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Clear cell sarcoma

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https://www.readbyqxmd.com/read/29401145/clear-cell-sarcoma-of-the-tongue-on-mri-and-pet-ct
#1
Mickael Tordjman, Matthieu Dubois, Maxime de Malherbe, Yves Denoux, Quentin Gillebert
F-FDG PET/CT and MRI were performed in a 44-year-old woman to characterize a mass of the anterior tongue. MR images showed a voluminous mass, well circumscribed and enhanced heterogeneously after gadolinium chelates injection. There was an intense uptake on PET/CT. Pathological examination and molecular analysis revealed the diagnosis of clear cell sarcoma of the tongue. We present a case of clear cell sarcoma of the tongue, which includes imaging features. It is an extremely rare tumor, with only 3 cases previously reported in the literature...
February 3, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29398970/alveolar-soft-part-sarcoma-presenting-as-hypervascular-adrenal-metastasis
#2
Manjunath Goroshi, Anurag R Lila, Tushar Bandgar, Nalini S Shah
Hypervascular adrenal masses include pheochromocytoma, metastases caused by clear renal cell carcinoma/hepatocellular carcinoma. Alveolar soft part sarcoma (ASPS) causing hypervascular metastases is not described in the literature. Here, we describe the first case of ASPS presenting as hypervascular metastasis. Our case was a 23-year-old male incidentally detected right adrenal mass during the evaluation of pain in the abdomen. On computed tomography (CT), adrenal mass showed bright enhancement in early arterial phase (unenhanced Hounsfield unit [HU]-45...
January 2018: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29397940/kshv-virf4-enhances-bcl6-transcription-via-downregulation-of-irf4-expression
#3
Hye Ryun Yu, Yeong Jun Kim, Hye-Ra Lee
Primary effusion lymphoma (PEL), strongly linked with latent infection of Kaposi's sarcoma-associated herpesvirus (KSHV), constitutively expresses cellular interferon regulatory factor 4 (IRF4) while suppressing the expression of B cell lymphoma 6 (BCL6). Recently, it was shown that IRF4, a key transcriptional repressor of BCL6, might be a pivotal regulator of KSHV for balancing between latency and its reactivation in PEL cells. However, the action of the BCL6-IRF4 transcription factor axis during KSHV's life cycle is not clear...
February 1, 2018: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/29397265/the-evaluation-of-circulating-endothelial-progenitor-cells-and-related-angiogenic-markers-as-prognostic-factors-in-soft-tissue-tumors
#4
Ioannis Karampinis, Elena Joas, Anna Dreyer, Ulrich Ronellenfitsch, Jens Jakob, Peter Hohenberger, Kai Nowak
INTRODUCTION: Neovascularisation is a critical step in the progression of malignant tumors. Circulating endothelial progenitor cells (cEPC) have been proposed as surrogate markers of vasculogenesis in malignancies. In this project, we studied the impact of tumor-specific therapy on cEPC and associated angiogenic factors in patients with soft tissue tumors. MATERIALS AND METHODS: Fifty-three patients with soft tissue tumors (25 soft tissue sarcomas, 19 GIST, 9 desmoids) and 15 healthy controls were included...
January 31, 2018: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29391414/design-and-synthesis-of-coumarin-based-organoselenium-as-a-new-hit-for-myeloprotection-and-synergistic-therapeutic-efficacy-in-adjuvant-therapy
#5
Arup Ranjan Patra, Somnath Singha Roy, Abhishek Basu, Avishek Bhuniya, Arin Bhattacharjee, Subhadip Hajra, Ugir Hossain Sk, Rathindranath Baral, Sudin Bhattacharya
A newly designed organoselenium compound, methyl substituted umbelliferone selenocyanate (MUS), was synthesized as a primary hit against the myelotoxic activity of carboplatin. MUS was administered at 6 mg/kg b.wt, p.o. in concomitant and pretreatment schedules with carboplatin (12 mg/kg b.wt, i.p. for 10 days) in female Swiss albino mouse. MUS treatment reduced (P < 0.001) the percentage of chromosomal aberrations, micronuclei formation, DNA damage and apoptosis in murine bone marrow cells and also enhanced (P < 0...
