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Epithelioid sarcoma

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https://www.readbyqxmd.com/read/28905842/malignant-perivascular-epithelioid-cell-tumor-of-the-orbit-report-of-a-case-and-review-of-literature
#1
Md Shahid Alam, Bipasha Mukherjee, S Krishnakumar, Jyotirmay Biswas
Perivascular epithelioid cell tumor (PEComa) is a rare neoplasm considered to arise from myomelanocytic cell lineage. The uterus is reportedly the most common site to be involved. Orbital PEComa is extremely rare with only two cases reported till date. A 5-year-old male presented with a right medial orbital mass for the last 6 months. The patient was diagnosed with alveolar soft part sarcoma elsewhere. Magnetic resonance imaging features were suggestive of lymphangioma with bleeding. The excision biopsy revealed multiple tumor cells comprising epithelioid cells with clear cytoplasm, along with nuclear atypia and mitosis...
September 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28832212/what-factors-influence-the-outcome-of-surgically-treated-soft-tissue-sarcomas-of-the-hand-and-wrist
#2
Matthew T Houdek, Brian E Walczak, Benjamin K Wilke, Sanjeev Kakar, Peter S Rose, Alexander Y Shin
BACKGROUND: Soft tissue sarcomas (STS) of the hand are exceedingly rare. The aim of this study was to review our institution's experience with STS of the hand to identify factors affecting outcomes and survivorship. METHODS: We retrospectively reviewed the records of 46 hand STS treated with definitive surgery at our institution between 1992 and 2013. Pertinent demographics as well as information regarding the surgical procedure, and disease status at latest follow-up were reviewed...
September 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28832074/programmed-death-ligand-1-pd-l1-expression-in-malignant-mesenchymal-tumors
#3
Kemal Kösemehmetoğlu, Ece Özoğul, Berrin Babaoğlu, Gaye Güler Tezel, Gökhan Gedikoğlu
OBJECTIVE: Programmed death ligand 1 (PD-L1) found on tumor cells has recently been reported to have a key role in the development and dissemination of many tumors, such as lung and breast carcinomas. In this study, we retrospectively analyzed PD-L1 expression among different types of sarcomas. MATERIAL AND METHOD: Tissue microarrays of 3-4 mm diameter were composed from paraffin blocks of 222 various sarcomas. Slides prepared from microarrays were stained for PD-L1 antibody (Cell Signaling, E1L3N®) using Leica Bond Autostainer...
2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28805986/histiocytic-sarcoma-new-insights-into-fna-cytomorphology-and-molecular-characteristics
#4
Yin P Hung, Scott B Lovitch, Xiaohua Qian
BACKGROUND: Histiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunophenotypic features of histiocytes. Molecular characteristics of HS and fine-needle aspiration (FNA) criteria for its diagnosis have not been established. METHODS: A case series of HS in 8 FNA samples from 6 patients was reviewed along with histopathologic and clinical data. Immunohistochemistry was performed on cell blocks (3 cases), core biopsies (5 cases), and surgical specimens (4 cases)...
August 2017: Cancer
https://www.readbyqxmd.com/read/28802505/soft-tissue-tumors-of-uncertain-histogenesis-a-review-for-dermatopathologists
#5
REVIEW
Darya Buehler, Paul Weisman
The mesenchymal tumors discussed herein represent a heterogeneous group of neoplasms with distinctive morphologic, immunophenotypic, and molecular genetic features. These uncommon tumors often arise in the dermis and subcutis and can pose a major diagnostic challenge to dermatopathologists because they closely mimic melanoma, carcinoma, fibrous histiocytoma, schwannoma, or granulomatous inflammation. This article reviews the clinical presentation, histopathology, differential diagnosis, and diagnostic pitfalls of epithelioid sarcoma, clear cell sarcoma, perivascular epithelioid cell tumor, ossifying fibromyxoid tumor, pleomorphic hyalinizing angiectatic tumor, and hemosiderotic fibrolipomatous tumor...
September 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/28799063/clinical-outcomes-of-patients-with-epithelioid-sarcomas-impact-and-management-of-nodal-metastasis
#6
Hidetatsu Outani, Yoshinori Imura, Takaaki Tanaka, Satoshi Takenaka, Kazuya Oshima, Kenichiro Hamada, Shigeki Kakunaga, Susumu Joyama, Norifumi Naka, Ikuo Kudawara, Takafumi Ueda, Nobuhito Araki, Hideki Yoshikawa
PURPOSE: An epithelioid sarcoma is a rare histological subtype of a soft tissue sarcoma with a high local recurrence rate, which frequently shows lymph node metastasis. However, because of the rarity of this tumor, the impact of nodal metastasis and its appropriate management remain unclear. The present study investigated the clinical outcomes of patients with epithelioid sarcomas, with a focus on lymph node metastasis. METHODS: We retrospectively evaluated the clinical outcomes of 27 patients with epithelioid sarcomas treated between 1985 and 2015...
August 10, 2017: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/28766950/imaging-features-of-rare-mesenychmal-liver-tumors-beyond-hemangiomas
#7
Rajesh Thampy, Khaled M Elsayes, Christine O Menias, Perry J Pickhardt, Hyunseon C Kang, Sandeep Deshmukh, Kareem Ahmed, Brinda Rao Korivi
Tumors arising from mesenchymal tissue components such as vascular, fibrous and adipose tissue can manifest in the liver. Although histopathology is often necessary for definitive diagnosis, many of these lesions exhibit characteristic imaging features. The radiologist plays an important role in suggesting the diagnosis, which can direct appropriate immunohistochemical staining at histology. The aim of this review is to present clinical and imaging findings of a spectrum of mesenchymal liver tumors such as hemangioma, epithelioid hemangioendothelioma, lipoma, PEComa, angiosarcoma, inflammatory myofibroblastic tumor, solitary fibrous tumor, leiomyoma, leiomyosarcoma, Kaposi sarcoma, mesenchymal hamartoma, undifferentiated embryonal sarcoma, rhabdomyosarcoma and hepatic metastases...
August 2, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28756018/epithelioid-sarcomas-how-important-is-loco-regional-control
#8
A Pradhan, R J Grimer, A Abudu, R M Tillman, S R Carter, L Jeys, P C Ferguson, A M Griffin, J S Wunder
AIMS: To investigate the impact of the method of treatment on the oncological outcomes in patients with epithelioid sarcomas managed at two international speciality sarcoma centres. METHODS: The databases of two centres were used to identify patients treated for epithelioid sarcomas between 1985 and 2012. Patient, tumor, treatment and outcome data was collected. RESULTS: There were 36 males and 18 females with a mean age of 38.3 years (range 9-79)...
September 2017: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28708250/cutaneous-syncytial-myoepithelioma-a-recently-described-neoplasm-which-may-mimic-nevoid-melanoma-and-epithelioid-sarcoma
#9
Ahmed K Alomari, Noah Brown, Aleodor A Andea, Bryan L Betz, Rajiv M Patel
Cutaneous syncytial myoepithelioma is a recently described rare tumor of the dermis. It is derived and composed purely of myoepithelial cells and shows a characteristic syncytial growth pattern of neoplastic cells with little intervening stroma and no recognizable ductal structures. It represents a diagnostic challenge to dermatopathologists given its rarity and unusual immunophenotype. Molecular testing for rearrangement of the EWSR1 gene plays a significant role in confirming the diagnosis in most cases. Herein, we present 2 cases with mundane clinical presentations and challenging histopathological findings...
October 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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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