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Clear cell sarcoma

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https://www.readbyqxmd.com/read/28454214/genome-wide-analysis-of-gynecologic-cancer-the-cancer-genome-atlas-in-ovarian-and-endometrial-cancer
#1
Moito Iijima, Kouji Banno, Ryuichiro Okawa, Megumi Yanokura, Miho Iida, Takashi Takeda, Haruko Kunitomi-Irie, Masataka Adachi, Kanako Nakamura, Kiyoko Umene, Yuya Nogami, Kenta Masuda, Eiichiro Tominaga, Daisuke Aoki
Cancer typically develops due to genetic abnormalities, but a single gene abnormality cannot completely account for the onset of cancer. The Cancer Genome Atlas (CGA) project was conducted for the cross-sectional genome-wide analysis of numerous genetic abnormalities in various types of cancer. This approach has facilitated the identification of novel AT-rich interaction domain 1A gene mutations in ovarian clear cell carcinoma, frequent tumor protein 53 (TP53) gene mutations in high-grade ovarian serous carcinoma, and Kirsten rat sarcoma and B-rapidly accelerated fibrosarcoma proto-oncogene, serine/threonine kinase gene mutations in low-grade ovarian serous carcinoma...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28440018/clear-cell-sarcoma-of-kidney-involving-a-horseshoe-kidney-and-harboring-egfr-internal-tandem-duplication
#2
Teresa Santiago, Michael R Clay, Elizabeth Azzato, Scott Newman, Israel Fernandez-Pineda, Kim E Nichols, Jinghui Zhang, James R Downing, Andrew Davidoff, Rachel C Brennan, David W Ellison
Clear cell sarcoma of kidney (CCSK) is a rare renal malignancy, previously unreported in horseshoe kidney (HSK). B-cell lymphoma 6 corepressor (BCOR) gene internal tandem duplication (ITD) was identified as a recurrent somatic alteration in approximately 85% of CCSKs. This and the YWHAE-NUTM2B/E fusion, the second most common recurrent molecular alteration in CCSK (10%), are considered to be mutually exclusive. However, there is a subset of CCSKs that do not harbor either the BCOR-ITD or YWHAE-NUTM2 translocation and lack known molecular alterations...
April 25, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28438624/bladder-recurrence-of-clear-cell-sarcoma-of-the-kidney-7-years-after-initial-presentation
#3
John Weaver, Tammy Ho, Adam Lang, Joel F Koenig, Douglas E Coplen, Louis Dehner, Erica J Traxel
Clear cell sarcoma of the kidney (CCSK) is the second most common pediatric renal malignancy after Wilms tumor. CCSK has the potential to metastasize to distant sites and was historically known as the bone metastasizing renal tumor. We report an exceedingly rare case of a bladder recurrence of CCSK. Our patient presented with gross hematuria 7 years after initial complete response. He was found to have a large sessile bladder tumor and underwent a partial cystectomy with right pelvic lymph node dissection. Final pathology was metastatic CCSK...
April 21, 2017: Urology
https://www.readbyqxmd.com/read/28426529/diagnostic-utility-of-pax8-pax2-and-ngfr-immunohistochemical-expression-in-pediatric-renal-tumors
#4
Nicoleta C Arva, Jeffrey Bonadio, Elizabeth J Perlman, Mariana M Cajaiba
Pediatric renal tumors (PRT) with small round blue or spindle cell morphology can be diagnostically challenging and only a limited number of immunohistochemical markers have been documented to help in the diagnosis: paired box (Pax) 2 and nerve growth factor receptor (NGFR) positivity have been demonstrated in Wilms tumor (WT) and clear cell sarcoma of the kidney (CCSK), respectively. However, the immunohistochemical expression of these markers in other PRT remains unknown. This study investigated Pax8, Pax2, and NGFR immunophenotype in a large series of PRT...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28421721/fnac-aided-diagnosis-of-clear-cell-sarcoma-of-kidney-report-of-two-cases-in-infants
#5
Indranil Das, Ram Narayan Das, Bibhas Saha Dalal, Janki Bisht, Banhishikha Kanjilal, Uttara Chatterjee
Clear cell sarcoma of kidney (CCSK) is a rare aggressive malignant renal neoplasm with a high metastatic potential. Its outcome has however, improved with the advent of doxorubicin based neoadjuvant chemotherapy. Here, we present two cases of CCSK in infants diagnosed on cytology followed by nephrectomy. The first case presented in the neonatal period and had the unusual histological finding of islands of cartilage. The second case presented at the age of eight months. The possibility of CCSK should, therefore, be considered in the differential diagnoses of renal masses in infants as well as neonates...
