Strategies for the Treatment of Infantile Soft Tissue Sarcomas With BCOR Alterations.

BCOR alterations are described in ultra-rare infantile soft tissue sarcomas including primitive myxoid mesenchymal tumor of infancy and undifferentiated round cell sarcoma (URCS). Previous reports often describe dismal outcomes. Thus, we undertook a retrospective, multi-institutional study of infants with BCOR -rearranged soft tissue sarcomas. Nine patients aged 6 weeks to 15 months were identified. One tumor carried a BCOR :: CCNB3 fusion, whereas 7 tumors harbored internal tandem duplication of BCOR , including 4 cases classified as primitive myxoid mesenchymal tumor of infancy, 1 case as URCS, and 2 cases characterized by a "hybrid morphology" in our evaluation. Four patients underwent upfront surgery with residual disease that progressed locally after a median of 2.5 months. Locoregional recurrences were observed in hybrid patients, and the URCS case recurred with brain metastases. Complete radiographic responses after chemotherapy were achieved in patients treated with vincristine/doxorubicin/cyclophosphamide alternating with ifosfamide/etoposide, vincristine/doxorubicin/cyclophosphamide alternating with cyclophosphamide/etoposide (regimen I), and ifosfamide/carboplatin/etoposide. Seven patients received radiotherapy. With a median of 23.5 months off therapy, 8 patients are with no evidence of disease. In our study, observation was inadequate for the management of untreated postsurgical residual disease. Tumors demonstrated chemosensitivity with anthracycline-based regimens and ifosfamide/carboplatin/etoposide. Radiotherapy was required to achieve durable response in most patients.

Endovascular embolization in renal trauma: a narrative review.

Approximately 1-3% of all trauma patients have a renal injury. Eighty percent of renal trauma is due to blunt injury, with the remainder due to penetrating trauma which is most often iatrogenic. Contrast enhanced computed tomography is used to triage patients and offers a quick and accurate assessment of any potential organ injury. If injury is present, The American Association for the Surgery of Trauma grading system can both grade renal injuries and be used to help guide management and intervention. Grades are assigned based on imaging and clinical features of renal trauma, and have prognostic and treatment implications for patients. The objective of this narrative review is to identify optimal management of patients with renal trauma, specifically which patients can be treated with endovascular interventions following renal trauma, which can be observed, and which would be best managed surgically. For hemodynamically stable patients with renal trauma, endovascular angiography and embolization is a non-invasive approach that can be used to control bleeding and potentially avoid surgery or nephrectomy in select cases. Future research is needed to determine if a specific antibiotic regimen is needed prior to or following embolization. Further research is needed to evaluate the effectiveness of endovascular management of high-grade renal trauma (grade V). Complications of renal embolization include short-term hypertension, long term hypertension in cases of significant ischemia, acute kidney injury, and infection.