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Epithelioid sarcoma

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https://www.readbyqxmd.com/read/28511396/a-clinicopathological-study-of-paediatric-liver-tumours-in-a-tertiary-care-hospital
#1
Esakki Muthuvel, Vimal Chander, Chitra Srinivasan
INTRODUCTION: Paediatric primary liver tumours are the third largest group of solid abdominal neoplasms in children next to neuroblastoma and Wilms tumour, accounting for about 0.5% to 2% of all paediatric neoplasms, in which hepatoblastoma is the most common. AIM: The present study was done to estimate the incidence of paediatric liver tumours over a period of five years and also, to study the clinical behaviour, alpha-fetoprotein correlation and histopathological features of paediatric liver tumours...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28506772/treatment-of-epithelioid-angiosarcoma-with-topical-ala-pdt-in-the-course-of-surgery
#2
Yang Gao, Wen-Sheng Wang, Hai-Lin Wang, Jia Liu, Yuan-Gang Lu
BACKGROUND: Epithelioid angiosarcoma is a kind of high grade malignancy sarcoma of soft tissue, which is hard to diagnose and completely exsect without causing functional and cosmetic problems. Moreover, the high rate of recurrence is hard to handle. Photodynamic therapy is a novel treatment protocol which can selectively destroy tumor cell with good functional and cosmetic outcomes. METHODS: This is a case about a 81 years old patient with Epithelioid angiosarcoma in the right medial angle of eye, which received surgery and photodynamic therapy...
May 12, 2017: Photodiagnosis and Photodynamic Therapy
https://www.readbyqxmd.com/read/28505957/spindle-cell-type-malignant-peripheral-nerve-sheath-tumor-arising-in-benign-schwannoma-with-multiple-intraosseous-spinal-metastasis-a-case-report
#3
Jae Yoon Chung, Sung Sun Kim, Sung Kyu Kim
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) arising in benign schwannoma with multiple intraosseous spinal metastasis is extremely rare, having a highly aggressive progression and poor prognosis. In such cases, the malignant cells of MPNST usually have an epithelioid morphology. Here, the authors present a very rare case of spindle cell type MPNST arising in benign schwannoma. CASE: A 47-year-old woman had a history of wide marginal excision of right buttock spindle cell sarcoma previously...
May 5, 2017: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/28498412/emmprin-released-as-a-microvesicle-in-epithelioid-sarcoma-interacts-with-fibroblasts
#4
Mikiko Aoki, Kaori Koga, Makoto Hamasaki, Nagayasu Egawa, Kazuki Nabeshima
Emmprin (extracellular matrix metalloproteinase inducer, CD147) is a glycosylated transmembrane protein, consisting of two immunoglobulin domains, that stimulates the production of matrix metalloproteinases (MMPs) by tumor-associated fibroblasts. These effects play important roles in tumor invasion and metastasis. However, the precise mechanisms by which emmprin acts on fibroblasts have not been fully elucidated, especially in sarcoma cells. Previously, we demonstrated that emmprin, expressed in conditioned medium collected from the epithelioid sarcoma cell line (FU-EPS-1), stimulates MMP-2 production via interactions with fibroblasts...
May 8, 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/28475514/recurrent-cutaneous-angiosarcoma-of-the-scalp-with-aberrant-expression-of-s100-a-case-report
#5
Laura Macías-García, Concepción Lara-Bohorquez, Enrique Jorquera-Barquero, Juan J Ríos-Martín
Angiosarcoma (AS) is a malignant mesenchymal neoplasm of endothelial origin with a predominantly lymphatic immunophenotype, which accounts for less than 1% of all sarcomas. Cutaneous AS of the scalp is associated with high rates of local recurrence and a poor prognosis. Histologically, poorly differentiated AS often comprises solid epithelioid cells, although rare variants involving spindle cells have been reported; diagnosis requires immunohistochemical analysis using vascular cell markers. We report on a cutaneous spindle-cell AS of the scalp in a female patient; key features included spontaneous regression after biopsy, local recurrence 2 years later, and aberrant nuclear staining for S100 protein in an area of the tumor not expressing CD34 or D2-40...
