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Epithelioid sarcoma

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https://www.readbyqxmd.com/read/28197724/histological-and-immunohistochemical-characteristics-of-undifferentiated-small-round-cell-sarcomas-associated-with-cic-dux4-and-bcor-ccnb3-fusion-genes
#1
Yuichi Yamada, Masaaki Kuda, Kenichi Kohashi, Hidetaka Yamamoto, Junkichi Takemoto, Takeaki Ishii, Kunio Iura, Akira Maekawa, Hirofumi Bekki, Takamichi Ito, Hiroshi Otsuka, Makoto Kuroda, Yumi Honda, Shinji Sumiyoshi, Takeshi Inoue, Naoe Kinoshita, Atsushi Nishida, Kyoko Yamashita, Ichiro Ito, Shizuo Komune, Tomoaki Taguchi, Yukihide Iwamoto, Yoshinao Oda
CIC-DUX4 and BCOR-CCNB3 fusion-gene-associated small round cell sarcomas account for a proportion of pediatric small round cell sarcomas, but their pathological features have not been sufficiently clarified. We reviewed a large number of soft tissue tumors registered at our institution, retrieved the cases of unclassified tumors with a small round cell component, and subjected them to histopathological, immunohistochemical, and gene profile analysis. We reviewed 164 cases of unclassified tumors with a small round cell component and analyzed them by RT-PCR and FISH...
February 14, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28195103/malignant-melanocytic-neoplasm-of-pancreas-with-liver-metastasis-is-it-malignant-melanoma-or-clear-cell-sarcoma
#2
Thomas Alex Kodiatte, Sam Varghese George, Raju Titus Chacko, Banumathi Ramakrishna
Malignant melanocytic neoplasm, usually seen in soft tissues, is rare in a visceral location and presents as a diagnostic dilemma. We present a case of pancreatic malignant melanocytic neoplasm with liver metastasis. A 58-year-old man presented with left upper abdominal swelling and loss of appetite. Imaging revealed a large mass arising from the pancreatic tail, and this was diagnosed as malignant neoplasm with melanocytic differentiation on biopsy with the possible differentials of malignant melanoma, clear cell sarcoma (CCS), and perivascular epithelioid cell neoplasm...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28152587/malignant-solitary-fibrous-tumor-with-heterologous-rhabdomyosarcomatous-differentiation-a-case-report
#3
Jeong-Hwa Kwon, Joon Seon Song, Hye Won Jung, Jong-Seok Lee, Kyung-Ja Cho
Malignant solitary fibrous tumor (MSFT) is a well-described entity, from which heterologous differentiation is extremely rare. We encountered a case of MSFT with rhabdomyosarcomatous differentiation in a 56-year-old man. This patient presented with a large mass in his posterior thigh. He had been treated with chemoradiation for sarcoma involving the cervical spine, right femoral head, and both lungs 6 months earlier. A wide excision was performed. The mass measured 10.6 cm and showed a fish-flesh cut surface with necrotic foci...
February 3, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28109176/oncogenic-roles-of-smarcb1-ini1-and-its-deficient-tumors
#4
Kenichi Kohashi, Yoshinao Oda
SMARCB1/INI1 is one of the core subunit proteins of the ATP-dependent SWI/SNF chromatin remodeling complex, and is identified as a potent and bona fide tumor suppressor. Interactions have been demonstrated between SMARCB1/INI1 and key proteins in various pathways related to tumor proliferation and progression: the p16-RB pathway, WNT signaling pathway, sonic hedgehog signaling pathway and Polycomb pathway. Initially, no detectable SMARCB1/INI1 protein expression was found in malignant rhabdoid tumor cells, whereas all other kinds of tumor cells and non-tumorous tissue showed SMARCB1/INI1 protein expression...
January 21, 2017: Cancer Science
https://www.readbyqxmd.com/read/28096927/sclerosing-epithelioid-fibrosarcoma-metastasizing-to-the-penile-shaft
#5
Michael D Bell, Fadi Brimo, Sungmi Jung, Armen G Aprikian
We present the case of a 50-year-old man with a periurethral mass. He was previously known for sclerosing epithelioid fibrosarcoma (SEF) of the left foot, having an amputation for local recurrence with >2 cm negative margins. A solid periurethral mass was surgically excised seven months later, yielding the diagnosis of metastatic SEF. This is the first documented metastasis of SEF to the penis. These sarcomas have proven difficult to treat, with high recurrence rates despite a multimodal approach.
