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Adult rhabdomyosarcoma

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https://www.readbyqxmd.com/read/29790086/testosterone-mediated-activation-of-androgenic-signalling-sustains-in-vitro-the-transformed-and-radioresistant-phenotype-of-rhabdomyosarcoma-cell-lines
#1
S Giannattasio, F Megiorni, V Di Nisio, A Del Fattore, R Fontanella, S Camero, C Antinozzi, C Festuccia, G L Gravina, S Cecconi, C Dominici, L Di Luigi, C Ciccarelli, P De Cesaris, A Riccioli, B M Zani, A Lenzi, R G Pestell, A Filippini, C Crescioli, V Tombolini, F Marampon
PURPOSE: Rhabdomyosarcoma (RMS), the most common soft-tissue sarcoma in childhood, rarely affects adults, preferring male. RMS expresses the receptor for androgen (AR) and responds to androgen; however, the molecular action of androgens on RMS is unknown. METHODS: Herein, testosterone (T) effects were tested in embryonal (ERMS) and alveolar (ARMS) RMS cell lines, by performing luciferase reporter assay, RT-PCR, and western blotting experiments. RNA interference experiments or bicalutamide treatment was performed to assess the specific role of AR...
May 22, 2018: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/29777888/primary-intracranial-rhabdomyosarcoma-in-the-cerebellopontine-angle-resected-after-preoperative-embolization
#2
Keisuke Yoshida, Tomoru Miwa, Takenori Akiyama, Masaaki Nishimoto, Dai Kamamoto, Kazunari Yoshida
BACKGROUND: Primary intracranial rhabdomyosarcoma is a very rare malignant tumor. Due to moderately vascular and firm characteristics of the tumor, complete removal without complications is often difficult. Moreover, in pediatric patients, the volume of total blood is less than adults and minimal intraoperative hemorrhage is desirable. CASE DESCRIPTION: A 6-year-old boy presented with ataxia and was found to have a large cerebellopontine angle tumor and hydrocephalus...
May 16, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29761260/fetal-type-rhabdomyoma-of-the-soft-palate-in-an-adult-patient-report-of-one-case-and-review-of-the-literature
#3
Zhenjian Cai, Jaiyeola Thomas, Ibrahim Alava, Nfn Aakash, Karan Saluja, Hui Zhu
Rhabdomyoma is a rare benign tumor with skeletal muscle differentiation. Rhabdomyoma is further classified into cardiac, adult, fetal, and genital subtypes. Out of these, fetal type rhabdomyoma (FTR) is the rarest. Only a small number of cases have been recorded in the literature. FTR typically affects male infants and young children and occurs predominantly in the head and neck region. FTR is exceedingly rare in the adult, with less than 30 cases reported. The classic FTR is composed of primitive undifferentiated spindle cells with scant eosinophilic cytoplasm embedded in a myxoid stroma...
May 14, 2018: Head and Neck Pathology
https://www.readbyqxmd.com/read/29750397/fdg-pet-ct-in-the-evaluation-of-primary-and-secondary-pancreatic-malignancies
#4
Casey E Bohl, Sara M Federico, Giles W Robinson, Armita Bahrami, Barry L Shulkin
PURPOSE: Primary pancreatic carcinoma and pancreatic metastases are rare in the pediatric population. Pancreatoblastoma is the most common pancreatic malignant tumor in young children and solid-pseudopapillary tumor in teenagers. Pancreatic adenocarcinoma is extremely rare under the age of 40 and is usually associated with underlying genetic abnormalities. Secondary malignancies of the pancreas occur more frequently than primary pancreatic malignancies in children and are most commonly seen with non-Hodgkin lymphomas (NHL) and mesenchymal sarcomas...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29661049/academic-facility-utilization-and-survival-outcomes-in-adult-head-and-neck-sarcomas-an-ncdb-analysis
#5
Richard B Cannon, Patrick S Carpenter, Dustin Boothe, Luke O Buchmann, Jason P Hunt, Shane Lloyd, Ying J Hitchcock, Jeffrey J Houlton, John R Weis, Hailey M Shepherd, Marcus M Monroe
Objectives To investigate clinicopathologic and treatment factors associated with survival in adult head and neck sarcomas in the National Cancer Database (NCDB). To analyze whether treatment settings and therapies received influence survival outcomes and to compare trends in utilization via an aggregated national data set. Study Design Prospectively gathered data. Setting NCDB. Subjects and Methods The study comprised a total of 6944 adult patients treated for a head and neck sarcoma from January 2004 to December 2013...
