (83%), and the most common pathology was mucoepidermoid carcinoma (46%). Lymphatic metastasis was identified in 37% of patients, nearly all with mucoepidermoid carcinoma. Although 65% of patients had prior treatment elsewhere, more than 75% of patients underwent surgical resection at our institution. External beam radiation was used in 45% of patients, with an average dose of 58.6 Gray. Average patient follow-up was 153 months. The overall survival rate was 93% at 5 years, and 26% developed a recurrence. A second primary was identified in 2 patients. Permanent facial paresis was noted in 7 patients (12%) and xerostomia in 1 patient (4%)., Conclusions: Survival of pediatric patients with major salivary gland carcinomas is favorable. Adverse outcomes were best predicted by tumor grade, margin status, and neural involvement. Radiation therapy is beneficial for locoregional control of disease, with acceptable long-term treatment sequelae, and without a significant risk for developing second primary tumors. Survivorship issues need to be addressed in this patient population into adulthood.
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- 2010
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15. Can anthracycline therapy for pediatric malignancies be less cardiotoxic?
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Fulbright JM, Huh W, Anderson P, and Chandra J
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- Adolescent, Adult, Anthracyclines administration & dosage, Anthracyclines analysis, Child, Delayed-Action Preparations administration & dosage, Delayed-Action Preparations toxicity, Disease Progression, Doxorubicin administration & dosage, Doxorubicin adverse effects, Drug Administration Schedule, Drug Carriers administration & dosage, Drug Carriers toxicity, Drug Dosage Calculations, Heart physiopathology, Heart Diseases chemically induced, Heart Diseases prevention & control, Heart Failure chemically induced, Heart Failure prevention & control, Humans, Iron Chelating Agents administration & dosage, Randomized Controlled Trials as Topic, Razoxane administration & dosage, Risk Factors, Treatment Outcome, Anthracyclines antagonists & inhibitors, Drug-Related Side Effects and Adverse Reactions prevention & control, Heart drug effects, Leukemia, Myeloid, Acute drug therapy, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy
- Abstract
Anthracyclines have a central role in the treatment of cancer in pediatric patients but confer an increased risk of cardiac dysfunction. Several strategies have been employed to help reduce anthracycline-induced cardiotoxicity, including pretreating the patient with the iron chelator dexrazoxane and infusing the dose of anthracycline over a longer period. Much focus has also been placed on the development of methods that decrease the toxicity of parent compounds, specifically through the use of drug carriers such as liposomes, and on the development of new, potentially less toxic anthracycline derivatives, such as amrubicin and pixantrone. We provide a review of these strategies, focusing on studies in pediatric patients when available, and support the idea that anthracycline therapy can be less cardiotoxic in pediatric patients.
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- 2010
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16. Modern multimodality therapy for pediatric nonorbital parameningeal sarcomas.
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Zevallos JP, Jain K, Roberts D, Santillan AA, Huh W, Hanna EY, and Kupferman ME
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- Adolescent, Chemotherapy, Adjuvant, Child, Child, Preschool, Disease-Free Survival, Epistaxis etiology, Female, Humans, Lymphatic Metastasis, Male, Nasal Cavity pathology, Nasal Cavity surgery, Nasal Obstruction etiology, Nasopharyngeal Neoplasms mortality, Nasopharyngeal Neoplasms pathology, Neoplasm Recurrence, Local, Nose Neoplasms mortality, Nose Neoplasms pathology, Radiotherapy, Adjuvant, Retrospective Studies, Sarcoma mortality, Sarcoma pathology, Nasopharyngeal Neoplasms therapy, Nose Neoplasms therapy, Sarcoma therapy
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Background: Nonorbital parameningeal sarcomas are rare, aggressive tumors that arise predominantly in the pediatric population. The purpose of this study was to review their management and to clarify the role of surgery in their treatment., Methods: Treatment, prognostic factors, and outcomes for patients over a 10-year period were reviewed., Results: In all, 34 patients were identified. The most common tumor site was the maxillary sinus, and 73% were rhabdomyosarcoma. Six patients (18%) presented with positive nodal metastases and 3 patients (10%) presented with distant metastases. Overall, 18 patients were treated surgically. Locoregional recurrence rate was 43%; no patients developed distant recurrence. Five-year overall survival was 66.8%, disease-specific survival was 68.1%, and disease-free survival was 31.9%., Conclusions: Pediatric nonorbital parameningeal sarcomas require aggressive multimodality treatment. Although chemotherapy and radiation constitute the treatment of choice in most cases, surgery may have a role as salvage therapy and in cases that fail to respond to chemoradiation.
