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Elaine Tan, Mayur D Mody, Nabil F Saba
Laryngeal cancer (LC) remains a challenging disease to treat. The majority of LCs diagnosed worldwide are squamous cell carcinomas (SCC), and current treatment guidelines are designed to address conventional laryngeal SCC. However, several histologically rare tumor types can originate in the larynx. There is a lack of guidelines regarding the best therapeutic approaches to these tumors and their treatment is often modeled after their recommended management at non-laryngeal sites. Understanding the role for systemic therapy in these rare tumors is important, especially for patients with advanced disease or those who are not surgical candidates...
July 2018: Oral Oncology
Adele P Williams, Alicia M Waters, Jerry E Stewart, Venkatram R Atigadda, Elizabeth Mroczek-Musulman, Donald D Muccio, Clinton J Grubbs, Elizabeth A Beierle
BACKGROUND: While patients with early-stage rhabdomyosarcoma (RMS) have seen steady improvement in prognosis over the last 50 y, those with advanced-stage or high-grade disease continue to have a dismal prognosis. Retinoids have been shown to cause growth suppression and terminal differentiation in RMS cells, but the toxicities associated with retinoic acid limit its use. Rexinoids provide an alternative treatment approach to retinoic acid. Rexinoids primarily bind the retinoid X receptor with minimal retinoic acid receptor binding, the entity responsible for many of the toxicities of retinoid therapies...
August 2018: Journal of Surgical Research
Shu-Wei Chou, Hsiu-Hao Chang, Meng-Yao Lu, Yung-Li Yang, Dong-Tsamn Lin, Kai-Hsin Lin, Shiann-Tarng Jou
BACKGROUND: Taiwan Pediatric Oncology Group (TPOG) initiated two consecutive protocols for treating pediatric patients with rhabdomyosarcoma since 1995. However, the results have not been analyzed and reported yet. The aim of this study is to investigate the treatment results of these two protocols in our hospital and to assess whether the results are comparable to other large-scaled studies. METHODS: Treatment of pediatric patients with rhabdomyosarcoma according to TPOG protocols at National Taiwan University Hospital began in 1995...
June 11, 2018: Journal of the Formosan Medical Association, Taiwan Yi Zhi
Bobae Shim, Jiwon Koh, Ji Hye Moon, In Ae Park, Han Suk Ryu
Rhabdomyosarcoma is a malignant soft tissue tumor which shows skeletal muscle differentiation. Leptomeningeal metastasis can occur as a late complication, but currently there are no reports that have documented the cytologic features in cerebrospinal fluid (CSF). We report a case of metastatic alveolar rhabdomyosarcoma diagnosed in the CSF of a 28-year-old male who was originally diagnosed with rhabdomyosarcoma on the neck, and that went through systemic therapy. The tumor was positive for anaplastic lymphoma kinase, but progressed despite additional therapy with crizotinib...
June 14, 2018: Journal of Pathology and Translational Medicine
Bradley A Morganstern, Samantha Scaccia, Wayland Wu, Alex K Williamson, Lane S Palmer
Embryonal rhabdomyosarcoma is a rare cancer that often requires multimodality therapy to treat; however, these therapies can cause changes in the biology of the tumor. Several reports have documented pathologic changes but only recently have genetic changes been mapped. We present case of two separate synchronous primary rhabdomyosarcomas in a 17-month-old patient and discuss the pathophysiology and genetic changes that occur with treatment. We hypothesize that a genetic field defect arising in development of the urogenital sinus caused the tumors, but that treatment modalities may have caused genetic alterations changing clinical behavior of the tumors and responses to treatment...
