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Epithelioid sarcoma

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https://www.readbyqxmd.com/read/29340997/immunohistochemical-biomarkers-of-mesenchymal-neoplasms-in-endocrine-organs-diagnostic-pitfalls-and-recent-discoveries
#1
Yin P Hung, Jason L Hornick
Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. In this review, we address potential pitfalls in diagnosing selected mesenchymal neoplasms arising within or near endocrine organs, including dedifferentiated liposarcoma, synovial sarcoma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma...
January 16, 2018: Endocrine Pathology
https://www.readbyqxmd.com/read/29302306/alveolar-soft-part-sarcoma-of-flexure-tendon
#2
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/29290913/retroperitoneal-epithelioid-sarcoma-a-case-report
#3
José A Coronado, Miguel Á Chávez, Martín A Manrique, Jony Cerna, Ana L Trejo
Epithelioid sarcoma (ES), a mesenchymatous malign neoformation, is often diagnosed in later stages and associated with high recurrence index, metastasis and mortality. We report a case of a 65 years old male, with history of abdominal pain and upper gastrointestinal bleeding. Endoscopy demonstrated a posterior duodenal wall perforation communicating with a solid retroperitoneal neoformation. Endoscopic biopsy was performed, with a final report of ES. The patient was submitted for surgical palliation due to the tumor's unresectability...
December 16, 2017: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/29290317/histiocytic-sarcoma-with-bladder-involvement-case-report-and-literature-review
#4
Mª Jesús Fernández-Aceñero, Pablo Pérez Alonso, Cristina Díaz Del Arco
We report an unusual case of histiocytic sarcoma with bladder involvement. An 80 year-old man with a previous history of diffuse large B-cell malignant lymphoma presented with hematuria and back pain. Serial urine cytologies revealed no urothelial malignant cells, but cystoscopy showed a large intravesical mass. The patient underwent transurethral resection (TUR) of the tumor. The bladder TUR specimen showed a widely infiltrating epithelioid neoplasm, with intense immunohistochemical positivity for CD45 and histiocytic markers (CD68, lysozime and fascin)...
January 2018: Revista Española de Patología
https://www.readbyqxmd.com/read/29286567/crizotinib-in-alk-inflammatory-myofibroblastic-tumors-current-experience-and-future-perspectives
#5
REVIEW
Till-Martin Theilen, Jan Soerensen, Konrad Bochennek, Martina Becker, Dirk Schwabe, Udo Rolle, Thomas Klingebiel, Thomas Lehrnbecher
Inflammatory myofibroblastic tumor (IMT) and its subtype epithelioid inflammatory myofibroblastic sarcoma (EIMS) are rare soft-tissue tumors. As about 50% of IMT and 100% of EIMS contain activating rearrangements of the anaplastic lymphoma kinase (ALK) gene, targeted kinase inhibition of ALK by compounds such as crizotinib is a potential treatment option. We performed a literature review and analyzed a total of 30 patients with IMT/EIMS treated with crizotinib. A total of 12 patients achieved complete or partial remission...
December 29, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#6
Bruce R Pawel
The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT)...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29279595/pseudomyogenic-epithelioid-sarcoma-like-hemangioendothelioma-a-rare-vascular-neoplasm-with-deceptive-morphology-and-distinctive-immunophenotype
#7
F F Cheo, K Sittampalam
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular (endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremities with a slight tendency of affecting young adult males. We report a case of pseudomyogenic hemangioendothelioma in a 15-year-old boy presenting initially with a 1 cm right thigh painless cutaneous lump. The lump was excised with the clinical impression of a sebaceous cyst. On microscopy, a poorly circumscribed, mild to moderately atypical spindle cell lesion in fascicular and storiform patterns with strikingly myoid-like eosinophilic cytoplasm was identified...
December 2017: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/29275417/cytomorphological-findings-of-follicular-dendritic-cell-sarcoma-on-fine-needle-aspiration-cytology
#8
Sandeep S Ojha, Rubal Jain, Farah Meenai, Ramrao Nilkanthe, Amit Haritwal
BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm arising from follicular dendritic cells of germinal centers. The most common site of origin is lymph nodes and it may mimic a variety of tumors at that location, including carcinomas and sarcomas. Diagnosis is frequently missed on cytology as there are very few case reports describing the cytological characteristics of the lesion. Even on histology, a high degree of suspicion is required for an appropriate diagnosis...
