Expanding the Phenotypic Spectrum of Mesenchymal Tumors Harboring the EWSR1-CREM Fusion

ATF1, CREB1, and CREM constitute the CREB family of transcription factors. The genes encoding these factors are involved in gene fusion events in human tumors. EWSR1-ATF1 and EWSR1-CREB1 are the 2 most characterized fusions, whereas EWSR1-CREM has been less studied. To better understand the phenotypic spectrum of mesenchymal tumors associated with the EWSR1-CREM fusion, we investigated archival cases using fluorescence in situ hybridization and/or RNA sequencing. Among 33 clear cell sarcomas of soft tissue tested, we found 1 specimen, a hand tumor bearing the rearrangements of EWSR1 and CREM, with classic histology and immunophenotype. None of 6 clear cell sarcoma-like tumors of the gastrointestinal tract tested harbored the EWSR1-CREM fusion. Among 11 angiomatoid fibrous histiocytomas, we found that 3 tumors of myxoid variant harbored the rearrangements of EWSR1 and CREM. All 3 tumors occurred in middle-aged men and involved the distal extremities (N=2) and the lung (N=1). Prominent lymphoid cuff, fibrous pseudocapsule, and amianthoid fiber were present in 3, 2, and 2 tumors, respectively, whereas none showed pseudoangiomatoid spaces. All 3 tumors were immunohistochemically positive for epithelial membrane antigen and desmin. These cases suggested a closer relationship between angiomatoid fibrous histiocytoma and a recently proposed novel group of myxoid tumors with CREB family fusions. Our cohort also included 2 unclassifiable sarcomas positive for EWSR1-CREM. One of these ...
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research

Related Links:

1295Introduction: Three-dimensional (3-D) displays for medical imaging is emerging field and very promising tools for diagnosis, therapy response and detection of tumor recurrence. Morphological criteria for therapy response (CR, PR, SD, PD) have been established with RECIST (Response evaluation criteria in solid tumors). With adoption of metabolic imaging with PET/CT, particularly with F-18-FDG in initial staging and follow up of tumors, there appears to be a need to modify these criteria not only on morphological imaging modalities like CT, but also on tumor metabolism parameters, like SUV (standardized uptake value) as ...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Musculoskeletal Posters Source Type: research
Liposarcoma of breast is a rare malignant breast tumor. It accounts for 0.3% of the breast sarcomas. Subtypes of breast sarcomas are angiosarcoma (most common), liposarcoma, malignant fibrous histiocytoma, leiomyosarcoma and fibrosarcoma. We hereby report a case of liposarcoma in a 55 year old female emphasizing the imaging features that can aid in differentiating it from benign fat -containing masses, carcinomas and other sarcomas. This is important as treatment of these varying conditions differ widely. Histologically, liposarcoma may be well differentiated, mixed or dedifferentiated (high grade). The risk of metastasis ...
Source: Journal of Radiology Case Reports - Category: Radiology Source Type: research
Abstract Pure mesenchymal sarcomas and phyllodes tumor with sarcomatous differentiation of the breast are rare tumors. Primary breast sarcomas are a heterogenous group of neoplasms which include malignant fibrous histiocytoma, fibrosarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. Sarcomatous differentiation in phyllodes tumor is seen in the form of angiosarcoma, chondrosarcoma, leiomyosarcoma, osteosarcoma, rhabdomyosarcoma, and liposarcoma. Here, we report a case of a 28-year-old pregnant woman with a breast lump during her second trimester which drastically increased in siz...
Source: Indian Journal of Pathology and Microbiology - Category: Pathology Authors: Tags: Indian J Pathol Microbiol Source Type: research
A 59-year-old man presented with a painless 3 cm × 2.5 cm pink nodule with central hemorrhagic crust and surrounding erythema on his right lower leg that had gradually enlarged over the past three years. Notably, the patient had a remote history of malignant fibrous histiocytoma (now undifferentiated pleomorphic sarcoma) of the right distal thigh treated with surgery and radiation. A superficial shave biopsy revealed scar. However, the patient returned two months later complaining that the lesion had enlarged, and a deep shave biopsy demonstrated sheets and fascicles of pleomorphic spindle cells, concerning for sarcoma.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research
This study aimed at analysing clinicopathological, recurrence and survival pattern of surgically treated HNSTS. Case records of 28 patients of HNSTS who underwent surgery at the Regional Cancer Centre (RCC), Trivandrum (India) between 2002 and 2012 were analysed retrospectively for demographic profile, clinical features, treatment given, recurrence pattern and outcome. The median age of patients was 37  years (range, 3–79) with male:female ratio of 3:2. Majority of patients presented with painless lump in the neck as the most common subsite affected followed by scalp and face. One patient had nodal disease, whil...
Source: Indian Journal of Surgical Oncology - Category: Cancer & Oncology Source Type: research
Conclusion Owing to the size, location and findings of the Magnetic Resonance Angiogram we initially suspected a Soft Tissue Sarcoma, but to our surprise, on further investigation the mass was revealed to be Nodular Fasciitis.
Source: International Journal of Surgery Case Reports - Category: Surgery Source Type: research
CONCLUSION: Curcumin and VAE can inhibit the proliferation and viability of STS cells. PMID: 28045549 [PubMed - as supplied by publisher]
Source: Nutrition and Cancer - Category: Cancer & Oncology Authors: Tags: Nutr Cancer Source Type: research
Conclusions Dermatoses at the injection sites of vaccines can be granulomatous, immunity-related conditions, infections, lichenoid, neutrophilic, or pseudolymphomatous. Basal cell carcinoma and squamous cell carcinoma are the most common vaccination site-associated malignancies; however, melanoma and sarcomas (dermatofibrosarcoma protuberans, fibrosarcoma, and malignant fibrous histiocytoma) are also smallpox vaccine-related site neoplasms. A cutaneous immunocompromised district that is created by vaccine-induced local immunologic changes is hypothesized to be the pathogenesis of vaccination site reactions.
Source: Dermatology and Therapy - Category: Dermatology Source Type: research
Authors: Wincewicz A, Lewitowicz P, Matykiewicz J, Głuszek S, Sulkowski S Abstract Here we present a systematic approach to histopathological reporting of high-grade myxofibrosarcoma of 66-year-old male patient. The tumor was biopsied with fine-needle aspiration (FNA) and core-needle biopsy (CNB) and then the whole myxoid tumor was excised with left musculus gluteus maximus. The lesion was stained with Hematoxylin-Eosin (HE), Periodic acid-Schiff (PAS), Alcian blue, Masson's trichrome, Ki67, alpha-smooth muscle actin (α-SMA), S100, CD34 and vimentin. FNA material grounded the diagnosis of non-epithelial neop...
Source: Romanian Journal of Morphology and Embryology - Category: Journals (General) Tags: Rom J Morphol Embryol Source Type: research
Abstract Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma. PMID: 26175585 ...
Source: Korean J Radiol - Category: Radiology Authors: Tags: Korean J Radiol Source Type: research
More News: Fibrosarcoma | Fibrous Histiocytoma | Gastroenterology | Genetics | Pathology | Pediatrics | Sarcomas | Study