June 2018: Canadian Journal of Urology
David W Wolff, Min-Hyung Lee, Mathivanan Jothi, Munmun Mal, Fengzhi Li, Asoke K Mal
Alveolar rhabdomyosarcoma (aRMS) is an aggressive subtype of the most common soft tissue cancer in children. A hallmark of aRMS tumors is incomplete myogenic differentiation despite expression of master myogenic regulators such as MyoD. We previously reported that histone methyltransferase KMT1A suppresses MyoD function to maintain an undifferentiated state in aRMS cells, and that loss of KMT1A is sufficient to induce differentiation and suppress malignant phenotypes in these cells. Here, we develop a chemical compound screening approach using MyoD-responsive luciferase reporter myoblast cells to identify compounds that alleviate suppression of MyoD-mediated differentiation by KMT1A...
May 25, 2018: Oncotarget
Lorenzo Angelini, Gianni Bisogno, Ciro Esposito, Marco Castagnetti
The latest multimodal protocols for treatment of bladder/prostate rhabdomyosarcoma (RMS) have shifted the goal of treatment from patient survival to bladder preservation. Consistently, partial resections, such as radical prostatectomy (RP), are favoured when surgery is deemed necessary. We sought to determine the oncological risks - that is, failure to achieve disease control - and the possible benefits in terms of urinary continence associated with RP in RMS patients based on a review of our experience and the data reported in the literature...
June 2018: Therapeutic Advances in Urology
Giulia Bertolini, Luca Bergamaschi, Andrea Ferrari, Salvatore L Renne, Paola Collini, Cecilia Gardelli, Marta Barisella, Giovanni Centonze, Stefano Chiaravalli, Cinzia Paolino, Massimo Milione, Maura Massimino, Michela Casanova, Patrizia Gasparini
BACKGROUND: Rhabdomyosarcomas (RMSs) are the most frequent soft tissue sarcoma in children and adolescents, defined by skeletal muscle differentiation and the status of FOXO1 fusions. In pediatric malignancies, in particular RMS, scant and controversial observations are reported about PD-L1 expression as a putative biomarker and few immune checkpoint clinical trials are conducted. METHODS: PD-L1 assessment was evaluated by immunohistochemistry (IHC) utilizing two anti-PDL1 antibodies, in a pilot cohort of 25 RMS...
June 13, 2018: BMC Cancer
Chunyan Liao, Ryan Beveridge, Jessica J R Hudson, Jacob D Parker, Shih-Chieh Chiang, Swagat Ray, Mohamed E Ashour, Ian Sudbery, Mark J Dickman, Sherif F El-Khamisy
Genomic damage can feature DNA-protein crosslinks whereby their acute accumulation is utilized to treat cancer and progressive accumulation causes neurodegeneration. This is typified by tyrosyl DNA phosphodiesterase 1 (TDP1), which repairs topoisomerase-mediated chromosomal breaks. Although TDP1 levels vary in multiple clinical settings, the mechanism underpinning this variation is unknown. We reveal that TDP1 is controlled by ubiquitylation and identify UCHL3 as the deubiquitylase that controls TDP1 proteostasis...
June 12, 2018: Cell Reports
Ireneusz Dziuba, Paweł Kurzawa, Michał Dopierała, Ana B Larque, Danuta Januszkiewicz-Lewandowska
The last 25 years have brought significant progress in the treatment of sarcomas in children, especially rhabdomyosarcoma (RMS). Nevertheless, treatment failure in some patients results from considerable biological heterogeneity noted in these tumours. RMS, the most common malignant soft tissue neoplasm in children, includes two main subtypes: embryonal (ERMS) and alveolar (ARMS). Due to greater aggressiveness and worse prognosis of ARMS in comparison to ERMS, discrimination between different rhabdomyosarcoma subtypes is of crucial clinical importance...