April 19, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28416534/pazopanib-associated-posterior-reversible-encephalopathy-syndrome-with-intracerebral-haemorrhage
#6
Cameron Miller-Patterson, Corey R Fehnel
Pazopanib is a tyrosine kinase receptor antagonist used for renal cell carcinoma and soft tissue sarcoma that inhibits tumour growth and angiogenesis. A common side effect of pazopanib is hypertension. We report a case of a 69-year-old woman with clear cell renal cell carcinoma who developed a large right occipital intracerebral haemorrhage 3 weeks after initiating pazopanib. Although this was initially suspected to be a haemorrhagic metastasis, MRI revealed bi-occipital oedema, supporting a diagnosis of posterior reversible encephalopathy syndrome (PRES)...
April 17, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28412212/mucoepidermoid-carcinoma-with-extensive-spindled-morphology-and-melanocytic-marker-expression
#7
Takashi Oide, Kenzo Hiroshima, Yoko Takahashi, Kazunori Fugo, Masanobu Yamatoji, Atsushi Kasamatsu, Yosuke Endo-Sakamoto, Masashi Shiiba, Katsuhiro Uzawa, Hideki Tanzawa, Toshitaka Nagao, Yukio Nakatani
Mucoepidermoid carcinoma (MEC) is the most common malignant neoplasm of the salivary gland. Albeit common, histological variants have rarely been noted in MEC. Here, we report a 49-year-old man with a sublingual gland tumor. Histologically, the tumor was composed of spindle cells arranged in interlacing fascicules or globular nests. A few bland small glands containing mucous cells were also scattered. The spindle tumor cells completely lacked immunoreactivity for cytokeratin, and exhibited immunoreactivity for vimentin, S-100, HMB-45, Melan A, and SOX10...
April 12, 2017: Human Pathology
https://www.readbyqxmd.com/read/28405155/multiple-clear-cell-sarcomas-of-small-intestine-with-parotid-gland-metastasis-a-case-report
#8
Hao Su, Wen-Sheng Liu, Wen-Hao Ren, Peng Wang, Lei Shi, Hai-Tao Zhou
Clear-cell sarcoma is a rare, malignant soft tissue tumor that displays melanocytic differentiation with a distinct molecular profile. It is rarely localized in the gastrointestinal tract. Herein we reported a case of multiple synchronous clear-cell sarcomas of the gastrointestinal tract with parotid gland metastasis. A 51-year-old male patient presented with a growing painless mass under the right ear. A preoperative positron emission tomography/computed tomography showed multiple intestinal masses and a mass in the right parotid with increased glucose uptake, and he underwent operative treatment with resection of three tumors in the jejunum and ileum and then received a right parotidectomy...
