Giovanni Cecchetto

BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity. The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24‐year period.METHODS: Fifty‐two patients age 8–18 years (median 8 years) were observed (1979–2002). Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases. Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted. Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy. Aggressive delayed surgery of residual disease, including compartmental resections, was recommended. Radiotherapy was suggested only for patients age > 3 years when surgery was incomplete or not feasible.RESULTS: The evaluation was performed dividing the patients into two categories according to their chemosensitivity. Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5. Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1. Nineteen of 21 patients with CTs‐NRSTS were alive without disease: the 5‐year overall survival (OS) and progression‐free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns‐NRSTS were alive without disease: 5‐year OS and PFS were 75.3% and 68.3%, respectively. Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors. The achievement of complete delayed resection was particularly important for CTns‐NRSTS. Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor.CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns‐NRSTS. Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread. Cancer 2005. © 2005 American Cancer Society.

BACKGROUND: In the current study, the authors aim to evaluate clinical features and treatment results observed in patients from the German and Italian studies who had nonmetastatic abdominal rhabdomyosarcomas (RMS).METHODS: One hundred sixty‐one patients were observed; 78 registered in the German studies between October 1980 and August 1995, and 83 registered in the Italian studies between April 1975 and December 1995. The age range of the patients was 0–18 years (median, 4 yrs). The distribution of tumor sites was as follows: 32 intraperitoneal, 42 retroperitoneal, 75 pelvic, and 12 not otherwise specified (NOS). Most patients had a large and invasive primary mass (26 T1b, 114 T2b). The breakdown in histology was as follows: 116 embryonal, 34 alveolar, and 11 other (leiomyomatous, pleomorphic, and NOS); all cases were staged according to the Intergroup Rhabdomyosarcoma Studies (IRS) system. Nine Group I patients were treated after surgery with chemotherapy (CT) (radiotherapy [RT] was delivered to treat alveolar RMS in the 1991 German and 1988 Italian studies); 19 Group II patients received CT + RT (40–44 Gy); 133 Group III patients underwent neoadjuvant CT ± surgery and/or RT (54 Gy) + CT. Different CT regimens (based primarily on the administration of vincristine, dactinomycin, doxorubicin, and cyclophosphamide or ifosfamide) were adopted. RT was not recommended for patients age < 3 years.RESULTS: The 10‐year overall survival (OS) and progression‐free survival (PFS) were 47.2% and 43.9%, respectively. The OS was related significantly to the following variables: histology (alveolar, 29.4% vs. nonalveolar, 52.1% [P = 0.0156]), tumor size (> 5 cm, 42.1% vs. < 5 cm, 81% [P = 0.005]), age (< 10 yrs, 51.4% vs. ≥ 10 yrs, 27.8% [P = 0.02]), complete surgery at diagnosis or after CT (±RT) (70.4% vs. 34.4% without it [P = 0.0015]). Most patients who achieved the delayed local control had responded well to neoadjuvant CT.CONCLUSIONS: Tumor size, histology, age, and initial or delayed achievement of local control were important prognostic factors. Most relapsed patients had unfavorable outcomes. Cancer 2003;97:1974–80. © 2003 American Cancer Society.DOI 10.1002/cncr.11285