February 1, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29390282/giant-solitary-fibrous-tumor-of-the-pleura-with-high-grade-sarcomatous-overgrowth-accompanied-by-lipid-rich-rhabdomyosarcomatous-and-pleomorphic-components-a-case-report
#6
Takeo Nakaya, Hisashi Oshiro, Ayako Takigami, Yoshihiko Kanai, Kenji Tetsuka, Koichi Hagiwara, Hirofumi Fujii, Shunsuke Endo, Akira Tanaka
RATIONALE: Solitary fibrous tumors are mesenchymal tumors presenting as fibroblastic neoplasms with prominent branching vascular patterns, which are often generated from the pleura. Most solitary fibrous tumors are benign; however, some can turn malignant. High-grade sarcomas from solitary fibrous tumors include multidirectional histopathological components. PATIENT CONCERNS: We describe our experience of a giant high-grade sarcoma with mixed components generated from a solitary fibrous tumor of the pleura in a 67-year-old female patient presenting with cough and left-sided chest pain...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29361725/chondrosarcoma-a-rare-misfortune-in-aging-human-cartilage-the-role-of-stem-and-progenitor-cells-in-proliferation-malignant-degeneration-and-therapeutic-resistance
#7
REVIEW
Karen A Boehme, Sabine B Schleicher, Frank Traub, Bernd Rolauffs
Unlike other malignant bone tumors including osteosarcomas and Ewing sarcomas with a peak incidence in adolescents and young adults, conventional and dedifferentiated chondrosarcomas mainly affect people in the 4th to 7th decade of life. To date, the cell type of chondrosarcoma origin is not clearly defined. However, it seems that mesenchymal stem and progenitor cells (MSPC) in the bone marrow facing a pro-proliferative as well as predominantly chondrogenic differentiation milieu, as is implicated in early stage osteoarthritis (OA) at that age, are the source of chondrosarcoma genesis...
January 21, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29333010/sinusoidal-obstruction-syndrome-during-treatment-for-wilms-tumor-a-life-threatening-complication
#8
Sidharth Totadri, Amita Trehan, Deepak Bansal, Richa Jain
Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29323103/oridonin-exerts-anticancer-effect-on-osteosarcoma-by-activating-ppar-%C3%AE-and-inhibiting-nrf2-pathway
#9
Ying Lu, Yang Sun, Jianwei Zhu, Lisha Yu, Xiubo Jiang, Jie Zhang, Xiaochen Dong, Bo Ma, Qi Zhang
Osteosarcoma is the most common high-grade human primary malignant bone sarcoma with lower survival in the past decades. Oridonin, a bioactive diterpenoid isolated from Rabdosia rubescens, has been proved to possess potent anti-cancer effects. However, its potential mechanism still remains not fully clear nowadays. In this study, we investigated the anticancer effect of oridonin on human osteosarcoma and illuminated the underlying mechanisms. In vitro, oridonin inhibited the cell viability of various osteosarcoma cells...
January 11, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29302306/alveolar-soft-part-sarcoma-of-flexure-tendon
#10
Chatchai Pruksapong, Kantang Satayasoontorn
Alveolar soft part sarcoma (ASPS) is a very rare sarcoma, report <1% of all soft tissue tumor. Majority of cases were young adult and tumor occurred in lower extremities and trunk. Here, we present a case of 53-year-old Thai female with rapidly glowing mass over her right forearm. The magnetic resonance imaging scan demonstrated a hypervascular mass with multiple feeding vessels located in flexure tendon of right forearm. Ultrasound-guided biopsy revealed malignant poorly differentiated epithelioid neoplasm with clear cell feature and focal necrosis...
December 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/29285586/intracranial-ewing-sarcoma-four-pediatric-examples
#11
Michael J Yang, Ros Whelan, Jennifer Madden, Jean M Mulcahy Levy, B K Kleinschmidt-DeMasters, Todd C Hankinson, Nicholas K Foreman, Michael H Handler
BACKGROUND: Ewing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis...