March 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28344878/targeting-cytokine-signaling-checkpoint-cis-activates-nk-cells-to-protect-from-tumor-initiation-and-metastasis
#9
Eva M Putz, Camille Guillerey, Kevin Kos, Kimberley Stannard, Kim Miles, Rebecca B Delconte, Kazuyoshi Takeda, Sandra E Nicholson, Nicholas D Huntington, Mark J Smyth
The cytokine-induced SH2-containing protein CIS belongs to the suppressor of cytokine signaling (SOCS) protein family. Here, we show the critical role of CIS in suppressing natural killer (NK) cell control of tumor initiation and metastasis. Cish-deficient mice were highly resistant to methylcholanthrene-induced sarcoma formation and protected from lung metastasis of B16F10 melanoma and RM-1 prostate carcinoma cells. In contrast, the growth of primary subcutaneous tumors, including those expressing the foreign antigen OVA, was unchanged in Cish-deficient mice...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28321815/immune-therapy-for-sarcomas
#10
Peter M Anderson
Absolute lymphocyte count (ALC) recovery rapidly occurring at 14 days after start of chemotherapy for osteosarcoma and Ewing sarcoma is a good prognostic factor. Conversely, lymphopenia is associated with significantly decreased sarcoma survival. Clearly, the immune system can contribute towards better survival from sarcoma. This chapter will describe treatment and host factors that influence immune function and how effective local control and systemic interventions of sarcoma therapy can cause inflammation and/or immune suppression but are currently the standard of care...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28320420/malignant-gastrointestinal-neuroectodermal-tumor-a-case-report-and-review-of-the-literature
#11
REVIEW
Mohammed J Alyousef, Jumana A Alratroot, Tarek ElSharkawy, Mohamed A Shawarby, Mohammad A Al Hamad, Tarek M Hashem, Ahmed Alsayyah
BACKGROUND: Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare entity that was first described by Zambrano et al. in 2003 as "Clear cell sarcoma-like tumor of the gastrointestinal tract". It shares some of the histological features of clear cell sarcoma (CCS) but lacks the immunohistochemical reactivity for melanocytic markers. We report a case of GNET that was initially misdiagnosed as gastrointestinal stromal tumor (GIST). Recognizing this entity is important to avoid misdiagnosis...
March 20, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28300276/reassessing-the-clinical-spectrum-associated-with-hereditary-leiomyomatosis-and-renal-cell-carcinoma-syndrome-in-french-fh-mutation-carriers
#12
Marie Muller, Sophie Ferlicot, Marine Guillaud-Bataille, Gwénaël Le Teuff, Catherine Genestie, Sophie Deveaux, Abdelhamid Slama, Nicolas Poulalhon, Bernard Escudier, Laurence Albiges, Nadem Soufir, Marie-Françoise Avril, Betty Gardie, Carolina Saldana, Yves Allory, Anne-Paule Gimenez-Roqueplo, Brigitte Bressac-de Paillerets, Stéphane Richard, Patrick R Benusiglio
We addressed uncertainties regarding Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC) by exploring all French cases, representing the largest series to date. Fumarate Hydratase (FH) germline testing was performed with Sanger sequencing and qPCR/MLPA. Enzyme activity was measured when necessary. We carried out whenever possible a pathology review of RCC and S-(2-succino)-cysteine (2SC)/fumarate hydratase immunohistochemistry. We estimated survival using non-parametric Kaplan-Meier. There were 182 cases from 114 families...
March 16, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28273978/renal-clear-cell-sarcoma-anaplastic-variant-a-rare-entity
#13
Vaishali Atmaram Walke, Nitin Y Shende, D T Kumbhalkar
Clear Cell Sarcoma of Kidney (CCSK) is known for its morphologic diversity, aggressive behaviour, tendency to recur and metastasis to bone. Amongst the various morphologic subtypes, anaplastic CCSK is associated with worse prognosis. Here, we report a case of this rare variant of CCSK. A five-year-old boy presented with history of lump and pain in abdomen since one week. The Computed Tomography (CT) scan revealed a large mass occupying the middle and inferior pole of right kidney. The clinical impression was Wilms tumour...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28262274/sinonasal-adamantinoma-like-ewing-sarcoma-a-case-report
#14
Borislav A Alexiev, Yanki Tumer, Justin A Bishop
We describe the case of a sinonasal adamantinoma-like Ewing sarcoma in a 41-year-old male. Histologically, the tumor exhibited distinctive areas of nested growth pattern with prominent stromal fibrosis and metaplastic bone formation. The tumor cells were small and uniform with minimal amount of pale eosinophilic to clear cytoplasm and round or oval nuclei with finely dispersed chromatin and small nucleoli. Approximately 20% of the tumor parenchyma comprised of small clusters of basaloid cells within an osteofibrous background resembling adamantinoma...