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- 2010
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17. Efficacy of adjuvant and neoadjuvant therapies for adult orbital sarcomas.
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Savar A, Trent J, Al-Zubidi N, Huh W, Anderson P, Shinder R, and Esmaeli B
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- Adult, Aged, 80 and over, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Female, Follow-Up Studies, Humans, Male, Middle Aged, Ophthalmologic Surgical Procedures, Orbital Neoplasms mortality, Orbital Neoplasms pathology, Radiotherapy, Sarcoma mortality, Sarcoma pathology, Survival Rate, Treatment Outcome, Visual Acuity physiology, Young Adult, Chemotherapy, Adjuvant, Neoadjuvant Therapy, Orbital Neoplasms therapy, Sarcoma therapy
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Purpose: To report a series of adults with orbital soft tissue sarcoma treated with various combinations of chemotherapy, radiation therapy, and surgery and to report on the efficacy of neoadjuvant therapy in these patients., Methods: The medical records of adults who presented to our institution with orbital soft tissue sarcoma between 2003 and 2008 were reviewed. Outcome measures reviewed included response to chemotherapy, type of surgery, length of follow-up, visual acuity at last follow-up, local recurrence, distant metastasis, disease-free interval, and death., Results: Thirteen patients were identified. Nine had primary orbital lesions, 1 had a locally recurrent orbital lesion, 1 had a secondary tumor extending from the paranasal sinuses, and 2 had metastases in the orbit from primary tumors at other sites. Six patients (46%) had chemotherapy, and 10 (77%) had external-beam radiation therapy; 12 patients (92%) underwent surgical resection. The mean follow-up time for all patients was 26 months (range, 2 months -7 years). Three patients (23%) received preoperative chemotherapy with or without radiation therapy. Two of these patients underwent globe-preserving surgery, and 1 required an orbital exenteration due to the extent of disease even after chemoreduction., Conclusion: Adults with orbital sarcomas may benefit from preoperative chemotherapy with or without radiation therapy. Multidisciplinary care with involvement of medical oncologists and radiation oncologists who are familiar with sarcoma chemotherapy and radiation may benefit these patients.
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- 2010
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18. Thyroid cancer in young adults.
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Ying AK, Huh W, Bottomley S, Evans DB, and Waguespack SG
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- Adult, Age Factors, Carcinoma, Medullary epidemiology, Carcinoma, Medullary pathology, Carcinoma, Medullary surgery, Female, Humans, Pregnancy, Pregnancy Complications, Neoplastic, Treatment Outcome, Thyroid Neoplasms epidemiology, Thyroid Neoplasms pathology, Thyroid Neoplasms therapy
- Abstract
The incidence of thyroid cancer in young adults is rising. Differentiated carcinoma (ie, papillary, follicular, and their variants) and medullary thyroid carcinoma (MTC) represent the two most common subtypes, with differing etiologies, prognoses, and management strategies. Ultrasound (US)-guided fine needle aspiration (FNA) is the best initial test for evaluating a nodule or mass suspicious for malignancy. Tumor histology, in addition to radiographic findings and clinical presentation, guides surgical management, the need for adjuvant therapies, and the optimal approach to long-term follow-up. Radioactive iodine (RAI) is used to reduce recurrence and improve survival for differentiated thyroid carcinomas (DTCs). Emerging systemic therapies provide options for patients with progressive metastatic MTC or radio-resistant DTC. Overall, the prognosis for the most common thyroid malignancy, papillary thyroid carcinoma (PTC), is excellent. The treatment of young adult thyroid cancer patients occurs optimally as part of a multidisciplinary coordination of care.
