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Epithelioid sarcoma

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https://www.readbyqxmd.com/read/28708250/cutaneous-syncytial-myoepithelioma-a-recently-described-neoplasm-which-may-mimic-nevoid-melanoma-and-epithelioid-sarcoma
#1
Ahmed K Alomari, Noah Brown, Aleodor Andea, Bryan L Betz, Rajiv M Patel
Myoepithelial cells possess dual immunohistochemical and ultrastructural features of smooth muscle cells and epithelial cells and are capable of divergent differentiation (1). As such, benign and malignant tumors of myoepithelial cells show a spectrum of morphologies and have been reported in a variety of anatomic locations, including but not limited to, the breast, larynx, soft tissues, salivary glands and more recently the skin (2-6).
July 14, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28699109/malignant-mesenchymal-neoplasms-of-the-dermis-and-subcutis-mimicking-benign-lesions-a-case-based-review
#2
Thomas Mentzel, Thomas Brenn
In this short review, malignant mesenchymal neoplasms of the dermis and subcutis mimicking benign lesions and their differential diagnoses are discussed. These include plaque-like dermatofibrosarcoma protuberans, superficial low-grade fibromyxoid sarcoma, low-grade superficial malignant peripheral nerve sheath tumour, epithelioid sarcoma, pseudomyogenic haemangioendothelioma, Kaposi sarcoma mimicking cavernous haemangioma and benign lymphangioendothelioma, and rare forms of angiosarcoma mimicking a benign vascular lesion...
July 12, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28698435/-histology-specific-chemotherapy-in-soft-tissue-sarcomas
#3
Eisuke Kobayashi, Akira Kawai
Soft-tissue sarcomas(STSs)are rare mesenchymal tumors, accounting for less than 1%of all adult malignancies. STSs also have diversity, with more than 50 different histological subtypes. While surgical complete resection is a definitive treatment for localized STS, chemotherapy is the treatment option for managing locally advanced and metastatic STS. Although doxorubicin ±ifosfamide is still the first-line therapy for most STS subtypes, some STSs(alveolar soft part sarcoma, clear cell sarcoma, epithelioid sarcoma and extraskeletal myxoid chondrosarcoma)have been reported to have little response to these cytotoxic chemotherapies...
June 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28697683/metastatic-sclerosing-epithelioid-fibrosarcoma-in-bone-marrow
#4
Rana Asakra, Shane Zaidi, Khin Thway
Sclerosing epithelioid fibrosarcoma (SEF) is an aggressive neoplasm thought to be related to low-grade fibromyxoid sarcoma, which typically occurs in middle-aged adults in the deep soft tissues of the lower extremities and trunk. It comprises nests and cords of relatively uniform epithelioid polygonal cells with clear or eosinophilic cytoplasm in densely sclerotic stroma, and it is typically associated with EWSR1 gene rearrangements, and most commonly EWSR1-CREB3L1 fusions. As primary SEF can arise in bone, and bone is also a common metastatic site for SEF, its recognition at this site is important...
July 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28692601/recurrent-braf-gene-rearrangements-in-myxoinflammatory-fibroblastic-sarcomas-but-not-hemosiderotic-fibrolipomatous-tumors
#5
Yu-Chien Kao, Valentina Ranucci, Lei Zhang, Yun-Shao Sung, Edward A Athanasian, David Swanson, Brendan C Dickson, Cristina R Antonescu
Myxoinflammatory fibroblastic sarcoma (MIFS) is a low grade soft tissue sarcoma with a predilection for acral sites, being associated with a high rate of local recurrence but very infrequent distant metastases. Although a t(1;10) translocation resulting in TGFBR3-MGEA5 fusion has been reported as a recurrent genetic event in MIFS, this abnormality is seen only in a subset of cases. As no studies to date have investigated the spectrum of alternative genetic alterations in TGFBR3-MGEA5 fusion negative MIFS, we undertook a genetic analysis of this particular cohort for further molecular classification...
July 7, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28683521/expression-of-cd34-and-%C3%AE-catenin-in-malignant-rhabdoid-tumor-of-the-liver-mimicking-proximal-type-epithelioid-sarcoma
#6
Woo Cheal Cho, Fabiola Balarezo
No abstract text is available yet for this article.
July 7, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28682861/right-femoral-pathological-fracture-caused-by-primary-bone-epithelioid-angiosarcoma-case-report
#7
Yatong Li, Xiongfei Zou, Xiaoyan Chang, Xiao Chang, Shengfang Sun, Baozhong Zhang
RATIONALE: Epithelioid angiosarcoma (EAS) is an extremely rare malignant disease, which accounts no more than 1% of all soft tissue sarcomas. In this article, we would report a new case of EAS with multiple bone destruction and right femoral pathological fracture, which was an even rarer manifestation of EAS. PATIENT CONCERNS: In this case, a 64-year-old man with right femoral fracture was reported. He had suffered from a progressive low back pain for about 8 months, and the imaging examinations prompted a multiple bone destruction in his vertebra and lower limbs...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28659687/management-of-epithelioid-sarcoma-of-the-inguinal-region-with-vascular-invasion
#8
Piyush Gupta, R Shankaran
No abstract text is available yet for this article.