April 1, 2018: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29543677/fluorescent-in-situ-hybridization-for-tp53-in-the-diagnosis-of-pediatric-osteogenic-sarcoma
#6
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
June 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29529647/synovial-sarcoma-is-not-associated-with-a-higher-risk-of-lymph-node-metastasis-compared-with-other-soft-tissue-sarcomas
#7
Andrew J Jacobs, Carol D Morris, Adam S Levin
BACKGROUND: Reported rates of the incidence of lymph node metastasis in soft tissue sarcoma vary considerably. Many are based on single-institution series and small patient populations. Certain sarcoma subtypes, including synovial sarcoma, have been associated with a higher risk of lymph node involvement. Most single centers have insufficient numbers of patients to assess lymph node metastasis accurately, but larger national databases may allow a more accurate estimation. QUESTIONS/PURPOSES: We queried a large national database and asked the following questions: (1) What proportion of patients with soft tissue sarcoma have lymph node metastasis and distant metastasis? (2) What histologic subtypes are associated with increased risk of nodal metastasis? (3) What is the impact of lymph node metastases and histologic subtype on survival? (4) Does lymph node excision improve survival of patients with soft tissue sarcoma? METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program is a national database that covers a geographic cross-section representing approximately 28% of the US population across demographic groups...
March 2018: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/29503221/collaborating-with-our-adult-colleagues-a-case-series-of-robotic-surgery-for-suspicious-and-cancerous-lesions-in-children-and-young-adults-performed-in-a-free-standing-children-s-hospital
#8
Briony K Varda, Patricia Cho, Andrew A Wagner, Richard S Lee
BACKGROUND: In adult urologic oncology the use of robotics has become commonplace; in pediatric urology it is rare. Herein, we describe a collaboration between an adult and a pediatric urologist performing robotic surgery for children and young adults with suspicious or cancerous genitourinary (GU) lesions. OBJECTIVES: To evaluate clinical and oncologic outcomes in children and young adults undergoing robotic surgery for suspicious or cancerous lesions of the GU tract; to describe our collaborative model between an adult and pediatric surgeon at a free-standing children's hospital...
March 2, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29475814/complete-genome-sequence-of-a-multi-recombinant-echovirus-6-strain-isolated-from-csf-in-ahvaz-southwestern-iran
#9
Samaneh Abbasi, Manoochehr Makvandi, Ali Teimoori, Alireza Samarbaf-Zadeh
BACKGROUND: Echovirus 6 (E6), is one of the main enteroviral serotypes, was initially isolated from patients with aseptic meningitis (AM) and is a major cause of hospitalization among children and adults worldwide. METHODS: A cerebrospinal fluid (CSF) sample was collected from patient with clinically suspected aseptic meningitis (AM) in August 2011. Following detection of a virus and subsequent virus serotyping, the whole genome sequence was determined. The sequence of the VP1 region of the isolated strain E6 RA/E6/Ahvaz/Iran/2011 showed 79% (>75%) nucleotide and 94% (>85%) amino acid homology with prototype strain D'Amori...
April 2018: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/29452297/lumbar-intraspinal-spindle-cell-rhabdomyosarcoma-as-a-rare-cause-of-spinal-stenosis-a-case-report
#10
Donel A Sequea, Maria E Reese
Rhabdomyosarcoma (RMS) represents the most common sarcoma in childhood yet is extremely rare in adults, with only a handful of cases reported. Here we present a case of intraspinal spindle cell RMS in an adult who presented as a typical case of spinal stenosis. To our knowledge, this is the first reported case of lumbar intraspinal spindle cell RMS in an adult patient. Furthermore, RMS phenotypically presents more aggressively in adults compared with children. LEVEL OF EVIDENCE: To be determined...