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- 2009
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19. Novel bone cancer drugs: investigational agents and control paradigms for primary bone sarcomas (Ewing's sarcoma and osteosarcoma).
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Anderson P, Kopp L, Anderson N, Cornelius K, Herzog C, Hughes D, and Huh W
- Subjects
- Animals, Antineoplastic Agents immunology, Drug Evaluation, Preclinical, Humans, Immunotherapy, Neoplasm Metastasis pathology, Osteosarcoma diagnostic imaging, Osteosarcoma immunology, Osteosarcoma pathology, Radiography, Sarcoma, Ewing diagnostic imaging, Sarcoma, Ewing immunology, Sarcoma, Ewing pathology, Antineoplastic Agents therapeutic use, Osteosarcoma drug therapy, Sarcoma, Ewing drug therapy
- Abstract
Background: New investigational agents and chemotherapy regimens including cyclophosphamide + topotecan, temozolomide + irinotecan, and anti-IGF-1R antibodies in Ewing's sarcoma (ES) and liposomal muramyltripeptide phosphatidylethanolamine (L-MTP-PE), aerosol therapy, and bone-specific agents in osteosarcoma (OS) may improve survival and/or quality of life on 'continuation' therapy., Objective: Review of investigational approaches and control paradigms for recurrent or metastatic primary bone tumors., Methods: Analyze temozolomide + irinotecan data and review in the context of other newer approaches including antiangiogenesis, anti-IGF-1R antibodies and bisphosphonates for ES. Review some current state-of-the-art approaches for OS including L-MTP-PE, anti-IGF-1R inhibition, aerosol therapies and bone specific agents., Results/conclusion: L-MTP-PE with chemotherapy in OS has been shown to improve survival; compassionate access is available for recurrence and/or metastases. Aerosol therapy (granulocyte-macrophage colony stimulating factor, cisplatin, gemcitabine) for lung metastases is a promising approach to reduce systemic toxicity. The bone-specific agents including denosumab (anti-receptor activator of NF-kappaB ligand antibody) and bisphosphonates may have benefit against giant cell tumor, ES and OS. Anti-IGF-1R antibody SCH717454 has preclinical activity in OS but best effectiveness will most likely be in combination with chemotherapy earlier in therapy. Both temozolomide + irinotecan and cyclophosphamide + topotecan combinations are very active in ES and are likely to be tested with anti-IGF-1R antibodies against ES.
- Published
- 2008
- Full Text
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20. Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV, 1978-1997: report from the Children's Oncology Group.
