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Clear cell sarcoma

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https://www.readbyqxmd.com/read/28086809/primary-mediastinal-clear-cell-sarcoma-a-case-report-and-review-of-the-literature
#1
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
#2
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...
December 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27969569/biology-and-treatment-of-renal-tumours-in-childhood
#3
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
#4
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...
November 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27869448/clear-cell-sarcoma-of-vulva-a-case-report
#5
Kristýna Němejcová, Pavel Dundr, Ivana Krajsová
We report the case of a 67-year-old female with clear cell sarcoma (CCS) of the vulva. Grossly, the tumor was a partly exophytical vulvar mass, measuring 20 x 15 cm. At the time of presentation, the patient showed metastases to the lung, inguinal and pelvic lymph nodes. Histologically, the tumor consisted of oval or spindle cells with only mild nuclear pleomorphism and rare mitoses (up to 2/10 HPF). The cytoplasm was pale eosinophilic or clear. The tumor cells were arranged in confluent sheets. There were large areas of necrosis and surface ulceration...
2016: Ceskoslovenská Patologie
https://www.readbyqxmd.com/read/27823979/high-frequency-of-clonal-ig-and-t-cell-receptor-gene-rearrangements-in-histiocytic-and-dendritic-cell-neoplasms
#6
Wenting Huang, Tian Qiu, Linshu Zeng, Bo Zheng, Jianming Ying, Xiaoli Feng
The 2008 World Health Organization (WHO) diagnostic criteria of histiocytic and dendritic cell neoplasms from hematopoietic and lymphoid tissues no longer required the absence of clonal B-cell/T-cell receptor gene rearrangements. It is true that the clonal B-cell/T-cell receptor gene rearrangements have been identified in rare cases of histiocytic and dendritic cell neoplasms, such as those with or following lymphoma/leukemia or in some sporadic histiocytic/dendritic cell sarcomas, but the clonal features of such group of tumor are still not clear...
November 3, 2016: Oncotarget
https://www.readbyqxmd.com/read/27817254/cytohistological-and-immunohistochemical-characteristics-of-spindle-shaped-mesenchymal-neoplasms-occurring-in-the-gastrointestinal-tract
#7
Antonio Ieni, Valeria Barresi, Luca Reggiani Bonetti, Giovanni Branca, Rosario Alberto Caruso, Giovanni Tuccari
The purpose of the present review is to analyze the cytohistological and immunohistochemical characteristics of spindle-shaped mesenchymal gastrointestinal neoplams (MGNs), a group of unusual neoplastic conditions with different biological behavior. These tumors exhibit clinical pictures strictly related to the site of origin and dimensions, even if they appear generally with an intramural localization. This latter point may suggest an useful application of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), mainly followed by the cell-block procedure (CBP) in the differential diagnostic approach...
March 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27816923/cryopreservation-of-ovarian-tissue-for-fertility-preservation-in-a-large-cohort-of-young-girls-focus-on-pubertal-development
#8
A K Jensen, C Rechnitzer, K T Macklon, M R S Ifversen, N Birkebæk, N Clausen, K Sørensen, J Fedder, E Ernst, C Yding Andersen
STUDY QUESTION: Is there an association between the need for medical puberty induction and the diagnosis or treatment received in girls who have undergone cryopreservation of ovarian tissue for fertility preservation? SUMMARY ANSWER: There was a clear association between the intensity of treatment received and requirement for medical puberty induction but no association with the diagnosis. WHAT IS KNOWN ALREADY: Although it cannot be predicted which girls will become infertile or develop premature ovarian insufficiency (POI) following intensive chemotherapy or irradiation, patients who are at high risk of POI should be offered ovarian tissue cryopreservation (OTC)...
January 2017: Human Reproduction
https://www.readbyqxmd.com/read/27799974/demsoplastic-small-round-cell-tumor-a-diagnostic-and-therapeutic-dilemma
#9
Moeinadin Safavi, Jahanbanoo Shahryari, Mohammad Mehdi Moeini Aghtaei, Hossein Nikpour
Desmoplastic small round cell tumor (DSCRT) is a rare variant of sarcoma with a highly aggressive behavior. It usually affects abdominal cavity and has a male predominance. Its correct diagnosis and treatment is sophisticated and requires an experienced multidisciplinary team. Hereby we present a 25 yr old man from Kerman Province in 2013 with abdominal mass and ascites who underwent sonography guided percutaneous needle biopsy which was misleading and inconclusive for diagnosis. Thus an open biopsy was fulfilled which revealed solid nests of small round cells with hyperchromatic nuclei and clear cytoplasm surrounded by a desmoplastic stroma suggestive for DSCRT...
