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Adult rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28273971/clinico-pathological-spectrum-of-ophthalmic-lesions-an-experience-in-tertiary-care-hospital-of-central-india
#1
Yashita Gupta, Renuka Gahine, Nighat Hussain, Mohd Jafar Memon
INTRODUCTION: Ophthalmic lesions show varied distribution on the basis of geographical location. Eye being a unique sensory organ needs to be studied both clinically and pathologically. AIM: This study was aimed to evaluate the histomorphological and clinico-pathlological spectrum of ophthalmic lesions at a tertiary care hospital of Central India. MATERIALS AND METHODS: We reviewed all the ophthalmic biopsies performed in the Department of Pathology of our institute between January 2008 and November 2014...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28271871/soft-tissue-sarcomas-in-adolescents-and-young-adults-a-comparison-with-their-paediatric-and-adult-counterparts
#2
REVIEW
Winette T A van der Graaf, Daniel Orbach, Ian R Judson, Andrea Ferrari
Survival outcomes for adolescent and young adult patients with soft tissue sarcomas lag behind those of children diagnosed with histologically similar tumours. To help understand these differences in outcomes, we discuss the following issues with regard to the management of these patients with soft tissue sarcomas: delays in diagnosis, trial availability and participation, aspects of the organisation of care (with an emphasis on age-specific needs), national centralisation of sarcoma care, international consortia, and factors related to tumour biology...
March 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28243595/cns-metastases-from-bone-and-soft-tissue-sarcomas-in-children-adolescents-and-young-adults-are-they-really-so-rare
#3
Monika Bekiesinska-Figatowska, Agnieszka Duczkowska, Marek Duczkowski, Hanna Bragoszewska, Anna Romaniuk-Doroszewska, Beata Iwanowska, Sylwia Szkudlinska-Pawlak, Jaroslaw Madzik, Katarzyna Bilska, Anna Raciborska
Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999-2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28222777/identification-of-somatic-and-germ-line-dicer1-mutations-in-pleuropulmonary-blastoma-cystic-nephroma-and-rhabdomyosarcoma-tumors-within-a-dicer1-syndrome-pedigree
#4
Lorena Fernández-Martínez, José Antonio Villegas, Íñigo Santamaría, Ana S Pitiot, Marta G Alvarado, Soledad Fernández, Héctor Torres, Ángeles Paredes, Pilar Blay, Milagros Balbín
BACKGROUND: DICER1 syndrome is a pediatric cancer predisposition condition causing a variety of tumor types in children and young adults. In this report we studied a family with two relatives presenting a variety of neoplastic conditions at childhood. METHODS: Germ-line mutation screening of the complete coding region of the DICER1 gene in genomic DNA from the proband was performed. The presence of somatic DICER1 mutation and further alterations in driver genes was investigated in genomic DNA obtained from available tumor samples...
February 21, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28211476/novel-roles-for-staufen1-in-embryonal-and-alveolar-rhabdomyosarcoma-via-c-myc-dependent-and-independent-events
#5
Tara E Crawford Parks, Kristen A Marcellus, Jonathan Langill, Aymeric Ravel-Chapuis, Jean Michaud, Kyle N Cowan, Jocelyn Côté, Bernard J Jasmin
Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults. Rhabdomyosarcomas are skeletal muscle-like tumours that typically arise in muscle beds, and express key myogenic regulatory factors. However, their developmental program remains blocked in the proliferative phase with cells unable to exit the cell cycle to fuse into myotubes. Recently, we uncovered a key role for the RNA-binding protein Staufen1 during myogenic differentiation through the regulation of c-myc translation. Given the known implication of c-myc in rhabdomyosarcoma, we hypothesized in the current work that Staufen1 controls rhabdomyosarcoma tumorigenesis...
February 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28194276/a-case-report-of-concurrent-embryonal-rhabdomyosarcoma-and-diffuse-large-b-cell-lymphoma-in-an-adult-without-identifiable-cancer-predisposition
#6
M D Mathias, M V Ortiz, H Magnan, S R Ambati, E K Slotkin, A J Chou, M F Walsh, K Offit, C Moskowitz, A Kentsis, L H Wexler
BACKGROUND: Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin lymphoma. Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood. makes up less than 1% of solid malignancies in adults with around 400 new cases each year in the United States. They have not previously been reported concurrently. CASE PRESENTATION: A 37 year old woman presented with painful enlarging leg mass. Biopsy of the mass was consistent with embryonal rhabdomyosarcoma...
