PURPOSE: Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period. PATIENTS AND METHODS: In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). RESULTS: Overall survival at 5 years was 89% in patients who underwent complete resection at diagnosis, 79% in patients who had marginal resection, 52% in initially unresected patients, and 17% in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70%. The rate of response to chemotherapy was 58%. CONCLUSION: The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.
PURPOSE:Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period. PATIENTS AND METHODS: In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). RESULTS: Overall survival at 5 years was 89% in patients who underwent complete resection at diagnosis, 79% in patients who had marginal resection, 52% in initially unresected patients, and 17% in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70%. The rate of response to chemotherapy was 58%. CONCLUSION: The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.
Authors: Christopher L Tinkle; Israel Fernandez-Pineda; April Sykes; Zhaohua Lu; Chia-Ho Hua; Michael D Neel; Armita Bahrami; Barry L Shulkin; Sue C Kaste; Alberto Pappo; Sheri L Spunt; Matthew J Krasin Journal: Cancer Date: 2017-07-31 Impact factor: 6.860
Authors: J R Kroep; M Ouali; H Gelderblom; A Le Cesne; T J A Dekker; M Van Glabbeke; P C W Hogendoorn; P Hohenberger Journal: Ann Oncol Date: 2010-07-23 Impact factor: 32.976
Authors: Andrea Ferrari; Iyad Sultan; Tseng Tien Huang; Carlos Rodriguez-Galindo; Ahmad Shehadeh; Cristina Meazza; Kirsten K Ness; Michela Casanova; Sheri L Spunt Journal: Pediatr Blood Cancer Date: 2011-07-25 Impact factor: 3.167
Authors: Andrea Ferrari; Rosalba Miceli; Annie Rey; Odile Oberlin; Daniel Orbach; Bernadette Brennan; Luigi Mariani; Modesto Carli; Gianni Bisogno; Giovanni Cecchetto; Gian Luca De Salvo; Michela Casanova; Max M Vannoesel; Anna Kelsey; Michael C Stevens; Meenakshi Devidas; Alberto S Pappo; Sheri L Spunt Journal: Eur J Cancer Date: 2010-12-08 Impact factor: 9.162
Authors: Fariba Navid; Catherine A Billups; Matthew J Krasin; Andrew M Davidoff; Joann Harper; Bhaskar N Rao; Sheri L Spunt Journal: J Pediatr Surg Date: 2009-10 Impact factor: 2.545