April 25, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28452074/clinicopathologic-features-and-immunohistochemical-spectrum-of-11-cases-of-epithelioid-malignant-peripheral-nerve-sheath-tumors-including-ini1-smarcb1-results-and-braf-v600e-analysis
#6
Bharat Rekhi, Kemal Kosemehmetoglu, Gaye Guler Tezel, Sergulen Dervisoglu
Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare, relatively less chemosensitive sarcoma. We report clinicopathologic features of 11 epithelioid MPNSTs, including rare forms, along with INI1 immunostaining and BRAF V600E mutation results. BRAF V600E mutation was tested by Real-time polymerase chain reaction (PCR) technique. Eleven tumors occurred in six men and five women (M:F ratio = 0.85:1) within an age range of 5-73 years (average = 44), mostly in lower limbs (five), followed by upper limbs (four)...
April 27, 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28450230/metastatic-alveolar-soft-part-sarcoma-of-the-spinal-cord-a-case-report-and-review-of-literature
#7
Michael J Randazzo, Jayesh P Thawani, Rashmi Manur, John S Brooks, Ali K Ozturk
BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare, malignant soft-tissue neoplasm typically seen in young adults that possesses an unusual tendency to metastasize. Metastases to the intramedullary compartment of the spinal cord, however, are exceptionally rare and have not been described in the literature. CASE DESCRIPTION: We report the case of a 23-year-old woman with disseminated ASPS to the lung and brain who presented with progressive lower extremity weakness and loss of sensation following radiation and chemotherapy...
April 24, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28449608/primary-pulmonary-myxoid-sarcoma-a-newly-described-entity-report-of-a-case-and-review-of-the-literature
#8
Carlos N Prieto-Granada, Rose B Ganim, Lei Zhang, Cristina Antonescu, James Mueller
Primary pulmonary myxoid sarcoma (PPMS) is a recently described, exceedingly rare low-grade lung sarcoma that tends to present in young females as an endobronchial mass and shows evidence of an EWSR1- CREB1 fusion. Herein, we present a case of PPMS with fluorescence in situ hybridization (FISH) analysis for EWSR1 and CREB1 rearrangements. An 80-year-old woman presented with an endobronchial, multinodular tumor exhibiting spindle, ovoid and epithelioid cells arranged in reticular/lattice-like and alveolar-like patterns in a myxoid background...
April 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28427232/embryonic-signature-distinguishes-pediatric-and-adult-rhabdoid-tumors-from-other-smarcb1-deficient-cancers
#9
Wilfrid Richer, Julien Masliah-Planchon, Nathalie Clement, Irene Jimenez, Laetitia Maillot, David Gentien, Benoît Albaud, Walid Chemlali, Christine Galant, Frederique Larousserie, Pascaline Boudou-Rouquette, Amaury Leruste, Celine Chauvin, Zhi Yan Han, Jean-Michel Coindre, Pascale Varlet, Paul Freneaux, Dominique Ranchère-Vince, Olivier Delattre, Franck Bourdeaut
Extra-cranial rhabdoid tumors (RT) are highly aggressive malignancies of infancy, characterized by undifferentiated histological features and loss of SMARCB1 expression. The diagnosis is all the more challenging that other poorly differentiated cancers lose SMARCB1 expression, such as epithelioid sarcomas (ES), renal medullary carcinomas (RMC) or undifferentiated chordomas (UC). Moreover, late cases occurring in adults are now increasingly reported, raising the question of differential diagnoses and emphasizing nosological issues...
