Anna Raciborska1, Katarzyna Bilska2, Magdalena Rychłowska-Pruszyńska2, Marek Duczkowski3, Agnieszka Duczkowska3, Katarzyna Drabko4, Radosław Chaber5, Grażyna Sobol6, Elżbieta Wyrobek7, Elżbieta Michalak8, Carlos Rodriguez-Galindo9, Wojciech Wożniak2. 1. Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Kasprzaka 17a str., 01-211 Warsaw, Poland. Electronic address: anna.raciborska@hoga.pl. 2. Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Kasprzaka 17a str., 01-211 Warsaw, Poland. 3. Department of Radiology, Institute of Mother and Child, Kasprzaka 17a str., 01-211 Warsaw, Poland. 4. Department of Pediatric Hematology, Oncology and Bone Marrow Transplantation, Medical University of Lublin, A. Gebali 6 str., 20-093 Lublin, Poland. 5. Department and Clinic of Pediatric Oncology, Hematology and Bone Marrow Transplantation, Wroclaw Medical University, Bujwida 44 str., 50-368 Wroclaw, Poland. 6. Unit of Pediatric Oncology, Hematology and Chemotherapy, Medical University of Silesia, Medyków 16 str., 40-752 Katowice, Poland. 7. Department of Oncology and Hematology, University Children's Hospital of Cracow, Poland. 8. Department of Pathology, Institute of Mother and Child, Kasprzaka 17a str., 01-211 Warsaw, Poland. 9. Pediatric Oncology, Dana-Farber Cancer Institute and Children's Hospital, Harvard Medical School, 450 Brookline Avenue, Boston, MA 02215, USA.
Abstract
BACKGROUND: Ewing sarcoma (ES) is the second most common pediatric malignant bone tumor with a wide spectrum of clinical presentations. Although metastatic disease to the lungs is often the cause of death, isolated lung metastases at diagnosis are not frequent. The specific role of chemotherapy, surgery, and lung radiation has not been clearly defined. We investigated prognostic factors and the impact of the different treatment modalities in a cohort of patients with ES with isolated lung metastases. MATERIALS AND METHODS: Thirty-eight patients with ES and isolated lung metastases were treated using modern multimodal therapy during the period 2000-2014. According to the imaging characteristics of lung nodules patients were allocated into one of four treatment groups: "0" without nodules, "1" one solitary nodule of <0.5cm or several nodules of <0.3cm, "2" solitary nodule of 0.5-1cm or multiple nodules of 0.3-0.5cm, "3" one pulmonary/pleural nodule of >1cm, or more than one nodule of >0.5cm. Factors predictive of outcome were analyzed. Overall survival was estimated by Kaplan-Meier methods and compared using long-rank test and Cox models. RESULTS: Treatment of the lung metastases was performed in 23 cases (60.5%): twenty patients underwent lung surgery, 6 of them received additional postoperative whole lung radiation; three patients received lung radiation only. Malignant cells were found in all lung nodules of patients from group "3", in 5 (62.5%) patients from group "2", and none of the group "1". There was a correlation between histological response of the primary tumor and outcome. Three-year estimates of EFS and OS were 45.19% and 60.7%, respectively. Patients with good response measured by chest CT had significantly better EFS than patients with poor response (81% vs. 27.66%, respectively, p=0.006). CONCLUSIONS: Metastatectomy may have a role in the treatment of highly selected patients with ES and isolated lung metastases depending on the histologic response to therapy. Further studies are needed to better define the use of surgery and the response-adapted criteria in the upfront management of this population.
BACKGROUND:Ewing sarcoma (ES) is the second most common pediatric malignant bone tumor with a wide spectrum of clinical presentations. Although metastatic disease to the lungs is often the cause of death, isolated lung metastases at diagnosis are not frequent. The specific role of chemotherapy, surgery, and lung radiation has not been clearly defined. We investigated prognostic factors and the impact of the different treatment modalities in a cohort of patients with ES with isolated lung metastases. MATERIALS AND METHODS: Thirty-eight patients with ES and isolated lung metastases were treated using modern multimodal therapy during the period 2000-2014. According to the imaging characteristics of lung nodules patients were allocated into one of four treatment groups: "0" without nodules, "1" one solitary nodule of <0.5cm or several nodules of <0.3cm, "2" solitary nodule of 0.5-1cm or multiple nodules of 0.3-0.5cm, "3" one pulmonary/pleural nodule of >1cm, or more than one nodule of >0.5cm. Factors predictive of outcome were analyzed. Overall survival was estimated by Kaplan-Meier methods and compared using long-rank test and Cox models. RESULTS: Treatment of the lung metastases was performed in 23 cases (60.5%): twenty patients underwent lung surgery, 6 of them received additional postoperative whole lung radiation; three patients received lung radiation only. Malignant cells were found in all lung nodules of patients from group "3", in 5 (62.5%) patients from group "2", and none of the group "1". There was a correlation between histological response of the primary tumor and outcome. Three-year estimates of EFS and OS were 45.19% and 60.7%, respectively. Patients with good response measured by chest CT had significantly better EFS than patients with poor response (81% vs. 27.66%, respectively, p=0.006). CONCLUSIONS: Metastatectomy may have a role in the treatment of highly selected patients with ES and isolated lung metastases depending on the histologic response to therapy. Further studies are needed to better define the use of surgery and the response-adapted criteria in the upfront management of this population.
Authors: Vivek Samuel Gaikwad; Rikki Rorima John; Reka Karuppusami; Tarun John K Jacob; Leni Grace Mathew; Jujju Jacob Kurian Journal: J Indian Assoc Pediatr Surg Date: 2022-03-01