February 13, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/29441110/case-of-mucinous-adenocarcinoma-of-the-lung-associated-with-congenital-pulmonary-airway-malformation-in-a-neonate
#11
Juneyoug Koh, Euiseok Jung, Se Jin Jang, Dong Kwan Kim, Byong Sop Lee, Ki-Soo Kim, Ellen Ai-Rhan Kim
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia...
January 2018: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/29375950/embryonal-rhabdomyosarcoma-of-the-cervix-a-rare-disease-at-an-uncommon-age
#12
Uroosa Ibrahim, Amina Saqib, Farhan Mohammad, Juan Ding, Blerina Salman, Fady K Collado, Meekoo Dhar
Embryonal rhabdomyosarcoma (RMS) is a rare type of sarcoma, primarily seen in the pediatric and adolescent population. Three subtypes of embryonal RMS are described, with the botryoid type being the most common. The incidence of this disease in adult females is 0.4% to 1% with the affected age group being patients in the third to fourth decade of life. It is exceedingly rare in patients above 40 years of age. We describe the case of a 48-year-old female, gravida 9 para 5, who presented with abnormal vaginal bleeding and an exophytic mass on examination...
November 21, 2017: Curēus
https://www.readbyqxmd.com/read/29355158/histological-types-of-soft-tissue-sarcomas-at-the-lagos-university-teaching-hospital
#13
Nzechukwu Zimudo Ikeri, Andrea Oludolapo Akinjo, Olugbende O Ajayi, Adekunbiola Aina Fehintola Banjo
OBJECTIVE: There is scanty data on histologically diagnosed soft-tissue sarcomas in the Nigerian literature. This is due to paucity of facilities for ancillary testing as well as a dearth of specialist soft tissue pathologists. Knowledge however of the common soft-tissue sarcomas is vital for the establishment of an effective sarcoma service. The aim of this study, therefore, was to determine the histological spectrum of soft-tissue sarcomas in Lagos, Nigeria. MATERIALS AND METHODS: Archival haematoxylin and eosin (H and E)-stained slides were retrieved and reviewed by a team of soft-tissue pathologists at the Royal National Orthopaedic Hospital, London, UK...
October 2017: Nigerian Postgraduate Medical Journal
https://www.readbyqxmd.com/read/29320751/uterine-rhabdomyosarcoma-in-adults
#14
Andre Pinto, Ryan M Kahn, Andrew E Rosenberg, Brian Slomovitz, Charles Matthew Quick, Michella K Whisman, Marilyn Huang
Rhabdomyosarcoma (RMS) is an aggressive mesenchymal tumor most commonly diagnosed in the pediatric population, and when occurring in adults, tends to develop in the deep soft tissue of the limbs. Primary uterine RMS comprises an even more restricted subset, with little known or reported when compared to most other gynecologic sarcomas. Our goal with this study was to retrospectively evaluate cases from two academic institutions and describe the main histopathologic findings of this rare gynecologic malignancy...
January 7, 2018: Human Pathology
https://www.readbyqxmd.com/read/29230040/congenital-embryonal-rhabdomyosarcoma-caused-by-heterozygous-concomitant-ptch1-and-ptch2-germline-mutations
#15
Julia Taeubner, Triantafyllia Brozou, Nan Qin, Jasmin Bartl, Sebastian Ginzel, Joerg Schaper, Joerg Felsberg, Simone Fulda, Christian Vokuhl, Arndt Borkhardt, Michaela Kuhlen
The sonic hedgehog (SHH) signaling pathway has been shown to play important roles in embryogenesis, cell proliferation as well as in cell differentiation. It is aberrantly activated in various common cancers in adults, but also in pediatric neoplasms, such as rhabdomyosarcoma (RMS) and atypical teratoid/rhabdoid tumors (AT/RTs). Dysregulation and germline mutation in PATCHED1 (PTCH1), a receptor for SHH, is responsible for the Gorlin Syndrome, a familial cancer predisposing syndrome including RMS. Here, we report a newborn diagnosed with congenital embryonal RMS...