March 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28420320/pediatric-non-vestibular-schwannoma
#10
Cory Broehm, Alyaa Al-Ibraheemi, Karen J Fritchie
While the clinicopathologic features of pediatric vestibular schwannomas, often in the context of neurofibromatosis type 2 (NF2), have been well studied, there is less data regarding the characteristics of pediatric non-vestibular schwannomas (NVS). Additionally, the rate of loss of SMARCB1/INI1 expression in this population has not been systematically evaluated. Our institutional archives were searched for cases of NVS arising in patients 18 years or younger. Clinicopathologic features including SMARCB1/INI1 status were assessed for each case...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28381140/proximal-type-epithelioid-sarcoma
#11
David Alfonso Suárez-Zamora, Luis Eduardo Barrera-Herrera, Paula Andrea Rodríguez-Urrego, Mauricio Alfonso Palau-Lázaro
Epithelioid sarcoma is a rare malignant mesenchymal neoplasm (less than 1% of all sarcomas) with epithelioid morphology. Among the 2 subtypes, proximal represents only one-third of cases and commonly involves deep tissues of pelvic region, including the perineum, genital area, and groin, and occurs more frequently in older patients who present a more aggressive course. In the female genital tract, proximal-type epithelioid sarcoma (PES) mainly affects the vulva and is extremely uncommon in the uterus. To our knowledge, only a few cases of PES involving the cervix and uterine body have been previously reported in the literature...
February 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28376587/-epithelioid-sarcoma-with-mesothelial-and-lymphatic-endothelial-differentiation-a-clinicopathologic-analysis-of-10-cases
#12
Z Y Ke, S J Yang
Objective: To investigate the multidirectional differentiation potential in epithelioid sarcoma (ES), with special emphasis on its mesothelial and lymphatic endothelial markers expression. Methods: Ten cases of distal-type ES were included. The clinical, histological, and immunohistochemical(including mesothelial and lymphatic endothelial markers expression)features and follow-up data were evaluated. Results: The patients aged between 8 to 66 years. Five cases were male and five were female. The tumors were located at the palm (2 cases), wrist (3 cases), upper arm (2 cases), poplitealfossa (1 case), lower leg (1 case) and thigh (1 case), respectively...
April 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28346326/sarcomas-with-cic-rearrangements-are-a-distinct-pathologic-entity-with-aggressive-outcome-a-clinicopathologic-and-molecular-study-of-115-cases
#13
Cristina R Antonescu, Adepitan A Owosho, Lei Zhang, Sonja Chen, Kemal Deniz, Joseph M Huryn, Yu-Chien Kao, Shih-Chiang Huang, Samuel Singer, William Tap, Inga-Marie Schaefer, Christopher D Fletcher
CIC-DUX4 gene fusion, resulting from either a t(4;19) or t(10;19) translocation, is the most common genetic abnormality detected in EWSR1-negative small blue round cell tumors. Following their discovery it was debated if these tumors should be classified as variants of Ewing sarcoma (ie, atypical Ewing sarcoma) or as a stand-alone pathologic entity. As such the WHO classification temporarily grouped the CIC-rearranged tumors under undifferentiated sarcomas with round cell phenotype, until further clinical evidence was available...
March 24, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28302385/primary-cutaneous-interdigitating-dendritic-cell-sarcoma-is-a-morphologic-and-phenotypic-simulator-of-poorly-differentiated-metastatic-melanoma-a-report-of-2-cases-and-review-of-the-literature
#14
REVIEW
Cynthia M Magro, Luke C Olson, Gerard Nuovo, Garron J Solomon
Interdigitating dendritic cell sarcoma (IDS) is a rare form of hematologic malignancy associated with an aggressive clinical course. Only 4 prior cases have been described as originating in the skin. We encountered two male patients ages 47 and 61years of age who presented with solitary cutaneous neoplasms diagnosed as IDS. Histologic exam showed a coalescing nested and multinodular proliferation of large pleomorphic epithelioid cells. In one case an initial diagnosis of melanoma was rendered. A recurrence 8months later was then interpreted as a primary cutaneous IDS...
February 20, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28297765/-epithelioid-inflammatory-myofibroblastic-sarcoma-of-small-bowel-mesentery-report-of-a-case
#15
N Fang, Q J Yang, Y T Deng, X Feng, H S Xia, Y G L Zhang, M W Wang, D Wu, H Zhou, F Guo
No abstract text is available yet for this article.