December 23, 2017: Acta Cytologica
https://www.readbyqxmd.com/read/29274042/sclerosing-epithelioid-fibrosarcoma-of-the-jaw-late-recurrence-from-a-low-grade-fibromyxoid-sarcoma
#9
Catherine Laliberte, Iona T Leong, Howard Holmes, Eric A Monteiro, Brian O'Sullivan, Brendan C Dickson
Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon variant of fibrosarcoma that is characterized by a distinct morphology. It most frequently presents in the deep soft tissues of the lower extremities, often in intimate association with fascia and periosteum, although reports of the head and neck involvement have been reported. A minority of cases show morphological, immunohistochemical and molecular overlap with low grade fibromyxoid sarcoma (LG-FMS). Herein, we describe a case of a bland spindle cell neoplasm presenting in the jaw that was initially incompletely excised...
December 22, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/29260248/-chronic-refractory-ulcer-on-the-ring-finger-manifestation-of-distal-epithelioid-sarcoma
#10
N S Häring, R Strohal, S Dertinger, F Offner
The epithelioid sarcoma classic, "distal" type was first published in 1970. It is a very rare, malignant, aggressive subcutaneous soft tissue sarcoma that shows characteristic positivity for both epithelial and mesenchymal immunohistochemical markers. It grows very slowly and mostly presents in young men. Clinically the tumor is characterized as a coarse cutaneous or subcutaneous nodular induration that often ulcerates in the course of the disease. An association with trauma is often described and can lengthen time to diagnosis...
December 19, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/29240581/cutaneous-melanocytoma-with-crtc1-trim11-fusion-report-of-5-cases-resembling-clear-cell-sarcoma
#11
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/29220293/pathologic-angiogenesis-of-malignant-vascular-sarcomas-implications-for-treatment
#12
Jalal A Khan, Robert G Maki, Vinod Ravi
Angiosarcoma, epithelioid hemangioendothelioma, and Kaposi sarcoma are classified according to the line of differentiation that these neoplastic cells most closely resemble: the endothelial cell. Although these malignant vascular sarcomas demonstrate immunohistochemical and ultrastructural features typical of this lineage, they vary dramatically in presentation and behavior, reflecting oncologic mechanisms unique to each. Antineoplastic therapies offer significant benefit, but because of the rarity of these cancers, novel therapies are slow to develop, and treatment options for these cancers remain limited...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29220290/synovial-sarcoma-current-concepts-and-future-perspectives
#13
Silvia Stacchiotti, Brian Andrew Van Tine
Synovial sarcoma (SS) is a rare sarcoma driven by a translocation between SS18 and SSX 1, 2, or 4. With approximately 800 to 1,000 cases a year in the United States, it most commonly affects young adults between the ages of 15 and 30 years. The resultant tumors are either monophasic (pure sarcomas), biphasic (a combination or epithelioid and sarcomatous components), or poorly differentiated. The hybrid transcription factor SS18:SSX alters SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling and global methylation patterns that may allow for future therapeutic opportunities...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29212287/the-benefit-of-everolimus-in-recurrent-epithelioid-angiosarcoma-patients-case-reports-and-literature-review
#14
Shi-Long Zhang, Li Liang, Yuan Ji, Zhi-Ming Wang, Yu-Hong Zhou
Epithelioid angiosarcoma (EA) is a kind of rare malignant soft tissue sarcoma, with high recurrence/metastatic rate and poor prognosis. To date, no effective standardized treatment regimen has been available for patients with recurrence/metastatic EA. Everolimus is an oral rapamycin derivative that highly inhibits the mechanistic target of rapamycin(mTOR) signal pathway. Previous studies have suggested that everolimus is effective and safe in some soft tissue sarcoma. We reported two cases with recurrence/metastatic EA, who received everolimus after failure of surgery, radiotherapy, chemotherapy or interventional therapy...