- Author
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Raney B, Anderson J, Breneman J, Donaldson SS, Huh W, Maurer H, Michalski J, Qualman S, Ullrich F, Wharam M, and Meyer W
- Subjects
- Adolescent, Adult, Child, Child, Preschool, Combined Modality Therapy, Female, Head and Neck Neoplasms mortality, Head and Neck Neoplasms pathology, Head and Neck Neoplasms therapy, Humans, Infant, Male, Neoplasm Metastasis therapy, Prognosis, Rhabdomyosarcoma mortality, Survival Analysis, Treatment Outcome, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Neoplasm Metastasis pathology, Rhabdomyosarcoma pathology, Rhabdomyosarcoma therapy
- Abstract
Purpose: Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II-IV., Patients: We identified 91 patients in the database, which includes newly diagnosed subjects <21 years old with rhabdomyosarcoma (RMS) and undifferentiated sarcoma, and reviewed their charts in detail., Results: The 54 males and 37 females were <1-19 years at diagnosis. Primary sites were nasopharynx-nasal cavity, middle ear/mastoid and parapharyngeal area ("better" sites, 55%), paranasal sinus and infratemporal-pterygopalatine area ("worse" sites, 42%), and other (3%). Sixty-eight percent of informative patients had direct intracranial extension. Major metastatic sites at diagnosis were lung (63%), bone marrow (33%), and bone (27%). Treatment included vincristine, actinomycin D, and cyclophosphamide (VAC) chemotherapy and radiotherapy to the primary tumor and up to five metastatic sites/tissues., Outcome: The estimated 10-year failure-free survival (FFS) rate was 32% (95% confidence interval [CI]: 22%, 42%). Sixty patients had progressive disease (N = 49) or death as a first event (N = 11); another developed myelodysplastic syndrome and died. Sites of first progression/relapse were distant (55%), local (12%), CNS extension (8%), mixed (6%), and uncertain (18%). Factors indicating likelihood of 10-year FFS included tumor arising in "better" versus "worse" sites (FFS 46% vs. 18%, P = 0.02) and embryonal versus other histology (FFS 37% vs. 19%, P = 0.06)., Conclusions: Cure was possible for some patients with metastatic cranial parameningeal sarcoma. Patients with the best outlook had embryonal RMS located in the nasopharynx/nasal cavity, middle ear/mastoid, or parapharyngeal region. Distant metastases were the most frequent type of recurrence, indicating that more effective systemic agents are needed to eliminate residual disease., ((c) 2008 Wiley-Liss, Inc.)
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- 2008
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21. Multimodality treatment of osteosarcoma: radiation in a high-risk cohort.
- Author
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Mahajan A, Woo SY, Kornguth DG, Hughes D, Huh W, Chang EL, Herzog CE, Pelloski CE, and Anderson P
- Subjects
- Adolescent, Adult, Bone Neoplasms drug therapy, Bone Neoplasms radiotherapy, Bone Neoplasms secondary, Child, Cisplatin administration & dosage, Cohort Studies, Combined Modality Therapy, Doxorubicin administration & dosage, Etoposide administration & dosage, Female, Follow-Up Studies, Humans, Ifosfamide administration & dosage, Male, Medical Records, Methotrexate administration & dosage, Neoplasm Recurrence, Local drug therapy, Neoplasm Recurrence, Local radiotherapy, Osteosarcoma drug therapy, Osteosarcoma pathology, Osteosarcoma radiotherapy, Radiotherapy Dosage, Retrospective Studies, Risk Factors, Survival Rate, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Bone Neoplasms therapy, Osteosarcoma therapy
- Abstract
Purpose: Chemotherapy during radiation and/or bone-seeking radioisotope therapy (153-samarium; 1 mCi/kg) during radiation may improve osteosarcoma cancer control., Patients and Methods: We analyzed our preliminary radiation experience in high-risk, metastatic, and/or recurrent patients during a consecutive period of 20 months (May 2005-December 2006)., Results: Thirty-nine high-risk osteosarcoma patients had radiotherapy; 119 sites were irradiated. A median four sites were irradiated per patient (range 1-14). The median radiation dose and number of fractions of radiation was 30 Gy in 10 fractions (range 10-70 Gy in 4-35 fractions). Chemotherapy, most commonly ifosfamide or methotrexate, was used in 80% (100/119) radiotherapy courses. Of 38 painful sites, 29 had improvement (76%), 4 had no change (10%), and 5 had more pain (13%). Objective and potentially durable responses were documented using PET-CT and bone scans with persistent and sustained reduction of standard uptake values (SUVs; initial SUV of indication lesion 9.5 became <4 at all subsequent time points) and serial bone scans [improvement in 29/39 (72%); stable 10/39 (25%), worse 1/39 (3%)]. The actuarial 4-year survival from development of metastasis was 39%., Conclusions: Our early results suggest that the use of multimodality therapy including chemotherapy with radiation in unresectable osteosarcoma may be beneficial., ((c) 2008 Wiley-Liss, Inc.)
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- 2008
- Full Text
- View/download PDF
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