March 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28291056/perspectives-on-low-grade-sarcomas-the-extraordinary-contributions-of-sharon-w-weiss-md
#16
Hope Hastings, John R Goldblum
Soft-tissue pathology encompasses a wide spectrum of neoplasms that represent some of the most challenging and problematic tumors in surgical pathology. Owing to the intensive work of dedicated pathologists, this once esoteric field has become increasingly well defined. In this review, Dr Sharon Weiss' monumental contributions to low-grade sarcomas, including low-grade fibromyxoid sarcoma/so-called hyalinizing spindle cell tumor, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma, epithelioid hemangioendothelioma, and dermatofibrosarcoma protuberans with fibrosarcomatous transformation will be discussed...
March 13, 2017: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/28288693/uterine-epithelioid-leiomyosarcoma-with-c-kit-expression-and-ywhae-gene-rearrangement-a-case-report-of-a-diagnostic-pitfall-of-uterine-sarcoma
#17
Terufumi Kubo, Shintaro Sugita, Ryuichi Wada, Noriaki Kikuchi, Masahiro Iwasaki, Yumika Ito, Taro Sugawara, Hiromi Fujita, Makoto Emori, Ryoichi Tanaka, Hiroshi Hirano, Tsuyoshi Saito, Tadashi Hasegawa
BACKGROUND: Uterine sarcoma is a rare tumor that is often difficult to classify based on morphological and immunohistochemical analysis alone. Limited access to molecular biological analysis in routine practice would hinder making a definitive diagnosis. CASE PRESENTATION: In this report, we describe a case of a mesenchymal tumor arising from the uterine cervix in a 52-year-old woman. From microscopic morphology of the resected specimen, epithelioid leiomyosarcoma, high-grade endometrial stromal sarcoma, or uterine gastrointestinal stromal tumor (GIST) were considered as differential diagnoses...
March 14, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28256572/clinicopathological-and-molecular-characterization-of-smarca4-deficient-thoracic-sarcomas-with-comparison-to-potentially-related-entities
#18
Akihiko Yoshida, Eisuke Kobayashi, Takashi Kubo, Makoto Kodaira, Toru Motoi, Noriko Motoi, Kan Yonemori, Yuichiro Ohe, Shun-Ichi Watanabe, Akira Kawai, Takashi Kohno, Hiroshi Kishimoto, Hitoshi Ichikawa, Nobuyoshi Hiraoka
A growing number of studies suggest critical tumor suppressor roles of the SWI/SNF chromatin remodeling complex in a variety of human cancers. The recent discovery of SMARCA4-deficient thoracic sarcomas has added to the list of tumor groups with the SMARCA4 inactivating mutation. To better characterize these tumors and establish their nosological status, we undertook a clinicopathological and molecular analysis of 12 SMARCA4-deficient thoracic sarcomas and compared them with three potentially related disease entities...
March 3, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28248728/clinicopathologic-features-of-the-non-cns-primary-ewing-sarcoma-family-of-tumors-in-the-head-and-neck-region
#19
Chang Gok Woo, Bora Lee, Joon Seon Song, Kyung-Ja Cho
Ewing sarcoma family of tumor (ESFT) is a group of malignant neoplasms that affect children and young adults. Primary ESFT does not commonly arise from the head and neck region. This study aimed to elucidate the clinicopathologic characteristics of ESFT of the head and neck region except for central nervous system primitive neuroectodermal tumors. Among the 207 cases of ESFT of the bone and soft tissue, diagnosed at Asan Medical Center during a 20-year period, 25 (12.1%) involved the head and neck region. Of those, 21 were available for histologic, immunohistochemical, and molecular studies...
February 28, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28248724/tle-1-positive-angiomatoid-fibrous-histiocytoma-mimicking-synovial-sarcoma
#20
Daniel J Zaccarini, Rana Naous, Yesha Sheth, Ola El-Zammar, Gustavo de la Roza, Christopher M Curtiss
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor most commonly seen in young adults and children. We report a case with morphology and immunohistochemistry supporting the diagnosis of synovial sarcoma. On core biopsy, the tumor had spindle cell and epithelioid morphology with a myxoid background. Staining for transducin-like enhancer of split 1 and CD99 were positive; however, subsequent fluorescence in situ hybridization for SYT (SS18, nBAF chromatin remodeling complex subunit) break apart returned negative...
February 28, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
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