2018: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
Hui-Min Hu, Xiao-Lin Zhang, Wei-Ling Zhang, Dong-Sheng Huang, Zhong-Dong Du
Background: Cardiotoxicity is one of the most serious chronic complications of anthracyclines therapy. Assessment of the left ventricular ejection fraction (LVEF) fails to detect subtle cardiac dysfunction of left ventricular (LV). This study aimed to detect and evaluate new parameters of subclinical anthracyclines' cardiotoxicity in children with solid tumor. Methods: A detailed echocardiographic examination was performed in 36 children with hepatoblastoma or rhabdomyosarcoma after receiving anthracyclines' chemotherapy and 36 healthy controls from January 2015 to December 2016...
June 20, 2018: Chinese Medical Journal
Alejandro Manzanares, Camilo A Restrepo-Perdomo, Gaia Botteri, Helena Castillo-Ecija, Guillem Pascual-Pasto, Francesc Cano, Laura Garcia-Alvarez, Carles Monterrubio, Bonaventura Ruiz, Manuel Vazquez-Carrera, Mariona Suñol, Jaume Mora, Jose A Tornero, Alejandro Sosnik, Angel M Carcaboso
Delivery of chemotherapy in the surgical bed has shown preclinical activity to control cancer progression upon subtotal resection of pediatric solid tumors, but whether this new treatment is safe for tumor-adjacent healthy tissues remains unknown. Here, Wistar rats are used to study the anatomic and functional impact of electrospun nanofiber matrices eluting SN-38-a potent chemotherapeutic agent-on several body sites where pediatric tumors such as neuroblastoma, Ewing sarcoma, and rhabdomyosarcoma arise. Blank and SN-38-loaded matrices embracing the femoral neurovascular bundle or in direct contact with abdominal viscera (liver, kidney, urinary bladder, intestine, and uterus) are placed...
June 11, 2018: Advanced Healthcare Materials
Nivedita Sharma, Nebu Abraham George, Rajesh Singh, Elizabeth Mathew Iype, Bipin T Varghese, Shaji Thomas
Head and neck soft tissue sarcoma (HNSTS) is a rare neoplasm accounting for 1% of all head and neck tumours. Because of rarity and varied biological behaviour among various subtypes, knowledge about these tumours is limited. This study aimed at analysing clinicopathological, recurrence and survival pattern of surgically treated HNSTS. Case records of 28 patients of HNSTS who underwent surgery at the Regional Cancer Centre (RCC), Trivandrum (India) between 2002 and 2012 were analysed retrospectively for demographic profile, clinical features, treatment given, recurrence pattern and outcome...
June 2018: Indian Journal of Surgical Oncology
Markus Eckstein, Abbas Agaimy, Joachim Woenckhaus, Alexander Winter, Iris Bittmann, Joerg Janzen, Simone Bertz, Florian Haller, Arndt Hartmann
Fibroepithelial polyps of the urinary tract are rare lesions. They occur mainly in the upper urinary tract of children. A high disease prevalence has been reported in families with pleuropulmonary blastoma. Here we present a case of a 46-year-old woman who presented with a giant botryoid fibroepithelial polyp of the urinary bladder. Histologically, the lesion showed prominent botryoid features with an embryonal rhabdomyosarcoma-like cambium layer lacking nuclear or cellular atypia. Immunohistochemical analysis ruled out rhabdomyoblastic differentiation...
June 5, 2018: Human Pathology
Christian Koelsche, Martin Mynarek, Daniel Schrimpf, Luca Bertero, Jonathan Serrano, Felix Sahm, David E Reuss, Yanghao Hou, Daniel Baumhoer, Christian Vokuhl, Uta Flucke, Iver Petersen, Wolfgang Brück, Stefan Rutkowski, Sandro Casavilca Zambrano, Juan Luis Garcia Leon, Rosdali Yesenia Diaz Coronado, Manfred Gessler, Oscar M Tirado, Jaume Mora, Javier Alonso, Xavier Garcia Del Muro, Manel Esteller, Dominik Sturm, Jonas Ecker, Till Milde, Stefan M Pfister, Andrey Korshunov, Matija Snuderl, Gunhild Mechtersheimer, Ulrich Schüller, David T W Jones, Andreas von Deimling
Patients with DICER1 predisposition syndrome have an increased risk to develop pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, and several other rare tumor entities. In this study, we identified 22 primary intracranial sarcomas, including 18 in pediatric patients, with a distinct methylation signature detected by array-based DNA-methylation profiling. In addition, two uterine rhabdomyosarcomas sharing identical features were identified. Gene panel sequencing of the 22 intracranial sarcomas revealed the almost unifying feature of DICER1 hotspot mutations (21/22; 95%) and a high frequency of co-occurring TP53 mutations (12/22; 55%)...
