Role of surgery for nonmetastatic abdominal rhabdomyosarcomas

A report from the Italian and German soft tissue cooperative groups studies

Giovanni Cecchetto, Gianni Bisogno, Jorn Treuner, Andrea Ferrari, Adrian Mattke, Michela Casanova, Patrizia Dall'Igna, Ilaria Zanetti, Sandra Volpato, Fortunato Siracusa, Gianni Scarzello, Camillo Boglino, Modesto Carli

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Abstract

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 = 0.01561), 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.

Original languageEnglish
Pages (from-to)1974-1980
Number of pages7
JournalCancer
Volume97
Issue number8
DOIs
Publication statusPublished - Apr 15 2003

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Rhabdomyosarcoma
Drug Therapy
Radiotherapy
Histology
Alveolar Rhabdomyosarcoma
Ifosfamide
Dactinomycin
Vincristine
Doxorubicin
Cyclophosphamide
Neoplasms

Keywords

  • Abdomen
  • Pediatric soft tissue sarcomas
  • Rbabdomyosarcoma
  • Surgery

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Role of surgery for nonmetastatic abdominal rhabdomyosarcomas : A report from the Italian and German soft tissue cooperative groups studies. / Cecchetto, Giovanni; Bisogno, Gianni; Treuner, Jorn; Ferrari, Andrea; Mattke, Adrian; Casanova, Michela; Dall'Igna, Patrizia; Zanetti, Ilaria; Volpato, Sandra; Siracusa, Fortunato; Scarzello, Gianni; Boglino, Camillo; Carli, Modesto.

In: Cancer, Vol. 97, No. 8, 15.04.2003, p. 1974-1980.

Research output: Contribution to journalArticle

Cecchetto, G, Bisogno, G, Treuner, J, Ferrari, A, Mattke, A, Casanova, M, Dall'Igna, P, Zanetti, I, Volpato, S, Siracusa, F, Scarzello, G, Boglino, C & Carli, M 2003, 'Role of surgery for nonmetastatic abdominal rhabdomyosarcomas: A report from the Italian and German soft tissue cooperative groups studies', Cancer, vol. 97, no. 8, pp. 1974-1980. https://doi.org/10.1002/cncr.11285
Cecchetto, Giovanni ; Bisogno, Gianni ; Treuner, Jorn ; Ferrari, Andrea ; Mattke, Adrian ; Casanova, Michela ; Dall'Igna, Patrizia ; Zanetti, Ilaria ; Volpato, Sandra ; Siracusa, Fortunato ; Scarzello, Gianni ; Boglino, Camillo ; Carli, Modesto. / Role of surgery for nonmetastatic abdominal rhabdomyosarcomas : A report from the Italian and German soft tissue cooperative groups studies. In: Cancer. 2003 ; Vol. 97, No. 8. pp. 1974-1980.
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abstract = "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 = 0.01561), 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.",
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T2 - A report from the Italian and German soft tissue cooperative groups studies

AU - Cecchetto, Giovanni

AU - Bisogno, Gianni

AU - Treuner, Jorn

AU - Ferrari, Andrea

AU - Mattke, Adrian

AU - Casanova, Michela

AU - Dall'Igna, Patrizia

AU - Zanetti, Ilaria

AU - Volpato, Sandra

AU - Siracusa, Fortunato

AU - Scarzello, Gianni

AU - Boglino, Camillo

AU - Carli, Modesto

PY - 2003/4/15

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N2 - 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 = 0.01561), 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.

AB - 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 = 0.01561), 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.

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KW - Pediatric soft tissue sarcomas

KW - Rbabdomyosarcoma

KW - Surgery

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