2017: Biomarker Research
https://www.readbyqxmd.com/read/28129857/discovering-novel-oncogenic-pathways-and-new-therapies-using-zebrafish-models-of-sarcoma
#7
M N Hayes, D M Langenau
Sarcoma is a type of cancer affecting connective, supportive, or soft tissue of mesenchymal origin. Despite rare incidence in adults (<1%), over 15% of pediatric cancers are sarcoma. Sadly, both adults and children with relapsed or metastatic disease have devastatingly high rates of mortality. Current treatment options for sarcoma include surgery, radiation, and/or chemotherapy; however, significant limitations exist with respect to the efficacy of these strategies. Strong impetus has been placed on the development of novel therapies and preclinical models for uncovering mechanisms involved in the development, progression, and therapy resistance of sarcoma...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28048825/su-f-t-50-evaluation-of-monte-carlo-simulations-performance-for-pediatric-brachytherapy-dosimetry
#8
C Chatzipapas, P Papadimitroulas, G Loudos, N Papanikolaou, G Kagadis
PURPOSE: Pediatric tumors are generally treated with multi-modal procedures. Brachytherapy can be used with pediatric tumors, especially given that in this patient population low toxicity on normal tissues is critical as is the suppression of the probability for late malignancies. Our goal is to validate the GATE toolkit on realistic brachytherapy applications, and evaluate brachytherapy plans on pediatrics for accurate dosimetry on sensitive and critical organs of interest. METHODS: The GATE Monte Carlo (MC) toolkit was used...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28039349/primary-embryonal-rhabdomyosarcoma-of-the-liver
#9
Aditi Arora, Ritika Jaiswal, Nidhi Anand, Nuzhat Husain
Rhabdomyosarcomas are malignant neoplasms with striated muscle differentiation. This is the most common type of soft-tissue sarcoma in children, but occurs rarely in adults. Its occurrence in liver is infrequent. We report a case of primary hepatic embryonal rhabdomyosarcoma in a 67-year-old man. The tumour was occupying the left lobe of the liver with large component of lesion seen bulging in left subhepatic space indenting over the stomach, compressing the pancreas and gall bladder. A percutaneous biopsy was performed which revealed embryonal rhabdomyosarcoma...
December 30, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/28035748/a-phase-i-study-of-perifosine-with-temsirolimus-for-recurrent-pediatric-solid-tumors
#10
Oren J Becher, Stephen W Gilheeney, Yasmin Khakoo, David C Lyden, Sofia Haque, Kevin C De Braganca, Jill M Kolesar, Jason T Huse, Shakeel Modak, Leonard H Wexler, Kim Kramer, Ivan Spasojevic, Ira J Dunkel
BACKGROUND: The PI3K/AKT/mTOR pathway is aberrantly activated in many pediatric solid tumors including gliomas and medulloblastomas. Preclinical data in a pediatric glioma model demonstrated that the combination of perifosine (AKT inhibitor) and temsirolimus (mTOR inhibitor) is more potent at inhibiting the axis than either agent alone. We conducted this study to assess pharmacokinetics and identify the maximum tolerated dose for the combination. PROCEDURE: We performed a standard 3+3 phase I, open-label, dose-escalation study in patients with recurrent/refractory pediatric solid tumors...
December 30, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28009608/fosb-is-a-useful-diagnostic-marker-for-pseudomyogenic-hemangioendothelioma
#11
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a distinctive vascular neoplasm of intermediate biological potential with a predilection for young adults and frequent multifocal presentation. Pseudomyogenic hemangioendothelioma is characterized by loose fascicles of plump spindled and epithelioid cells with abundant eosinophilic cytoplasm and coexpression of keratins and endothelial markers. Recently, a SERPINE1-FOSB fusion has been identified as a consistent genetic alteration in pseudomyogenic hemangioendothelioma...
December 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27998313/embryonal-rhabdomyosarcoma-of-the-perineum-in-an-adult-a-case-report
#12
Sidy Ka, Freddy Gnangnon, Mamadou Moustapha Dieng, Doudou Diouf, Jaafar Thiam, Pape Macoumba Gaye, Ahmadou Dem
BACKGROUND: We report the case of an adult patient with embryonal rhabdomyosarcoma of the perineum admitted to our practice at Joliot Curie Institute in Dakar. It is a rare tumor at this age and has a bad prognosis at this localization. CASE PRESENTATION: We describe the case of a 22-year-old African man admitted for a perineal mass that had evolved over 6 months. He complained of tenesmus, obstinate constipation, and dysuria. A clinical examination revealed perineal swelling spread over his anus, scrotum, penis, testicles, and inguinal lymph nodes...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27986268/-incidence-and-risk-factors-for-ifosfamide-related-encephalopathy-in-sarcoma-patients
#13
Natacha Stern, Ilyes Sakji, Anne-Sophie Defachelles, Cyril Lervat, Thomas Ryckewaert, Guillaume Marliot, Charlotte Peugniez, Dominique Deplanque, Nicolas Penel
CONTEXT: Ifosfamide remains one of the major cytotoxic drugs for sarcoma management. Ifosfamid-related encephalopathy (IRE) is a rare but severe adverse event, without clearly identified risk factors. METHOD: We have carried out a single-center, retrospective study to assess the occurrence and the risk factors for IRE after the two first cycles of chemotherapy. We have collected the data-describing patients, biological data, tumors characteristics (histology, leptomeningeal metastasis) and ifosfamide administration modalities...