December 28, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29243812/dysregulated-mitogen-activated-protein-kinase-signalling-as-an-oncogenic-basis-for-clear-cell-sarcoma-of-the-kidney
#12
Colin Kenny, Naomi McDonagh, Antonio Lazaro, Elaine O'Meara, Rut Klinger, Darran O'Connor, Fiona Roche, Karsten Hokamp, Maureen J O'Sullivan
The oncogenic mechanisms and tumour biology underpinning Clear Cell Sarcoma of Kidney (CCSK), the second commonest paediatric renal malignancy, are poorly understood and currently therapy depends heavily on doxorubicin with cardiotoxic side-effects. Previously, we characterised the balanced t(10;17)(q22;p13) chromosomal translocation, identified at that time as the only recurrent genetic aberration in CCSK. This translocation results in an in-frame fusion of the genes YWHAE (encoding 14-3-3ε)and NUTM2, with a somatic incidence of 12%...
December 15, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/29241974/endometriosis-associated-ovarian-neoplasia
#13
REVIEW
Xavier Matias-Guiu, Colin J R Stewart
This article reviews the most relevant pathological and molecular features of ovarian tumours that are associated with endometriosis. Endometriosis is a common condition, affecting 5-15% of all women, and it has been estimated that 0.5-1% of cases are complicated by neoplasia. The most common malignant tumours in this setting are endometrioid adenocarcinoma and clear cell adenocarcinoma, each accounting for approximately 10% of ovarian carcinomas in Western countries. A minority of cases are associated with Lynch syndrome...
December 11, 2017: Pathology
https://www.readbyqxmd.com/read/29240581/cutaneous-melanocytoma-with-crtc1-trim11-fusion-report-of-5-cases-resembling-clear-cell-sarcoma
#14
Lucie Cellier, Emilie Perron, Daniel Pissaloux, Marie Karanian, Veronique Haddad, Laurent Alberti, Arnaud de la Fouchardière
We report 5 cases of primary intradermal nodular unpigmented tumors with a melanocytic immunophenotype associated with a novel CRTC1-TRIM11 fusion. Clinically, the cutaneous nodules were slowly growing in 3 women and 2 men (25 to 82 y old, median, 28 y) with no specific topography. Lesion size ranged from 4 to 12 mm (median, 5 mm). The tumors were strictly located in the dermis with a nodular pattern. The cells were arranged in confluent nests and fascicules. Central fibronecrotic areas were present in 2 cases...
December 7, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29226988/cns-high-grade-neuroepithelial-tumor-with-bcor-internal-tandem-duplication-a-comparison-with-its-counterparts-in-the-kidney-and-soft-tissue
#15
Yuka Yoshida, Sumihito Nobusawa, Satoshi Nakata, Mitsutoshi Nakada, Yoshiki Arakawa, Yohei Mineharu, Yasuo Sugita, Takako Yoshioka, Asuka Araki, Yuichiro Sato, Hideo Takeshima, Masahiko Okada, Akira Nishi, Tatsuya Yamazaki, Kenichi Kohashi, Yoshinao Oda, Junko Hirato, Hideaki Yokoo
Central nervous system high-grade neuroepithelial tumors with BCOR alteration (CNS HGNET-BCOR) are a recently reported rare entity, identified as a small fraction of tumors previously institutionally diagnosed as so-called CNS primitive neuroectodermal tumors. Their genetic characteristic is a somatic internal tandem duplication in the 3' end of BCOR (BCOR ITD), which has also been found in clear cell sarcomas of the kidney (CCSK) and soft tissue undifferentiated round cell sarcomas/primitive myxoid mesenchymal tumors of infancy (URCS/PMMTI), and these BCOR ITD-positive tumors have been reported to share similar pathological features...
