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Epithelioid sarcoma

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https://www.readbyqxmd.com/read/27926791/high-prevalence-of-mitf-staining-in-undifferentiated-pleomorphic-sarcoma-caution-in-use-of-melanocytic-markers-in-sarcoma
#1
Bonnie Choy, Elizabeth Hyjek, Anthony G Montag, Peter Pytel, Rex Haydon, Hue H Luu, Chao Jie Zhen, Bradley C Long, Sabah Kadri, Jeremy P Segal, Larissa V Furtado, Nicole A Cipriani
AIMS: Diagnosis of undifferentiated pleomorphic sarcoma (UPS) may be challenging, as other lesions with undifferentiated spindle cell morphology must be excluded, including melanoma. Microphthalmia-associated transcription factor (MiTF) stains nevi and epithelioid melanomas, as well as some mesenchymal neoplasms. We evaluated the prevalence of MiTF and melanocytic markers in UPS and a subset of atypical fibroxanthoma (AFX). METHODS AND RESULTS: MiTF, SOX10, Melan-A, HMB45, and S100 immunostains were performed on resection specimens from 19 UPS and 5 AFX...
December 7, 2016: Histopathology
https://www.readbyqxmd.com/read/27922155/metastatic-epithelioid-sarcoma-revealed-with-18-f-fdg%C3%A2-pet-ct-imaging
#2
Burcu Dirlik Serim, Betul Vatankulu, Metin Halaç, Suleyman Dikici, Fatih Kantarcı, Kerim Sönmezoğlu
No abstract text is available yet for this article.
December 6, 2016: Nuklearmedizin. Nuclear Medicine
https://www.readbyqxmd.com/read/27895527/phase-ii-trial-of-angiotensin-1-7-for-the-treatment-of-patients-with-metastatic-sarcoma
#3
Paul D Savage, James Lovato, K Bridget Brosnihan, Antonius A Miller, W Jeffrey Petty
Background. Angiotensin-(1-7) [Ang-(1-7)] is an endogenous antiangiogenic hormone with anticancer activity. In a phase I study of Ang-(1-7), two of three patients with metastatic sarcoma experienced disease stabilization. This phase II study examined clinical and biomarker outcomes for patients with metastatic sarcoma. Methods. Ang-(1-7) was administered by subcutaneous injection at a dose of 20 mg daily. If excessive toxicities occurred in the first cohort, a dose deescalation cohort was allowed. Blood samples were obtained to measure changes in biomarkers...
2016: Sarcoma
https://www.readbyqxmd.com/read/27874193/alk-oncoproteins-in-atypical-inflammatory-myofibroblastic-tumours-novel-rrbp1-alk-fusions-in-epithelioid-inflammatory-myofibroblastic-sarcoma
#4
Jen-Chieh Lee, Chien-Feng Li, Hsuan-Ying Huang, Mei-Jun Zhu, Adrián Mariño-Enríquez, Chung-Ta Lee, Wen-Bin Ou, Jason L Hornick, Jonathan A Fletcher
ALK oncogenic activation mechanisms were characterized in four conventional spindle-cell inflammatory myofibroblastic tumours (IMT) and five atypical IMT, each of which had ALK genomic perturbations. Constitutively activated ALK oncoproteins were purified by ALK immunoprecipitation and electrophoresis, and were characterized by mass spectrometry. The four conventional IMT had TPM3/4-ALK fusions (two cases) or DCTN1-ALK fusions (two cases), whereas two atypical spindle-cell IMT had TFG-ALK and TPM3-ALK fusion in one case each, and three epithelioid inflammatory myofibroblastic sarcomas had RANBP2-ALK fusions in two cases, and a novel RRBP1-ALK fusion in one case...
November 22, 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27818284/cardiac-angiosarcoma-histopathologic-immunohistochemical-and-cytogenetic-analysis-of-10-cases
#5
Charles Leduc, Sarah M Jenkins, William R Sukov, Jeannette G Rustin, Joseph J Maleszewski
Angiosarcoma (AS) is the most common cardiac sarcoma with differentiation, and is poorly characterized from a molecular genetic standpoint. Prognosis remains poor, owing to several factors including aggressive tumor biology, poor response to adjuvant therapy, and lack of targeted therapy. The clinical, pathologic and molecular cytogenetic features were studied in ten cardiac AS surgically resected at Mayo Clinic (1994-2015) using a whole genome single nucleotide polymorphism based platform (OncoScan). Mean patient age was 47...