December 13, 2016: Bulletin du Cancer
https://www.readbyqxmd.com/read/27955731/non-rhabdomyosarcoma
#14
REVIEW
Roshni Dasgupta, David Rodeberg
Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group encompassing more than 50 different histological diagnoses arising from primitive mesenchymal tissue. Together, they comprise about half the soft tissue sarcomas diagnosed in children and young adults. Despite each histologies relative rarity, their management schema is similar among the different NRSTS histologies. Surgical management is an important component of the multimodal treatment strategy of all these tumors. Resection with negative margins, while maintaining function, plays an important role as a primary treatment of these patients as well as diminishing the risks of local and distant recurrence...
October 2016: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/27942480/diagnostic-imaging-of-benign-and-malignant-neck-masses-in-children-a-pictorial-review
#15
REVIEW
Ruth Elizabeth Brown, Srikrishna Harave
Neck masses are frequently encountered in pediatric medicine, and can present a diagnostic dilemma for the clinicians involved. There are several means by which neck masses in children can be subdivided, for example by age at presentation, anatomical location including compartments and fascia of the neck, their classical appearance when imaged, or by etiology. When imaging children the clinicians must be mindful of radiation exposure and as such ultrasound (US) is often attempted first. Cross sectional imaging can be helpful for problem solving with CT being particularly useful for assessing the patient in more acute scenarios, for example when there is airway compromise...
October 2016: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/27932889/alveolar-rhabdomyosarcoma-of-the-sphenoid-sinus-mimicking-optic-neuritis-presenting-with-intermittent-visual-loss-in-an-adult
#16
Wendong Liu, Libin Jiang, Yulan Jin, Bentao Yang, Timothy Yy Lai
A 23-year-old male with intermittent visual loss in his left eye for 4 months was originally treated as optic neuritis elsewhere. Upon presentation to our hospital (Beijing Tongren Hospital), his left eye visual acuity was reduced to hand motion at face. The left eye ocular motility was limited in upgaze, downgaze, adduction, and abduction. Computed tomography and magnetic resonance imaging revealed lesions in the sphenoid and ethmoid sinuses, orbital apex and carvernous sinus invading adjacent extraocular muscles...
2016: OncoTargets and Therapy
https://www.readbyqxmd.com/read/27896667/head-and-neck-rhabdomyosarcoma-clinical-and-pathologic-characterization-of-seven-cases
#17
Eleanor Chen, Robert Ricciotti, Neal Futran, Dolphine Oda
Head and neck rhabdomyosarcoma occurs frequently in children and adolescents, and has been well studied in that population. In contrast, it is rare in adults and is not as well characterized clinically and pathologically. Seven cases of adult rhabdomyosarcoma occurring in head and neck were retrieved from the archives of Department of Pathology and Division of Oral Pathology at University of Washington. Radiologic findings and clinical history, as well as pathologic findings from hematoxylin and eosin slides and immunohistochemistry for myogenic markers were reviewed...
November 28, 2016: Head and Neck Pathology
https://www.readbyqxmd.com/read/27894759/pancreaticoduodenectomy-for-pediatric-and-adolescent-pancreatic-malignancy-a-single-center-retrospective-analysis
#18
Erika B Lindholm, Abdulaziz K Alkattan, Sara J Abramson, Anita P Price, Todd E Heaton, Vinod P Balachandran, Michael P La Quaglia
PURPOSE: While pancreaticoduodenectomy (PD) has been extensively studied in adults, there are few data pertaining specifically to pediatric patients. We retrospectively analyzed PD-associated morbidity and mortality in pediatric patients. METHODS: Our analytic cohort included all consecutive patients ≤18years of age treated at our institution from 1993 to 2015 who underwent PD. Patient data (demographics, disease characteristics, surgical and adjuvant treatment, length of hospital stay, and postoperative course) were extracted from the medical records...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27879537/rhabdomyosarcoma-of-the-breast-in-adolescent-and-young-adult-aya-women
#19
Anthony N Audino, Bhuvana A Setty, Nicholas D Yeager
Soft tissue sarcoma constitutes 8% of all tumors in adolescent and young adults (AYA), with rhabdomyosarcoma (RMS) accounting for 5.2% to 6.5% of the soft tissue sarcoma total within this group. AYAs have a higher propensity for metastasis and inferior outcomes. Metastases to the breast have been reported in ∼3% to 6% of RMS cases. A review of our hospital's tumor registry identified cases of RMS diagnosed between January 1, 2004 and December 31, 2013. A total of 46 patients with RMS were identified, having a mean age of 12...
January 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27879517/dux4-immunohistochemistry-is-a-highly-sensitive-and-specific-marker-for-cic-dux4-fusion-positive-round-cell-tumor
#20
Bradford Siegele, Jon Roberts, Jennifer O Black, Erin Rudzinski, Sara O Vargas, Csaba Galambos
The histologic differential diagnosis of pediatric and adult round cell tumors is vast and includes the recently recognized entity CIC-DUX4 fusion-positive round cell tumor. The diagnosis of CIC-DUX4 tumor can be suggested by light microscopic and immunohistochemical features, but currently, definitive diagnosis requires ancillary genetic testing such as conventional karyotyping, fluorescence in situ hybridization, or molecular methods. We sought to determine whether DUX4 expression would serve as a fusion-specific immunohistochemical marker distinguishing CIC-DUX4 tumor from potential histologic mimics...
March 2017: American Journal of Surgical Pathology
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