June 2017: Indian Journal of Surgery
https://www.readbyqxmd.com/read/28652442/primary-gastric-alk-negative-ebv-negative-anaplastic-large-cell-lymphoma-presenting-with-iron-deficiency-anemia
#9
Wei Zhang, Samuel Burton, Shaobin Wu, Xia Qian, Mhd Nabeel Rajeh, Katie Schroeder, Mark Shuldberg, Adam Merando, Jin-Ping Lai
Anaplastic large cell lymphoma (ALCL) is a rare subtype of non-Hodgkin lymphoma (NHL). Primary gastric anaplastic lymphoma kinase (ALK) negative ALCL is extremely rare. Diagnosis of primary gastric ALK-negative ALCL is difficult to establish and prognosis is worse than ALK-positive ALCL. Here, we report a case of an 82-year-old man with a history of cerebrovascular disease presented with weakness and iron deficiency anemia. He denied any abdominal discomforts. The esophagogastroduodenoscopy revealed a large ulcerated, friable mass in the gastric body which encompassed about 80% of entire stomach...
July 2017: In Vivo
https://www.readbyqxmd.com/read/28648941/low-grade-fibromyxoid-sarcoma-clinical-morphologic-and-genetic-features
#10
REVIEW
Mustafa Mohamed, Cyril Fisher, Khin Thway
Low-grade fibromyxoid sarcoma (LGFMS) is a bland spindle cell neoplasm that typically arises in the deep soft tissues of the proximal extremities or trunk of young adults. The majority of LGFMS are characterized by a recurrent (7;16)(q34;p11) translocation, resulting in the FUS-CREB3L2 fusion gene, which generates a chimeric protein with transcriptional regulatory activity. Small numbers harbor a FUS-CREB3L1 fusion resulting from t(11;16)(p11;p11), whilst rare cases harbor the EWSR1-CREB3L1 fusion. LGFMS is of low to moderate cellularity and consists of bland spindle cells with small, angulated nuclei and scant, wispy cytoplasm, arranged in a whorled growth pattern and typically showing abrupt transition from myxoid to fibrous areas...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28645742/gastrointestinal-stromal-tumors-gist-presenting-in-the-liver-diagnostic-prognostic-and-therapeutic-issues
#11
Natacha Joyon, Jérôme Dumortier, Aude Aline-Fardin, Caroline Caramella, Pierre-Jean Valette, Jean-Yves Blay, Jean-Yves Scoazec, Peggy Dartigues
CONTEXT: Extra-gastrointestinal stromal tumors (E-GIST) presenting in the liver are exceedingly rare and raise difficult diagnostic and therapeutic challenges. METHODS: We report on two cases of liver E-GIST with different clinical presentations. We describe their clinical and imaging features, their histopathological and molecular characteristics, their treatment and their course. RESULTS: The first case was that of a 56-year-old male presenting with a 10-cm liver mass; the initial diagnosis, made in 1986 from a biopsy sample, was leiomyosarcoma; liver transplantation was performed in 1987; no extra-hepatic tumor was found; the course was uneventful until 1999, when tumor recurrence was diagnosed along the initial biopsy route; after reevaluation of available material, the definitive pathological diagnosis was GIST; imatinib treatment resulted in major response; the patient died of end-stage kidney disease 22 years after the initial diagnosis and 9 years after tumor recurrence...
June 20, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28630763/malignant-tumours-of-the-foot-and-ankle
#12
E Mascard, N Gaspar, L Brugières, C Glorion, S Pannier, A Gomez-Brouchet
Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients...
May 2017: EFORT open reviews
https://www.readbyqxmd.com/read/28622428/cutaneous-pseudomyogenic-epithelioid-sarcoma-like-hemangioendothelioma-fosb-immunohistochemistry-demonstrating-the-serpine1-fosb-fusion-gene
#13
Victoria Alegría-Landa, Carlos Santonja, Margarita Jo-Velasco, Heinz Kutzner, Luis Requena
Pseudomyogenic hemangioendothelioma is a distinctive vascular neoplasm characterized by a multicentric presentation that mostly involves the lower extremities of young adults. Histopathologic resemblance to epithelioid sarcoma and co-expression of both keratins and endothelial markers are its hallmarks. A specific SERPINE1-FOSB fusion gene derived from t(7;19)(q22;q13) with significantly higher FOSB mRNA expression in neoplastic cells is the characteristic cytogenetic anomaly of this tumor. This article is protected by copyright...