November 3, 2016: Human Pathology
https://www.readbyqxmd.com/read/27800093/proximal-type-epithelioid-sarcoma-a-new-case-report-and-literature-review
#6
Abdelmoughit Echchaoui, Yassine Sadrati, Youssef Elbir, Abderrahim Elktaibi, Malika Benyachou, Samir El Mazouz, Nour-Eddine Gharib, Abdellah Abbassi
Proximal-type epithelioid sarcoma is a rare soft tissue neoplasm which arises from the more proximal part of body and occurs more often in young people; the definite diagnosis depends mainly on the pathological examination; early detection and complete excision remain the foundation of treatment. Due to its aggressive behavior, high capacity of recurrence and the great ability to metastasize, a careful clinical long-term monitoring is required. We report a new case of a 20 years old girl, presented with proximal-type epithelioid sarcoma in her right scapular region, confirmed by pathological examination and removed surgically without recurrence or metastasis at eighteen months of follow-up...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27785659/implications-of-lymph-node-evaluation-in-the-management-of-resectable-soft-tissue-sarcoma
#7
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/27776545/epithelioid-sarcoma-with-multiple-lesions-on-the-left-arm-a-case-report
#8
Rie Nishibaba, Yuko Higashi, Yuko Goto, Masanori Hisaoka, Takuro Kanekura
BACKGROUND: Epithelioid sarcoma is a rare, high-grade malignant tumor of the soft tissue. The incidence of local recurrence, regional lymph node involvement, and distant metastases is high. Epithelioid sarcoma is most often seen in adolescents and young adults. In the early stage before the development of full clinical features, epithelioid sarcoma is often misdiagnosed as a benign disease such as granuloma. CASE PRESENTATION: We report a case of a 74-year-old Japanese woman whose epithelioid sarcoma was initially misdiagnosed as fungal infection...
October 24, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27770126/gastrointestinal-bleeding-caused-by-epitheloid-sarcoma-a-case-report
#9
F Y Lee, L Limi, T Gee
Epithelioid sarcoma (ES) of the small bowel is a rare gastrointestinal tumour. We report a case of gastrointestinal bleeding secondary to small bowel ES in a 55-year-old gentleman. After gastroscopy and colonoscopy failed to identify the source of bleeding, we proceeded with computed tomography angiogram of the mesentery, which revealed intraluminal blood clot in the distal jejunum with features of obstruction. This is a rare cause of obscure gastrointestinal bleeding and emphasises the need for additional evaluation in the presence of negative endoscopic findings...
August 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/27769872/primary-epithelioid-sarcoma-of-the-kidney-and-adrenal-gland-report-of-2-cases-with-immunohistochemical-and-molecular-cytogenetic-studies
#10
Mir B Alikhan, Garrison Pease, William Watkin, Raymon Grogan, Thomas Krausz, Tatjana Antic
Epithelioid sarcoma (ES) is a malignant mesenchymal neoplasm with some morphologic or immunophenotypic evidence of epithelial differentiation. The "classical" subtype occurs in younger patients, often in distal extremities as compared to the "proximal" type. Tumors of the proximal type primarily arising in solid organs are rare with only few case reports in the literature. We report two cases of primary ES in the kidney of a 27-year-old female and the adrenal gland of a 73-year-old male. Clinical exam and imaging, including computer tomography and positron emission tomography did not reveal tumor elsewhere in both cases...
October 18, 2016: Human Pathology
https://www.readbyqxmd.com/read/27716413/pulmonary-tumor-diagnosed-as-an-undifferentiated-sarcoma-with-epithelioid-features-a-case-report
#11
Mohamed Réda El Ochi, Mohammed Massine El Hammoumi, Abdelhamid Biyi, Mohamed Allaoui, El Hassane Kabiri, Abderrahman Albouzidi, Mohamed Oukabli
BACKGROUND: Pulmonary sarcomas are uncommon accounting for 0.5 % of all primary lung cancers. Undifferentiated sarcomas account for up to 20 % of soft tissue sarcomas. A lung tumor revealed to be an undifferentiated sarcoma with epithelioid features has never been reported in the literature. CASE PRESENTATION: A 61-year-old white Moroccan man presented with 2 months' history of hemoptysis and dyspnea. Chest computed tomography showed a cystic mass involving the lower field of his right lung evoking first a hydatid cyst...