2016: Iranian Journal of Pathology
https://www.readbyqxmd.com/read/27793634/-18-f-fdg-pet-ct-follow-up-of-follicular-dendritic-cell-sarcoma
#10
D Albano, G Bosio, F Bertagna
Follicular dendritic cell sarcoma (FDCS) is a rare lymphoid neoplasm which occurs mainly in lymph nodes, especially cervical and mediastinal; it can be difficult to diagnose and often is misdiagnosed. Its pathogenesis is still not clear, like its evolution. We report a case of 60-year-old woman with FDCS of left lateral cervical node followed for almost 10 years, after a left selective neck dissection, who underwent five (18)F-FDG PET/CT. This technique has proven to be an useful method for the management of this patient, mainly for the follow up, detection of relapse and therapy response evaluation...
October 25, 2016: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/27785659/implications-of-lymph-node-evaluation-in-the-management-of-resectable-soft-tissue-sarcoma
#11
Brett L Ecker, Madalyn G Peters, Matthew T McMillan, Andrew J Sinnamon, Paul J Zhang, Rachel R Kelz, Robert E Roses, Jeffrey A Drebin, Douglas L Fraker, Giorgos C Karakousis
BACKGROUND: The rate of lymph node (LN) metastasis is rare in soft tissue sarcoma, but there are histologic subtypes that metastasize via the lymphatics. The prognostic value of LN evaluation in these high-risk histologies is unknown. METHODS: Resected soft-tissue sarcoma patients with clear cell sarcoma, epithelioid sarcoma, rhabdomyosarcoma, or angiosarcoma (n = 2993) were identified in the National Cancer Data Base (2004-2013). Cox regression evaluated the association of omission of LN assessment (NX) and overall survival (OS)...
October 26, 2016: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/27760618/-primary-clear-cell-sarcoma-of-small-intestine-report-of-a-case
#12
D M Zhang, J G Wei, X F Yao
No abstract text is available yet for this article.
October 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/27748279/diagnostic-utility-of-wilms-tumour-1-protein-wt-1-immunostaining-in-paediatric-renal-tumours
#13
Surbhi Goyal, Kiran Mishra, Urvee Sarkar, Satendra Sharma, Anita Kumari
BACKGROUND & OBJECTIVES: Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms' tumour (commonest tumour) from non-Wilms' tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms' tumour-1 protein (WT1) in paediatric renal tumours. METHODS: A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study...
May 2016: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/27738815/-new-features-in-the-2014-who-classification-of-uterine-neoplasms
#14
S F Lax
The 2014 World Health Organization (WHO) classification of uterine tumors revealed simplification of the classification by fusion of several entities and the introduction of novel entities. Among the multitude of alterations, the following are named: a simplified classification for precursor lesions of endometrial carcinoma now distinguishes between hyperplasia without atypia and atypical hyperplasia, the latter also known as endometrioid intraepithelial neoplasia (EIN). For endometrial carcinoma a differentiation is made between type 1 (endometrioid carcinoma with variants and mucinous carcinoma) and type 2 (serous and clear cell carcinoma)...
November 2016: Der Pathologe
https://www.readbyqxmd.com/read/27719746/brain-metastasis-from-gastrointestinal-clear-cell-sarcoma
#15
Sahejmeet S Guraya, Richard A Prayson
Clear cell sarcoma (CCS) of the gastrointestinal tract presents a diagnostic challenge to the pathologist due to its morphological and immunohistochemical similarity to melanoma. It usually metastasizes to regional lymph nodes, liver, and lungs. Herein, we report the first known metastasis of a gastrointestinal CCS to the central nervous system. Cytogenetic testing showed the t(12,22) translocation corresponding to the presence of the EWS/ATF1 hybrid consistent with CCS. The literature that compares melanoma to CCS is reviewed in the context of this rare presentation to differentiate between the two diseases...
