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Clear cell sarcoma

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https://www.readbyqxmd.com/read/28344878/targeting-cytokine-signaling-checkpoint-cis-activates-nk-cells-to-protect-from-tumor-initiation-and-metastasis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28195103/malignant-melanocytic-neoplasm-of-pancreas-with-liver-metastasis-is-it-malignant-melanoma-or-clear-cell-sarcoma
#13
Thomas Alex Kodiatte, Sam Varghese George, Raju Titus Chacko, Banumathi Ramakrishna
Malignant melanocytic neoplasm, usually seen in soft tissues, is rare in a visceral location and presents as a diagnostic dilemma. We present a case of pancreatic malignant melanocytic neoplasm with liver metastasis. A 58-year-old man presented with left upper abdominal swelling and loss of appetite. Imaging revealed a large mass arising from the pancreatic tail, and this was diagnosed as malignant neoplasm with melanocytic differentiation on biopsy with the possible differentials of malignant melanoma, clear cell sarcoma (CCS), and perivascular epithelioid cell neoplasm...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28179300/the-antiproliferative-and-colony-suppressive-activities-of-stat3-inhibitors-in-human-cancer-cells-is-compromised-under-hypoxic-conditions
#14
Jilai Tian, Hui Xiao, Ruohan Wu, Yang Cao, Chenglong Li, Ronald Xu, Christopher R Pierson, Jonathan L Finlay, Fang Yang, Ning Gu, Jiayuh Lin
Constitutive activation of signal transducer and activator of transcription 3 (STAT3) has been indicated as a novel cancer drug target, since it plays an important role in diverse oncogenic processes including survival, cell proliferation and migration. Emerging STAT3 inhibitors have demonstrated efficacy in cancer cells and animal tumor models. It is well known that most solid tumors are characterized by hypoxia, but it is not clear if hypoxic conditions affect activity of STAT3 inhibitors. To examine this, two STAT3 inhibitors were tested to investigate their inhibitory efficacy in cancer cells grown under hypoxic conditions compared with those without hypoxia...
2017: Anticancer Research
https://www.readbyqxmd.com/read/28141799/the-spectrum-of-ewsr1-rearranged-neoplasms-at-a-tertiary-sarcoma-centre-assessing-772-tumour-specimens-and-the-value-of-current-ancillary-molecular-diagnostic-modalities
#15
Jonathan Noujaim, Robin L Jones, John Swansbury, David Gonzalez, Charlotte Benson, Ian Judson, Cyril Fisher, Khin Thway
BACKGROUND: EWSR1 rearrangements were first identified in Ewing sarcoma, but the spectrum of EWSR1-rearranged neoplasms now includes many soft tissue tumour subtypes including desmoplastic small round cell tumour (DSRCT), myxoid liposarcoma (MLPS), extraskeletal myxoid chondrosarcoma (EMC), angiomatoid fibrous histiocytoma (AFH), clear cell sarcoma (CCS) and myoepithelial neoplasms. We analysed the spectrum of EWSR1-rearranged soft tissue neoplasms at our tertiary sarcoma centre, by assessing ancillary molecular diagnostic modalities identifying EWSR1-rearranged tumours and reviewing the results in light of our current knowledge of these and other Ewing sarcoma-like neoplasms...
February 28, 2017: British Journal of Cancer
https://www.readbyqxmd.com/read/28108880/unusual-signal-patterns-of-break-apart-fish-probes-used-in-the-diagnosis-of-soft-tissue-sarcomas
#16
Gergő Papp, Dóra Mihály, Zoltán Sápi
Break-apart FISH probes are the most popular and reliable type of FISH probes used to confirm certain pathological diagnoses. The interpretation is usually easy, however, in some instances it is not so unequivocal. Our aim was to reveal and elucidate the problems occurring in the process of evaluation of the break-apart probe results. Altogether 301 soft tissue sarcomas with confirmed molecular tests using break-apart probes were assessed to reveal the frequency and type of unusual signal pattern. Among 89 synovial sarcoma (SS18) 11%, 12 alveolar rhabdomyosarcoma (FOXO1) 50%, 53 myxoid liposarcoma (DDIT3) 7...
January 20, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28086809/primary-mediastinal-clear-cell-sarcoma-a-case-report-and-review-of-the-literature
#17
REVIEW
Long Jin, Yuxia Sui, Haili Zhu, Zhizhong Chen, Shuguang Liu
BACKGROUND: Clear cell sarcoma (CCS) is a rare malignant soft-tissue neoplasm that displays melanocytic markers and exhibits striking histopathological features. The tumour has a predilection for the lower extremities and rarely presents in the mediastinum. CASE PRESENTATION: We present a case of primary mediastinal CCS in a 57-year-old man. Computer tomography (CT) revealed a 12 × 12 × 7.5 cm mass in the anterior mediastinum. Microscopically, the tumour mainly consisted of epithelioid cells with oval vesicular nuclei and eosinophilic cytoplasm...
January 13, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28009602/ewsr1-fusions-with-creb-family-transcription-factors-define-a-novel-myxoid-mesenchymal-tumor-with-predilection-for-intracranial-location
#18
Yu-Chien Kao, Yun-Shao Sung, Lei Zhang, Chun-Liang Chen, Sumathi Vaiyapuri, Marc K Rosenblum, Cristina R Antonescu
Recurrent gene fusions involving EWSR1 with members of the cAMP response element binding protein (CREB) family (ATF1 and CREB1) have been reported in a diverse group of tumors including angiomatoid fibrous histiocytoma (AFH), soft tissue and gastrointestinal clear cell sarcoma, primary pulmonary myxoid sarcoma, and hyalinizing clear cell carcinoma of salivary gland. We have recently encountered a group of 5 myxoid mesenchymal tumors positive for EWSR1 fusions with one of the CREB family member (ATF1, CREB1, and CREM), with histologic features distinct from any of the previously described pathologic entities...
April 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27969569/biology-and-treatment-of-renal-tumours-in-childhood
#19
REVIEW
Jesper Brok, Taryn D Treger, Saskia L Gooskens, Marry M van den Heuvel-Eibrink, Kathy Pritchard-Jones
In Europe, almost 1000 children are diagnosed with a malignant renal tumour each year. The vast majority of cases are nephroblastoma, also known as Wilms' tumour (WT). Most children are treated according to Société Internationale d'Oncologie Pédiatrique Renal Tumour Study Group (SIOP-RTSG) protocols with pre-operative chemotherapy, surgery, and post-operative treatment dependent on stage and histology. Overall survival approaches 90%, but a subgroup of WT, with high-risk histology and/or relapsed disease, still have a much poorer prognosis...
October 28, 2016: European Journal of Cancer
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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