December 11, 2017: Brain Pathology
https://www.readbyqxmd.com/read/29208319/defining-the-incidence-and-clinical-significance-of-lymph-node-metastasis-in-soft-tissue-sarcoma
#16
Emily Z Keung, Yi-Ju Chiang, Rachel K Voss, Janice N Cormier, Keila E Torres, Kelly K Hunt, Barry W Feig, Christina L Roland
INTRODUCTION: The incidence and clinical significance of lymph node metastasis (LNM, N1) in soft tissue sarcoma (STS) is unclear. Recent studies have focused on extremity/trunk STS (ETSTS). We sought to define the subgroup of patients with LNM at sarcoma diagnosis across all disease sites and histologies. METHODS: We identified and categorized 89,870 STS patients from the National Cancer Data Base (1998-2012) by nodal stage. Pathologically confirmed LNM (pN1) were identified in 1404 patients; 1750 had clinically suspicious but not pathologically confirmed LNM (cN1)...
November 27, 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29204000/malignant-gastrointestinal-neuroectodermal-tumor-a-unique-rare-neoplasm
#17
Surbhi Kansal, Seema Rao
Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignant tumor. It is also referred to as clear cell sarcoma-like gastrointestinal tumor (CCSLGT). It is an aggressive tumor with a high rate of local recurrence, metastases, and early death from disease. Its pathogenesis is not known. It shows evidence of neural differentiation and lacks immunohistochemical and ultrastructural evidence of melanocytic differentiation. It needs to be distinguished from various mimickers owing to its aggressive course...
December 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29200690/unusual-presentation-of-metanephric-stromal-tumor-in-a-neonate
#18
Prashant Sadashiv Patil, Abhaya Gupta, Rahul Gupta, Paras L Kothari, Geeta Kekre, Kedar Mudkhedkar, Vishesh Dikshit
Metanephric stromal tumor (MST) of kidney is an under-reported benign stromal specific renal neoplasm with good prognosis. This tumor is to be differentiated from congenital mesoblastic nephroma and clear-cell sarcoma of the kidney. In this case report, we describe the imaging, gross, and microscopic features of MST with ischemic left kidney in a 1-month-old child and discuss treatment with relevant literature.
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29200103/bcor-internal-tandem-duplication-in-high-grade-uterine-sarcomas
#19
Adrián Mariño-Enriquez, Alexandra Lauria, Joanna Przybyl, Tony L Ng, Magdalena Kowalewska, Maria Debiec-Rychter, Raji Ganesan, Vaiyapuri Sumathi, Suzanne George, W Glenn McCluggage, Marisa R Nucci, Cheng-Han Lee, Jonathan A Fletcher
Endometrial stromal sarcomas (ESSs) are mesenchymal uterine tumors characterized by recurrent genetic events, most commonly chromosomal rearrangements, that create oncogenic gene fusions. High-grade endometrial stromal sarcomas (HG-ESSs), as defined in the 2014 World Health Organization Classification, typically contain oncogenic YWHAE-NUTM2 fusions; however, although not well characterized, there are tumors morphologically overlapping with HG-ESS that do not contain the YWHAE-NUTM2 fusions. These fusions are also found in certain pediatric primitive sarcomas, including clear cell sarcoma of the kidney and soft tissue undifferentiated round cell sarcoma of infancy...
December 1, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29197033/establishment-and-proteomic-characterization-of-patient-derived-clear-cell-sarcoma-xenografts-and-cell-lines
#20
Marimu Sakumoto, Rieko Oyama, Mami Takahashi, Yoko Takai, Fusako Kito, Kumiko Shiozawa, Zhiwei Qiao, Makoto Endo, Akihiko Yoshida, Akira Kawai, Tadashi Kondo
Clear cell sarcoma (CCS) is an aggressive mesenchymal malignancy characterized by the unique chimeric EWS-ATF1 fusion gene. Patient-derived cancer models are essential tools for the understanding of tumorigenesis and the development of anti-cancer drugs; however, only a limited number of CCS cell lines exist. The objective of this study was to establish patient-derived CCS models. We established patient-derived CCS models from a 43-yr-old female patient. We prepared the patient-derived xenografts (PDXs) from tumor tissues obtained through biopsy or surgery and isolated stable cell lines from PDXs and the original tumor tissue...
December 1, 2017: In Vitro Cellular & Developmental Biology. Animal
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