Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor, which is prone to occur in teenagers DSRCT is a rare malignant tumor that often occurs in adolescents. Early diagnosis is difficult and the prognosis is poor. In this case report, the ultrasonography of DSRCT showed that the huge solid mass was in the abdomen with unclear boundary, irregular shape, insufficient blood supply but without obvious liquefaction and calcification. The masses encircled the vessels, but no evidence of vascular invasion. Intrahepatic metastases with peripheral hypoechoic aureole and abdominal lymph node metastases were observed. The tumor mass compressed adjacent tissues and organs, causing bilateral hydronephrosis and bone erosion. In a word, the ultrasonographic characteristics could be used for diagnosing the DSRCT in the clinic.
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http://dx.doi.org/10.12182/20200960607 | DOI Listing |
Am J Case Rep
December 2024
Department of Molecular Medicine and Surgery, Center for Molecular Medicine, Karolinska Institute, Stockholm, Sweden.
BACKGROUND Limb-girdle muscular dystrophy recessive 1 (LGMDR1) is an autosomal recessive degenerative muscle disorder characterized by progressive muscular weakness caused by pathogenic variants in the CAPN3 gene. Desmoplastic small round cell tumors (DSRCT) are ultra-rare and aggressive soft tissue sarcomas usually in the abdominal cavity, molecularly characterized by the presence of a EWSR1::WT1 fusion transcript. Mouse models of muscular dystrophy, including LGMDR1, present an increased risk of soft tissue sarcomas.
View Article and Find Full Text PDFBMC Med Imaging
December 2024
Department of Radiology, The First Affiliated Hospital of Xiamen University, 55 Zhenhai Road, Xiamen, 361000, People's Republic of China.
Purpose: Exploring the computed tomography (CT), magnetic resonance imaging (MRI), and fluorodeoxyglucose positron emission tomography (FDG-PET)/CT Multimodal Imaging Characteristics of Desmoplastic Small Round Cell Tumor (DSRCT) to enhance the diagnostic proficiency of this condition.
Methods: A retrospective analysis was performed on clinical data and multimodal imaging manifestations (CT, MRI, FDG-PET/CT) of eight cases of DSRCT. These findings were systematically compared with pathological results to succinctly summarize imaging features and elucidate their associations with both clinical and pathological characteristics.
Cancers (Basel)
December 2024
The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
Desmoplastic small round cell tumors (DSRCTs) of the gastrointestinal (GI) tract are a rare and highly aggressive variant of soft tissue sarcomas, predominantly affecting the abdominal region. These tumors are believed to originate from multipotent mesenchymal stem cells or primitive progenitor cells. They are composed of small round tumor cells associated with prominent stromal desmoplasia, polyphenotypic differentiation, and gene fusion.
View Article and Find Full Text PDFCan Assoc Radiol J
December 2024
Abdominal Division, Joint Department of Medical Imaging, Toronto, ON, Canada.
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