November 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28086809/primary-mediastinal-clear-cell-sarcoma-a-case-report-and-review-of-the-literature
#6
REVIEW
Long Jin, Yuxia Sui, Haili Zhu, Zhizhong Chen, Shuguang Liu
BACKGROUND: Clear cell sarcoma (CCS) is a rare malignant soft-tissue neoplasm that displays melanocytic markers and exhibits striking histopathological features. The tumour has a predilection for the lower extremities and rarely presents in the mediastinum. CASE PRESENTATION: We present a case of primary mediastinal CCS in a 57-year-old man. Computer tomography (CT) revealed a 12 × 12 × 7.5 cm mass in the anterior mediastinum. Microscopically, the tumour mainly consisted of epithelioid cells with oval vesicular nuclei and eosinophilic cytoplasm...
January 13, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28012782/epithelioid-hemangioendothelioma-presenting-as-inferior-vena-cava-obstruction-diagnosed-using-an-endovascular-thrombectomy-device
#7
Loay Kabbani, Praveen Balraj, Jeff Mathews, Francis Tinney, Scott Schwartz, David Kwon, Felix Braun, Sara Wasilenko
Epithelioid hemangioendothelioma is a rare vascular sarcoma that arises from the lining (intima) of blood vessels. We report a case of epithelioid hemangioendothelioma in a 43-year-old male patient, who presented with inferior vena cava obstruction and acute bilateral lower extremity deep vein thrombosis. Mechanical thrombectomy was performed with an endovascular thrombectomy device, followed by stent placement in the inferior vena cava. The initial pathology on the retrieved specimen revealed an undifferentiated neoplasm, and surgical resection of the retroperitoneal soft tissue tumor of the inferior vena cava documented a rare case of epithelioid hemangioendothelioma...
December 21, 2016: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28009608/fosb-is-a-useful-diagnostic-marker-for-pseudomyogenic-hemangioendothelioma
#8
Yin P Hung, Christopher D M Fletcher, Jason L Hornick
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a distinctive vascular neoplasm of intermediate biological potential with a predilection for young adults and frequent multifocal presentation. Pseudomyogenic hemangioendothelioma is characterized by loose fascicles of plump spindled and epithelioid cells with abundant eosinophilic cytoplasm and coexpression of keratins and endothelial markers. Recently, a SERPINE1-FOSB fusion has been identified as a consistent genetic alteration in pseudomyogenic hemangioendothelioma...
December 22, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27926791/high-prevalence-of-mitf-staining-in-undifferentiated-pleomorphic-sarcoma-caution-in-the-use-of-melanocytic-markers-in-sarcoma
#9
Bonnie Choy, Elizabeth Hyjek, Anthony G Montag, Peter Pytel, Rex Haydon, Hue H Luu, Chao J Zhen, Bradley C Long, Sabah Kadri, Jeremy P Segal, Larissa V Furtado, Nicole A Cipriani
AIMS: The 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 naevi and epithelioid melanomas, as well as some mesenchymal neoplasms. The aim of this study was to evaluate 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 immunostaining was performed on resection specimens from 19 UPSs and five AFXs...
December 7, 2016: Histopathology
https://www.readbyqxmd.com/read/27922155/metastatic-epithelioid-sarcoma-revealed-with-18-f-fdg%C3%A2-pet-ct-imaging
#10
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
#11
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
#12
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...
February 2017: Journal of Pathology
https://www.readbyqxmd.com/read/27818284/cardiac-angiosarcoma-histopathologic-immunohistochemical-and-cytogenetic-analysis-of-10-cases
#13
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...
February 2017: Human Pathology
https://www.readbyqxmd.com/read/27800093/proximal-type-epithelioid-sarcoma-a-new-case-report-and-literature-review
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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%...
January 1, 2017: Cancer
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