January 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29217286/pediatric-protocol-of-multimodal-therapy-is-associated-with-improved-survival-in-ayas-and-adults-with-rhabdomyosarcoma
#16
Trevan D Fischer, Shrawan G Gaitonde, Bradley C Bandera, Mehul V Raval, Sanjeev A Vasudevan, Kenneth W Gow, Elizabeth A Beierle, John J Doski, Adam B Goldin, Monica Langer, Jed G Nuchtern, Stacey Stern, Leland J Foshag, Melanie Goldfarb
BACKGROUND: Multimodal therapy is the standard treatment for pediatric rhabdomyosarcoma, but for adolescents and young adults (AYAs: ages 15-39) and older adults with rhabdomyosarcoma, the use of adjuvant therapy is variable, and survival is greatly decreased compared with younger patients. METHODS: All patients with rhabdomyosarcoma who had a curative operative were identified from the 1998-2012 National Cancer Database. Regression analyses identified independent factors relating to receipt of multimodal therapy (resection + chemotherapy + radiation) and the influence of multimodal therapy on 5-year overall survival...
February 2018: Surgery
https://www.readbyqxmd.com/read/29159540/is-fertility-preservation-safe-for-adult-non-metastatic-gynecologic-rhabdomyosarcoma-patients-systematic-review-and-pooled-survival-analysis-of-137-patients
#17
REVIEW
Maha At Elsebaie, Zeinab Elsayed
PURPOSE: Recently, conservative approaches such as wide local excisions and neoadjuvant chemotherapy are being considered to select young adult females with gynecologic RMS who have strong desire to preserve fertility. This analysis aims to identify prognosticators affecting survival outcomes and defining potential candidacy for fertility-preservation. Another focus is to explore the role of chemotherapy in reducing the need for aggressive surgery and the role of radiotherapy in decreasing rates of local failure...
March 2018: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/29118528/adult-testicular-cancer-two-decades-of-saudi-national-data
#18
REVIEW
Mohammed Abomelha
There is a paucity of data regarding testicular cancer among Saudis as well as the nonexistent of published national data. Furthermore, a substantial increase of the incidence of testicular cancer among Saudis was lately noted. The aim of the study is to determine the trends and patterns of testicular cancer among adult Saudis using national data over a period of 20 years. The national database of the Saudi Cancer Registry (SCR) on testicular cancer over the last two decades was studied including epidemiological and histological patterns...
October 2017: Urology Annals
https://www.readbyqxmd.com/read/29100276/temozolomide-combined-with-irinotecan-caused-regression-in-an-adult-pleomorphic-rhabdomyosarcoma-patient-derived-orthotopic-xenograft-pdox-nude-mouse-model
#19
Kentaro Igarashi, Kei Kawaguchi, Tasuku Kiyuna, Takashi Murakami, Shinji Miwa, Scott D Nelson, Sarah M Dry, Yunfeng Li, Arun S Singh, Hiroaki Kimura, Katsuhiro Hayashi, Norio Yamamoto, Hiroyuki Tsuchiya, Fritz C Eilber, Robert M Hoffman
Adult pleomorphic rhabdomyosarcoma (RMS) is a rare and recalcitrant, highly-malignant mesenchymal tumor in need of improved therapeutic strategies. Our laboratory pioneered the patient-derived orthotopic xenograft (PDOX) nude mouse model with the technique of surgical orthotopic implantation (SOI). We previously described the development of a PDOX model of adult pleomorphic RMS where the tumor behaved similar to the patient donor. A high-grade pleomorphic rhabdomyosarcoma from a striated muscle was previously grown orthotopically in the right biceps-femoris muscle of nude mice to establish the PDOX model...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29098099/life-threatening-irinotecan-induced-toxicity-in-an-adult-patient-with-alveolar-rhabdomyosarcoma-the-role-of-a-ugt1a1-polymorphism
#20
Arnaud Jannin, Benjamin Hennart, Antoine Adenis, Bruno Chauffert, Nicolas Penel
Alveolar rhabdomyosarcoma (AR) in adult patients is an exceptional malignancy. Management of AR is based on (neo)adjuvant chemotherapy combining ifosfamide, vincristine, and actinomycin D and local curative-intent surgery/radiotherapy. In cases of relapsing AR, the combination of temozolomide/irinotecan is regarded as a possible option. Here we describe life-threatening long-lasting toxicity related to the 1st cycle of irinotecan-based chemotherapy in a 56-year-old woman suffering from locally advanced and metastatic head and neck AR...
2017: Case Reports in Oncological Medicine
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