Monika Sparber-Sauer1, Thekla von Kalle2, Guido Seitz3, Tobias Dantonello1, Monika Scheer1, Marc Münter4, Joerg Fuchs5, Ruth Ladenstein6, Stefan S Bielack1,7, Thomas Klingebiel8, Ewa Koscielniak1,9. 1. Olgahospital, Pediatrics 5 (Pediatric Oncology, Hematology, Immunology), Zentrum für Kinder-, Jugend- und Frauenmedizin, Klinikum Stuttgart, Stuttgart, Germany. 2. Olgahospital, Institute of Radiology, Zentrum für Kinder-, Jugend- und Frauenmedizin, Klinikum Stuttgart, Stuttgart, Germany. 3. Department of Pediatric Surgery, University Children's Hospital, Marburg, Germany. 4. Department of Radiotherapy, Klinikum Stuttgart, Stuttgart, Germany. 5. Department of Pediatric Surgery and Urology, University Children's Hospital, Tuebingen, Germany. 6. Department of Pediatric Hematology and Oncology, St. Anna Kinderspital Wien, Wien, Austria. 7. Department of Pediatric Hematology and Oncology, University Children's Hospital Muenster, Muenster, Germany. 8. Hospital for Children and Adolescents, University of Frankfurt, Frankfurt/Main, Germany. 9. Department of Pediatric Oncology, University Children's Hospital, Tuebingen, Germany.
Abstract
BACKGROUND: Patients with metastatic rhabdomyosarcoma (RMS) have a poor prognosis apart from children with embryonal RMS whose metastases are confined to the lungs (PRME). The prognostic significance of response in patients with metastatic disease is still unknown and optimal treatment remains to be defined. METHODS: Patient-, tumor- and treatment-related factors of patients with PRME treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (CWS) (1981-2013) were analyzed with a focus on response to induction chemotherapy. Response at week 7-10 was based on anatomic imaging and determined (1) for the primary tumor as complete response (CR), good response (GR), partial response (PR) and no response (NR) and (2) for pulmonary metastases as either complete lack of residual lesions (pCR) or no complete response (no-pCR). Event-free (EFS) and overall survival (OS) were the endpoints. RESULTS: EFS and OS of all 53 eligible patients was 41% (±13 confidence interval [CI] 95%) and 52% (±11 CI 95%), respectively. pCR at week 7-10 and maintenance therapy (MT) were favorable prognostic factors. Interestingly, response of primary tumor at week 7-10 and number of metastases were not prognostic factors. The 5-year OS was 68% (±18 CI 95%) for 26 patients in pCR, but only 36% (±18 CI 95%) for 27 patients not in pCR at week 7-10 (P = 0.004) despite achieving pCR under continuation of chemotherapy or local therapy. CONCLUSION: Achievement of pCR at week 7-10 by induction chemotherapy is a prognostic factor.
BACKGROUND:Patients with metastatic rhabdomyosarcoma (RMS) have a poor prognosis apart from children with embryonal RMS whose metastases are confined to the lungs (PRME). The prognostic significance of response in patients with metastatic disease is still unknown and optimal treatment remains to be defined. METHODS:Patient-, tumor- and treatment-related factors of patients with PRME treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (CWS) (1981-2013) were analyzed with a focus on response to induction chemotherapy. Response at week 7-10 was based on anatomic imaging and determined (1) for the primary tumor as complete response (CR), good response (GR), partial response (PR) and no response (NR) and (2) for pulmonary metastases as either complete lack of residual lesions (pCR) or no complete response (no-pCR). Event-free (EFS) and overall survival (OS) were the endpoints. RESULTS: EFS and OS of all 53 eligible patients was 41% (±13 confidence interval [CI] 95%) and 52% (±11 CI 95%), respectively. pCR at week 7-10 and maintenance therapy (MT) were favorable prognostic factors. Interestingly, response of primary tumor at week 7-10 and number of metastases were not prognostic factors. The 5-year OS was 68% (±18 CI 95%) for 26 patients in pCR, but only 36% (±18 CI 95%) for 27 patients not in pCR at week 7-10 (P = 0.004) despite achieving pCR under continuation of chemotherapy or local therapy. CONCLUSION: Achievement of pCR at week 7-10 by induction chemotherapy is a prognostic factor.
Authors: Timothy B Lautz; Yueh-Yun Chi; Jing Tian; Abha A Gupta; Suzanne L Wolden; Jonathan C Routh; Dana L Casey; Roshni Dasgupta; Douglas S Hawkins; David A Rodeberg Journal: Int J Cancer Date: 2020-02-15 Impact factor: 7.396
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
Authors: Roelof van Ewijk; Reineke A Schoot; Monika Sparber-Sauer; Simone A J Ter Horst; Nina Jehanno; Lise Borgwardt; Bart de Keizer; Johannes H M Merks; Alberto de Luca; Kieran McHugh; Thekla von Kalle; Jürgen F Schäfer; Rick R van Rijn Journal: Pediatr Radiol Date: 2021-06-17