October 3, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27696380/phase-2-study-of-dasatinib-in-patients-with-alveolar-soft-part-sarcoma-chondrosarcoma-chordoma-epithelioid-sarcoma-or-solitary-fibrous-tumor
#12
Scott M Schuetze, Vanessa Bolejack, Edwin Choy, Kristen N Ganjoo, Arthur P Staddon, Warren A Chow, Hussein A Tawbi, Brian L Samuels, Shreyaskumar R Patel, Margaret von Mehren, Gina D'Amato, Kirsten M Leu, David M Loeb, Charles A Forscher, Mohammed M Milhem, Daniel A Rushing, David R Lucas, Rashmi Chugh, Denise K Reinke, Laurence H Baker
BACKGROUND: Alveolar soft part sarcoma (ASPS), chondrosarcoma (CS), chordoma, epithelioid sarcoma, and solitary fibrous tumor (SFT) are malignant tumors that are relatively resistant to chemotherapy and for which more effective drug therapy is needed. METHODS: The 5 listed subtypes were enrolled into a single indolent sarcoma cohort in a phase 2 study of dasatinib using a Bayesian continuous monitoring rule for enrollment. The primary objective was to estimate the 6-month progression-free survival (PFS) rate according to the Choi criteria with a target of ≥50%...
October 3, 2016: Cancer
https://www.readbyqxmd.com/read/27684838/uncommon-liver-tumors-case-report-and-literature-review
#13
Chia-Hung Wu, Nai-Chi Chiu, Yi-Chen Yeh, Yu Kuo, Sz-Shian Yu, Ching-Yao Weng, Chien-An Liu, Yi-Hong Chou, Yi-You Chiou
BACKGROUND: Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms. METHODS: From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27683125/reduced-argininosuccinate-synthetase-expression-in-refractory-sarcomas-impacts-on-therapeutic-potential-and-drug-resistance
#14
Youngji Kim, Eisuke Kobayashi, Daisuke Kubota, Yoshiyuki Suehara, Kenta Mukaihara, Keisuke Akaike, Ayumu Ito, Kazuo Kaneko, Hirokazu Chuman, Akira Kawai, Shigehisa Kitano
BACKGROUND: Treating drug-resistant sarcomas remains a major challenge. The present study aimed to identify a novel therapy for drug-resistant sarcomas based on metabolic errors involving argininosuccinate synthetase1 (ASS1). RESULTS: ASS1 expression was reduced in Dox-resistant sarcoma cells. Immunohistochemistry and real-time PCR showed an inverse correlation between ASS1 and P-gp expressions. The inhibition of cellular proliferation with G1-arrest was shown to lead to autophagy with arginine deprivation...
September 23, 2016: Oncotarget
https://www.readbyqxmd.com/read/27641819/-a-hybrid-lesion-low-grade-fibromyxoid-sarcoma-lgfms-and-sclerosing-epithelioid-fibrosarcoma-sef
#15
Carole Kesrouani, Leila Zemoura, Martine Trassard, Marick Laé
We report the case of a 34-year-old woman presenting a 10cm axillary mass diagnosed as hybrid tumor low-grade fibromyxoid sarcoma/sclerosing epithelioid fibrosarcoma. Microbiopsy for morphological and immunohistochemical analyses was associated with molecular analysis (FISH and PCR) to confirm the diagnosis.
October 2016: Annales de Pathologie
https://www.readbyqxmd.com/read/27631873/sclerosing-epithelioid-fibrosarcoma
#16
James W Patterson, Georgi Tchernev, Anastasiya Atanasova Chokoeva, Mark R Wick
We report the case of a 55-year-old woman who developed a mass in the soft tissue of the anterior right proximal thigh. Microscopic findings in the biopsy specimen supported the diagnosis of sclerosing epithelioid fibrosarcoma (SEF). This entity is part of a spectrum of lesions that includes low-grade fibromyxoid sarcoma (LGFMS) and hyalinizing spindle cell tumor with giant rosettes (HSCTGR). It shares with LGFMS occasional overlapping histopathologic features and immunopositivity for MUC4. Although FUS and CREB3L2 gene rearrangements, characteristics of LGFMS, have been found in hybrid tumors (those with features of both SEF and LGFMS) and in examples of LGFMS with relapses showing SEF morphology, it appears that EWSR1 and CREB3L1 rearrangements predominate over FUS and CREB3L2 rearrangements among "pure" SEF tumors...
