Emily J Brady1, Meera Hameed2, William D Tap3, Sinchun Hwang4. 1. Department of Radiology, New York Presbyterian/Weill Cornell, 525 East 68th St, Box 141, New York, NY, 10065, USA. 2. Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, 10065, USA. 3. Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, 10065, USA. 4. Department of Radiology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, New York, NY, 10065, USA. Hwangs1@mskcc.org.
Abstract
OBJECTIVE: To describe the pre-treatment imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. MATERIALS AND METHODS: In this retrospective study, several pre-treatment imaging features (tumor location, size, enhancement pattern, necrosis, flow voids, calcification, and FDG avidity) and the clinical course of patients were evaluated. RESULTS: In 12 patients with CIC-DUX4 sarcomas (median age, 24 years; range, 12-75), sarcomas were located in the soft tissue (n = 10), bone (n = 1), and lungs (n = 1). On MRI, all 10 CIC-DUX4 sarcomas presented as a large necrotic mass (mean size 6.7 cm, range 2.3-11.3) with 100% demonstrating contrast enhancement, 60% showing flow voids, and 20% demonstrating fluid-fluid levels. On PET, the mean SUVmax was 13.2 (range, 8.5-18.1). Among 12 patients with follow-up, 3 died within a year of diagnosis. The most common site of metastases was the lungs (5/12). In 5 patients with BCOR-CCNB3 sarcomas (median age, 14 years; range, 2-17), sarcomas were located in the spine (n = 2), femur (n = 1), tibia (n = 1), and pelvis (n = 1). On radiograph or CT, 2 were lytic, 3 were sclerotic. Soft tissue calcifications occurred in 40% of BCOR-CCNB3 sarcomas. On MRI, all 3 BCOR-CCNB3 tumors enhanced with 33% demonstrating flow voids and 66% exhibiting necrosis. On PET, the mean SUVmax was 6.3 (range 5.7-6.9). CONCLUSION: CIC-DUX4 sarcomas often present as necrotic and hypermetabolic soft tissue masses while sarcomas with BCOR-CCNB3 translocations are vascular bone lesions with necrosis at imaging. CIC-DUX4 sarcomas are clinically more aggressive than BCOR-CCNB3 sarcomas.
OBJECTIVE: To describe the pre-treatment imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. MATERIALS AND METHODS: In this retrospective study, several pre-treatment imaging features (tumor location, size, enhancement pattern, necrosis, flow voids, calcification, and FDG avidity) and the clinical course of patients were evaluated. RESULTS: In 12 patients with CIC-DUX4 sarcomas (median age, 24 years; range, 12-75), sarcomas were located in the soft tissue (n = 10), bone (n = 1), and lungs (n = 1). On MRI, all 10 CIC-DUX4 sarcomas presented as a large necrotic mass (mean size 6.7 cm, range 2.3-11.3) with 100% demonstrating contrast enhancement, 60% showing flow voids, and 20% demonstrating fluid-fluid levels. On PET, the mean SUVmax was 13.2 (range, 8.5-18.1). Among 12 patients with follow-up, 3 died within a year of diagnosis. The most common site of metastases was the lungs (5/12). In 5 patients with BCOR-CCNB3 sarcomas (median age, 14 years; range, 2-17), sarcomas were located in the spine (n = 2), femur (n = 1), tibia (n = 1), and pelvis (n = 1). On radiograph or CT, 2 were lytic, 3 were sclerotic. Soft tissue calcifications occurred in 40% of BCOR-CCNB3 sarcomas. On MRI, all 3 BCOR-CCNB3 tumors enhanced with 33% demonstrating flow voids and 66% exhibiting necrosis. On PET, the mean SUVmax was 6.3 (range 5.7-6.9). CONCLUSION: CIC-DUX4 sarcomas often present as necrotic and hypermetabolic soft tissue masses while sarcomas with BCOR-CCNB3 translocations are vascular bone lesions with necrosis at imaging. CIC-DUX4 sarcomas are clinically more aggressive than BCOR-CCNB3 sarcomas.
Authors: A Raciborska; K Bilska; K Drabko; E Michalak; R Chaber; M Pogorzała; K Połczyńska; G Sobol; M Wieczorek; K Muszyńska-Rosłan; M Rychlowska-Pruszyńska; C Rodriguez-Galindo; M Dziuk Journal: Clin Transl Oncol Date: 2015-08-07 Impact factor: 3.405
Authors: Winnie A Mar; Mihra S Taljanovic; Rochelle Bagatell; Anna R Graham; Donald P Speer; Tim B Hunter; Lee F Rogers Journal: J Comput Assist Tomogr Date: 2008 Jan-Feb Impact factor: 1.826
Authors: Yu-Chien Kao; Adepitan A Owosho; Yun-Shao Sung; Lei Zhang; Yumi Fujisawa; Jen-Chieh Lee; Leonard Wexler; Pedram Argani; David Swanson; Brendan C Dickson; Christopher D M Fletcher; Cristina R Antonescu Journal: Am J Surg Pathol Date: 2018-05 Impact factor: 6.394
Authors: Mark A Applebaum; Jennifer Worch; Katherine K Matthay; Robert Goldsby; John Neuhaus; Daniel C West; Steven G Dubois Journal: Cancer Date: 2011-01-10 Impact factor: 6.860
Authors: B Henninger; B Glodny; A Rudisch; T Trieb; A Loizides; D Putzer; W Judmaier; M F Schocke Journal: Skeletal Radiol Date: 2013-05-19 Impact factor: 2.199
Authors: Elizabeth A Connolly; Vivek A Bhadri; Johnathon Wake; Katrina M Ingley; Jeremy Lewin; Susie Bae; Daniel D Wong; Anne P Long; David Pryor; Stephen R Thompson; Madeleine C Strach; Peter S Grimison; Annabelle Mahar; Fiona Bonar; Fiona Maclean; Angela Hong Journal: Cancer Med Date: 2022-02-17 Impact factor: 4.711