January 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/27716285/safety-pharmacokinetics-and-antitumor-properties-of-anlotinib-an-oral-multi-target-tyrosine-kinase-inhibitor-in-patients-with-advanced-refractory-solid-tumors
#16
Yongkun Sun, Wei Niu, Feng Du, Chunxia Du, Shuting Li, Jinwan Wang, Li Li, Fengqing Wang, Yu Hao, Chuan Li, Yihebali Chi
BACKGROUND: Anlotinib is a novel multi-target tyrosine kinase inhibitor that is designed to primarily inhibit VEGFR2/3, FGFR1-4, PDGFR α/β, c-Kit, and Ret. We aimed to evaluate the safety, pharmacokinetics, and antitumor activity of anlotinib in patients with advanced refractory solid tumors. METHODS: Anlotinib (5-16 mg) was orally administered in patients with solid tumor once a day on two schedules: (1) four consecutive weeks (4/0) or (2) 2-week on/1-week off (2/1)...
October 4, 2016: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/27710896/mirna-133b-and-mirna-135a-induce-apoptosis-via-the-jak2-stat3-signaling-pathway-in-human-renal-carcinoma-cells
#17
Wenbin Zhou, Xingjie Bi, Guojun Gao, Lijiang Sun
Aberrantly expressed microRNAs (miRNAs) are involved in many diseases including cancer. In clear cell renal cell carcinoma (ccRCCs) expression of miR-133b and miR-135a is reduced compared to control tissue and other sarcomas but the functional effects of this downregulation are not fully understood. This study aimed at evaluating the miR-133b and miR-135a expression profiles in different RCC subtypes and the functional role of these miRNAs. Viability and apoptosis were examined in three different ccRCC cell lines (786-O, A498 and SN12-PM6) after over-expression of these miRNAs...
December 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27661665/pancreatic-resection-for-renal-cell-carcinoma-metastasis-an-exceptionally-rare-coexistence
#18
Stergios Boussios, Ioannis Zerdes, Ourania Batsi, Vasilios P Papakostas, Esmeralda Seraj, George Pentheroudakis, George K Glantzounis
INTRODUCTION: Pancreatic metastases are uncommon and only found in a minority of patients with widespread metastatic disease at autopsy. The most common primary cancer site resulting in pancreatic metastases is the kidney, followed by colorectal cancer, melanoma, breast cancer, lung carcinoma and sarcoma. PRESENTATION OF CASE: Herein, we report a 63-year-old male patient who presented -3.5 years after radical nephrectomy performed for renal cell carcinoma (RCC)-with a well-defined lobular, round mass at the body of the pancreas demonstrated by abdominal Magnetic Resonance Imaging (MRI)...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27591237/evaluation-of-minimal-disseminated-disease-in-cryopreserved-ovarian-tissue-from-bone-and-soft-tissue-sarcoma-patients
#19
M M Dolmans, Y Iwahara, J Donnez, M Soares, J L Vaerman, C A Amorim, H Poirel
STUDY QUESTION: What is the risk of finding malignant cells in cryopreserved ovarian tissue from sarcoma patients? SUMMARY ANSWER: Minimal disseminated disease (MDD) was not detected in frozen-thawed ovarian tissue from 26 patients by any of the sensitive methods applied. WHAT IS KNOWN ALREADY: In case of leukemia, the risk of malignant cell transmission through the graft is well known and widely documented. However, for bone cancer, like Ewing sarcoma or osteosarcoma, only a small number of case reports, have been published...
October 2016: Human Reproduction
https://www.readbyqxmd.com/read/27588498/acquisition-of-an-oncogenic-fusion-protein-serves-as-an-initial-driving-mutation-by-inducing-aneuploidy-and-overriding-proliferative-defects
#20
Jacob M Loupe, Patrick J Miller, Benjamin P Bonner, Elaine C Maggi, Jyothi Vijayaraghavan, Jovanny Zabaleta, Christopher M Taylor, Fern Tsien, Judy S Crabtree, Andrew D Hollenbach
While many solid tumors are defined by the presence of a particular oncogene, the role that this oncogene plays in driving transformation through the acquisition of aneuploidy and overcoming growth arrest are often not known. Further, although aneuploidy is present in many solid tumors, it is not clear whether it is the cause or effect of malignant transformation. The childhood sarcoma, Alveolar Rhabdomyosarcoma (ARMS), is primarily defined by the t(2;13)(q35;q14) translocation, creating the PAX3-FOXO1 fusion protein...
September 27, 2016: Oncotarget
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