September 15, 2016: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27589687/phase-i-dose-escalation-study-of-the-mtor-inhibitor-sirolimus-and-the-hdac-inhibitor-vorinostat-in-patients-with-advanced-malignancy
#17
Haeseong Park, Ignacio Garrido-Laguna, Aung Naing, Siqing Fu, Gerald S Falchook, Sarina A Piha-Paul, Jennifer J Wheler, David S Hong, Apostolia M Tsimberidou, Vivek Subbiah, Ralph G Zinner, Ahmed O Kaseb, Shreyaskumar Patel, Michelle A Fanale, Vivianne M Velez-Bravo, Funda Meric-Bernstam, Razelle Kurzrock, Filip Janku
Preclinical models suggest that histone deacetylase (HDAC) and mammalian target of rapamycin (mTOR) inhibitors have synergistic anticancer activity. We designed a phase I study to determine the safety, maximum tolerated dose (MTD), recommended phase II dose (RP2D), and dose-limiting toxicities (DLTs) of combined mTOR inhibitor sirolimus (1 mg-5 mg PO daily) and HDAC inhibitor vorinostat (100 mg-400 mg PO daily) in patients with advanced cancer. Seventy patients were enrolled and 46 (66%) were evaluable for DLT assessment since they completed cycle 1 without dose modification unless they had DLT...
August 31, 2016: Oncotarget
https://www.readbyqxmd.com/read/27574351/management-of-epithelioid-sarcoma-of-the-inguinal-region-with-vascular-invasion
#18
Amit Mahajan, Indrani Sen, Darpanarayan Hazra, Sunil Agarwal
Epithelioid sarcoma (ES) is a rare clinically polymorphic tumor that mimics both benign and malignant conditions. It presents with dermal or subcutaneous nodules on the extremities in young adults. We present here a case of epithelioid sarcoma of the inguinal region infiltrating the femoral vessels. Biopsy is diagnostic and good histopathological evaluation is critical in management.
August 2016: Indian Journal of Surgery
https://www.readbyqxmd.com/read/27515856/diagnostic-utility-of-fosb-immunohistochemistry-in-pseudomyogenic-hemangioendothelioma-and-its-histological-mimics
#19
Shintaro Sugita, Hiroshi Hirano, Noriaki Kikuchi, Terufumi Kubo, Hiroko Asanuma, Tomoyuki Aoyama, Makoto Emori, Tadashi Hasegawa
BACKGROUND: Pseudomyogenic hemangioendothelioma (PHE) is an unusual vascular tumor of intermediate malignancy that rarely metastasizes and tends to arise in the lower limbs of young adults and children. Histologically, PHE shows fascicular proliferation of eosinophilic spindle cells and/or epithelioid cells showing "pseudomyogenic" morphology. Immunohistochemically, PHE is usually positive for vimentin, cytokeratin, CD31 and ERG. METHOD: We examined FOSB immunohistochemistry (IHC) in 27 cases consisting of 4 PHE and its histologic mimics including 6 epithelioid hemangioendotheliomas (EHE), 8 angiosarcomas (AS), 4 Kaposi sarcomas (KS) and 5 epithelioid sarcomas (ES)...
August 11, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27493191/epithelioid-sarcoma-like-hemangioendothelioma-on-the-chest-wall
#20
Norifumi Tsubokawa, Hiroaki Harada, Daiki Taniyama, Takahiro Uemura, Kazuya Kuraoka, Yoshinori Yamashita
Epithelioid sarcoma-like hemangioendothelioma is a rare vascular neoplasm that usually occurs in the distal extremities of young adults. Although the overall behavior of this neoplasm is usually indolent with a low risk of distant metastasis, the risk of local recurrence is significant. Therefore, initial surgical treatment with an adequate margin is important to improve the prognosis. However, epithelioid sarcoma-like hemangioendothelioma is frequently misdiagnosed. A preoperative misdiagnosis could result in resection without a sufficient margin...
October 2016: Asian Cardiovascular & Thoracic Annals
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