November 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/29208563/malignant-perivascular-epithelioid-cell-tumor-of-the-oropharynx-with-strong-tfe3-expression-mimicking-alveolar-soft-part-sarcoma-a-case-report-and-review-of-the-literature
#15
Karan Saluja, Jaiyeola Thomas, Songlin Zhang, Erich M Sturgis, Kunal S Jain, Victor G Prieto, Adel K El-Naggar, Diana Bell
Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked as alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition TFE3 gene rearranged PEComa, as a distinct subtype of PEComa is essential to avoid misdiagnosis...
December 2, 2017: Human 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/29202999/epithelioid-sarcoma-of-the-plantar-fascia-mimicking-morbus-ledderhose-a-severe-pitfall-for-clinical-and-histopathological-misinterpretation
#17
Andreas Toepfer, Norbert Harrasser, Florian Dreyer, Carolin Mogler, Markus Walther, Rüdiger von Eisenhart-Rothe
Plantar fibromatosis, also known as Morbus Ledderhose, is a well known and frequently encountered disorder of the planta pedis. When conservative treatment fails, surgical therapy with complete resection is the therapeutical procedure of choice. Soft tissue sarcoma is a heterogeneous and rare malignant disease of the musculoskeletal system with over 50 histopathological subtypes which can potentially arise in any localization but is most commonly found at the extremities. Here, we report the case of an epithelioid sarcoma of the sole of the foot which was initially and repeatedly clinically and histopathologically misinterpreted as plantar fibromatosis, receiving insufficient resection and subsequently ending in amputation of the lower leg...
December 2017: Foot and Ankle Surgery: Official Journal of the European Society of Foot and Ankle Surgeons
https://www.readbyqxmd.com/read/29200103/bcor-internal-tandem-duplication-in-high-grade-uterine-sarcomas
#18
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/29179965/-pseudomyogenic-hemangioendothelioma-in-the-upper-limb-a-case-report-and-literature-review
#19
Carlos Mayoral-Guisado, Weimar Toro-Zambrano, María López-Macías, Antonio Ruíz-Guerrero, Alejandro Rubio-Fernández, Mario Díaz-Delgado
Pseudomyogenic hemangioendothelioma, also called epithelioid sarcoma-like hemangioendothelioma, is a rare, vascular neoplasm usually with indolent behaviour. It was introduced in the latest World Health Organization (WHO) Classification of Tumours of Soft Tissue. We report a case of a 45 year-old patient presenting with a localized, palpable and slightly painful lesion in the left arm. Histologically it consisted of fascicles of spindle and epithelioid cells with ample eosinophilic cytoplasm, without nuclear pleomorphism or significant mitotic activity...
January 2017: Revista Española de Patología
https://www.readbyqxmd.com/read/29174934/primary-lesions-that-may-imitate-metastatic-tumors-histologically-a-selective-review
#20
REVIEW
Mark R Wick
Several primary pathologic entities in diverse anatomic locations have the potential to simulate metastatic neoplasms histologically. Their misinterpretation as such may result in needless and extensive clinical evaluations that are intended to detect a presumed malignancy at another site. More importantly, mistakes of this type can deprive patients of surgical excisions that could be curative. This presentation considers a review of selected primary lesions that can simulate metastases. They include hemangioblastoma, glioblastoma and meningioma with epithelial metaplasia, choroid plexus carcinomas, primary neuroendocrine carcinomas in unusual locations, special forms of sinonasal and salivary glandular adenocarcinoma, clear-cell thyroid carcinomas, unusual microscopic subtypes of pulmonary adenocarcinoma, epithelioid myomelanocytomas ("sugar tumors"), mesotheliomas, primary thymic carcinomas, endodermal choristomas of the interatrial myocardium, peripheral cholangiocarcinoma, adrenocortical carcinoma, adenocarcinomas of the urinary bladder, mucinous and "rhabdoid" tumors of the ovaries, rete testis adenocarcinomas, interdigitating dendritic-cell sarcoma of lymph nodes, selected sweat gland carcinomas, cutaneous Merkel cell carcinoma, primary dermal and subcutaneous melanoma, mucosal and visceral melanomas, epithelioid sarcoma, clear-cell sarcoma, and adamantinoma of long bones...
November 17, 2017: Seminars in Diagnostic Pathology
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