June 7, 2018: Acta Neuropathologica
Clayton B Hess, Daniel J Indelicato, Arnold C Paulino, William F Hartsell, Christine E Hill-Kayser, Stephanie M Perkins, Anita Mahajan, Nadia N Laack, Ralph P Ermoian, Andrew L Chang, Suzanne L Wolden, Victor S Mangona, Young Kwok, John C Breneman, John P Perentesis, Sara L Gallotto, Elizabeth A Weyman, Benjamin V M Bajaj, Miranda P Lawell, Beow Y Yeap, Torunn I Yock
Background/objectives: The Pediatric Proton Consortium Registry (PPCR) was established to expedite proton outcomes research in the pediatric population requiring radiotherapy. Here, we introduce the PPCR as a resource to the oncology community and provide an overview of the data available for further study and collaboration. Design/methods: A multi-institutional registry of integrated clinical, dosimetric, radiographic, and patient-reported data for patients undergoing proton radiation therapy was conceived in May 2010...
2018: Frontiers in Oncology
(no author information available yet)
Findings from a 10-year clinical trial show improved survival rates among children with high-risk rhabdomyosarcoma who receive low-dose maintenance chemotherapy following standard treatment. The results have established a new standard of care for this disease in the EU.
June 7, 2018: Cancer Discovery
Othmane Alaoui
We report the case of a newborn male admitted on the first day of his life with temporo-occipital swelling detected at birth. Clinical examination showed conscious, hemodynamically and respiratory stable, afebrile, vigorous and responsive newborn; craniofacial examination objectified soft, mobile, temporo-occipital mass covered by the scalp measuring 10 cm along its longer left paramedian axis (A). Craniocerebral CT scan showed large heterogeneous left subgaleal parietal-temporal-occipital mass measuring 40/80 mm, suggesting a tumor of soft tissue components of the scalp (B)...
2018: Pan African Medical Journal
Genevieve C Kendall, Sarah Watson, Lin Xu, Collette A LaVigne, Whitney Murchison, Dinesh Rakheja, Stephen X Skapek, Franck Tirode, Olivier Delattre, James F Amatruda
Alveolar rhabdomyosarcoma is a pediatric soft-tissue sarcoma caused by PAX3/7-FOXO1 fusion oncogenes and is characterized by impaired skeletal muscle development. We developed human PAX3-FOXO1 -driven zebrafish models of tumorigenesis and found that PAX3-FOXO1 exhibits discrete cell lineage susceptibility and transformation. Tumors developed by 1.6-19 months and were primitive neuroectodermal tumors or rhabdomyosarcoma. We applied this PAX3-FOXO1 transgenic zebrafish model to study how PAX3-FOXO1 leverages early developmental pathways for oncogenesis and found that her3 is a unique target...
June 5, 2018: ELife
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
AIMS: Infantile fibrosarcoma is characterized by intersecting fascicles of spindle cells and ETV6-NTRK3 gene fusion in most cases. Given histologic overlap with other spindle-cell tumors, the diagnosis can be challenging and often requires molecular confirmation. A recently developed pan-TRK antibody shows promise for identifying tumors with NTRK fusions. The purpose of this study was to evaluate the potential diagnostic utility of pan-TRK immunohistochemistry for infantile fibrosarcoma...
June 4, 2018: Histopathology
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