December 2, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28258518/adrenocortical-carcinoma-arising-in-an-adrenal-rest-a-case-report-and-review-of-the-literature
#15
Kristine M Cornejo, Henrietta A Afari, Peter M Sadow
Carcinomas arising from embryonic adrenal rests are rare with only a handful of reported cases. We report a case of an adrenocortical carcinoma arising from an adrenal rest located between the bladder and prostate in a 51 year-old man. The patient presented following a year of rectal pain and constipation. Computed tomography (CT) scan revealed a 9 cm pelvic mass that appeared to arise from the soft tissue between the bladder and prostate, with displacement of the organs and narrowing of the rectal lumen, suspected to be a sarcoma...
March 3, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28256570/recurrent-bcor-internal-tandem-duplication-and-bcor-or-bcl6-expression-distinguish-primitive-myxoid-mesenchymal-tumor-of-infancy-from-congenital-infantile-fibrosarcoma
#16
Teresa Santiago, Michael R Clay, Sariah J Allen, Brent A Orr
Primitive myxoid mesenchymal tumor of infancy is a rare sarcoma that preferentially affects infants. It can be locally aggressive and rarely metastasizes, but the long-term outcome of children with this tumor is mostly unknown. Histologically, it is characterized by primitive cells with abundant myxoid stroma. Internal tandem duplication of B-cell CLL/lymphoma 6 (BCL6)-interacting co-repressor (BCOR) exon 15 has recently been described in clear cell sarcoma of kidney, central nervous system high-grade neuroepithelial tumor with BCOR alteration, and primitive myxoid mesenchymal tumor of infancy...
March 3, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28248728/clinicopathologic-features-of-the-non-cns-primary-ewing-sarcoma-family-of-tumors-in-the-head-and-neck-region
#17
Chang Gok Woo, Bora Lee, Joon Seon Song, Kyung-Ja Cho
Ewing sarcoma family of tumor (ESFT) is a group of malignant neoplasms that affect children and young adults. Primary ESFT does not commonly arise from the head and neck region. This study aimed to elucidate the clinicopathologic characteristics of ESFT of the head and neck region except for central nervous system primitive neuroectodermal tumors. Among the 207 cases of ESFT of the bone and soft tissue, diagnosed at Asan Medical Center during a 20-year period, 25 (12.1%) involved the head and neck region. Of those, 21 were available for histologic, immunohistochemical, and molecular studies...
February 28, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28247037/accuracy-of-pre-operative-hysteroscopic-guided-biopsy-for-predicting-final-pathology-in-uterine-malignancies
#18
Fabio Martinelli, Antonino Ditto, Giorgio Bogani, Mauro Signorelli, Valentina Chiappa, Domenica Lorusso, Edward Haeusler, Francesco Raspagliesi
PURPOSE: To evaluate concordance (C) between pre-operative hysteroscopic-directed sampling and final pathology in uterine cancers. METHODS: A retrospective cross-sectional evaluation of prospectively collected data of women who underwent hysterectomy for uterine malignancies and a previous hysteroscopic-guided biopsy was performed. Diagnostic concordance between pre-operative (hysteroscopic biopsy) and postoperative (uterine specimen) histology was evaluated. In endometrioid-endometrial cancers cases Kappa (k) statistics was applied to evaluate agreement for grading (G) between the preoperative and final pathology...
March 1, 2017: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/28195107/concurrent-occurrence-of-neuroblastoma-and-clear-cell-sarcoma-of-kidney-in-an-infant-is-it-a-random-event
#19
Ramesh B Deshpande
No abstract text is available yet for this article.
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28195106/dual-tumor-clear-cell-sarcoma-of-kidney-with-differentiating-neuroblastoma-a-rare-case-with-review-of-literature
#20
REVIEW
Preeti Rajeev Doshi, Manjiri N Karandikar, Ravi C Swami, Harvinder A Singh, Ravindra C Nimbargi
Occurrence of two distinct synchronous primary tumors is a rare event in children as well as in adults. Here, we report an extremely rare case of an infant found to have two synchronous Tumours namely clear cell sarcoma of kidney(CCSK) and differentiating neuroblastoma of preaortic region. To our knowledge, this may be the first case of synchronous CCSK and differentiating neuroblastoma being reported.
January 2017: Indian Journal of Pathology & Microbiology
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