June 16, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28598585/epithelioid-hemangioendotheliomas-of-the-liver-and-lung-in-children-and-adolescents
#14
REVIEW
Simone Hettmer, Geoffroy Andrieux, Jochen Hochrein, Philipp Kurz, Jochen Rössler, Silke Lassmann, Martin Werner, Nikolas von Bubnoff, Christoph Peters, Ewa Koscielniak, Monika Sparber-Sauer, Charlotte Niemeyer, Thomas Mentzel, Hauke Busch, Melanie Boerries
Epithelioid hemangioendothelioma (EHE) is a rare, vascular sarcoma. Visceral forms arise in the liver/ lungs. We review the clinical and molecular phenotype of pediatric visceral EHE based on the case of a 9-year-old male child with EHE of the liver/lungs. His tumor expressed the EHE-specific fusion oncogene WWTR1-CAMTA1. Molecular characterization revealed a low somatic mutation rate and activated interferon signaling, angiogenesis regulation, and blood vessel remodeling. After polychemotherapy and resection of lung tumors, residual disease remained stable on oral lenalidomide...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28569045/a-novel-ews-creb3l3-gene-fusion-in-a-mesenteric-sclerosing-epithelioid-fibrosarcoma
#15
Barbara Dewaele, Louis Libbrecht, Gabriel Levy, Benedicte Brichard, Vanessa Vanspauwen, Raf Sciot, Maria Debiec-Rychter
Sclerosing epithelioid fibrosarcoma (SEF) is a rare, malignant fibroblastic neoplasm, morphologically composed of cords, nests or sheets of monotonous epithelioid cells within a collagenous matrix. It has been recently characterized by recurrent pathogenic EWS-CREB3L1/2 or FUS-CREB3L2 fusions and common MUC4 protein expression by immunohistochemistry. Typically SEF occur in middle-aged adults and rarely have been reported within the abdominal cavity. Here we report an 18-year-old man with intraabdominal tumor and multiple disseminated liver metastases, presenting pure SEF histologic and immunophenotypic features...
May 31, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28567329/carcinosarcoma-of-the-tunica-vaginalis-following-radiation-therapy-for-localized-prostate-cancer
#16
Charles D Viers, Subodh M Lele, Tara Kirkpatrick, Chad A LaGrange
Spermatic cord tumors (SCTs) are rare neoplasms with 80% exhibiting benign pathology. Of the malignant SCTs, 90% are sarcomas. To date there has only been one documented case of primary CS of the spermatic cord which occurred in a 40 year old with no reported medical history. A 76-year-old male with a history of biopsy proven Gleason score 7 (3 + 4) prostatic adenocarcinoma underwent external beam radiation therapy (7920 cGy) in 44 fractions in 2004. He presented with a 3 year history of an asymptomatic right hydrocele...
July 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28544573/spindle-cell-subtype-of-pulmonary-clear-cell-tumor-with-prominent-calcification-and-malignant-potential
#17
Yinghua Song, Fangfang Chen, Caiqing Zhang, Xiaoyan Lin
Perivascular epithelioid cell tumor of the lung, also known as clear cell "sugar" tumor, is a rare benign tumor arising from perivascular epithelioid cells. Herein, we present a case of spindle cell subtype of pulmonary perivascular epithelioid cell tumor with prominent calcification and malignant potential in a 49-year-old woman. Histologically, the striking feature of this lesion was attributed to the presence of spindle cells arranged in a diffuse pattern, which is a pitfall for diagnosis. However, some of the lesion contained polygonal tumor cells with clear abundant cytoplasm surrounded by thin-walled vascular spaces...
May 25, 2017: Thoracic Cancer
https://www.readbyqxmd.com/read/28543105/-alk-oncoproteins-in-atypical-inflammatory-myofibroblastic-tumours-novel-rrbp1-alk-fusions-in-epithelioid-inflammatory-myofibroblastic-sarcoma
#18
(no author information available yet)
No abstract text is available yet for this article.
June 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28541826/ini-expressing-epithelioid-sarcoma-with-osteoclastic-giant-cells-in-a-child-a-case-report-with-summary-of-prior-published-cases
#19
Riju Bhattacharyya, Ranajoy Ghosh, Koushik Saha, Uttara Chatterjee
BACKGROUND: Epithelioid sarcoma is a heterogeneous tumor with 2 subtypes, classic and proximal. The proximal variant is more aggressive and occurs in proximal location in young adults. CASE REPORT: We present a proximal epithelioid sarcoma in the leg of an 8 year old girl with rhabdoid morphology and scattered osteoclastic giant cells. Nuclear INI-1 was retained. Despite wide local excision, local recurrence occurred at 8 months. Following re-excision, she developed a chest wall metastasis after 9 months...
May 25, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28535796/identification-of-eml4-alk-as-an-alternative-fusion-gene-in-epithelioid-inflammatory-myofibroblastic-sarcoma
#20
Quan Jiang, Han-Xing Tong, Ying-Yong Hou, Yong Zhang, Jing-Lei Li, Yu-Hong Zhou, Jing Xu, Jiong-Yuan Wang, Wei-Qi Lu
BACKGROUND: Known as solid tumors of intermediate malignant potential, most inflammatory myofibroblastic tumors (IMTs) are treatable as long as the tumor is en-bloc resected. However, in some cases, the tumors have recurred and grown rapidly after successful surgery. Some of these tumors were classified as an epithelioid inflammatory myofibroblastic sarcoma (EIMS). Most previously reported EIMSs have been caused by RANBP2-ALK fusion gene. We herein report an EIMS case caused by an EML4-ALK fusion gene...
May 23, 2017: Orphanet Journal of Rare Diseases
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