Abstract
BACKGROUND: Rhabdomyosarcoma (RMS) occurring at nonparameningeal head and neck (NPM-HN) sites carries a better prognosis than parameningeal RMS and some other sites. We analyzed the treatments administered and results obtained in patients with localized NPM-HN RMS, included in the protocols coordinated by the Italian Soft Tissue Sarcoma Committee (STSC), in an effort to identify prognostic factors that could facilitate the tailoring of treatment.
METHODS: Sixty-six patients up to 18 years of age with previously untreated, localized NPM-HN RMS were prospectively registered in three consecutive protocols: RMS79, RMS88, and RMS96. Primary surgery was recommended when complete tumor resection was deemed feasible without mutilations. In other cases, only a biopsy was performed, followed by chemotherapy and delayed surgery and/or radiotherapy (RT).
RESULTS: NPM-HN RMS showed favorable characteristics: 72.7% were <5 cm, 72.7% were T1, and 80.3% were N0. With a median follow-up of 16 years (range 7-27), the 10-year progression-free survival and overall survival for the whole group were 65.1% (confidence interval [CI]: 52.3-75.3) and 74.2% (CI: 61.8-83.1). Progressive improvement has been seen in the successive protocols. Age and RT emerged as independent prognostic factors. The group of young children (age <l year) not irradiated was at high risk of failure.
CONCLUSION: The experience of the Italian STSC confirms that NPM-HN RMS has a favorable prognosis, which has improved over the years due to a better, multimodal treatment approach. RT has an important role, but different modalities such as proton therapy and brachytherapy should be explored in systematic ways in very young children.
| Original language | English |
|---|---|
| Journal | Pediatric Blood and Cancer |
| Volume | 65 |
| Issue number | 3 |
| Early online date | Nov 8 2017 |
| DOIs | |
| Publication status | Published - Mar 2018 |
Fingerprint
Keywords
- Journal Article
Cite this
Long-term results in children with head and neck rhabdomyosarcoma : A report from the Italian Soft Tissue Sarcoma Committee. / Affinita, Maria Carmen; Ferrari, Andrea; Milano, Giuseppe Maria; Scarzello, Giovanni; De Leonardis, Francesco; Coccoli, Luca; Pericoli, Roberta; Basso, Eleonora; Zanetti, Ilaria; Scagnellato, Angela; Bisogno, Gianni.
In: Pediatric Blood and Cancer, Vol. 65, No. 3, 03.2018.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Long-term results in children with head and neck rhabdomyosarcoma
T2 - A report from the Italian Soft Tissue Sarcoma Committee
AU - Affinita, Maria Carmen
AU - Ferrari, Andrea
AU - Milano, Giuseppe Maria
AU - Scarzello, Giovanni
AU - De Leonardis, Francesco
AU - Coccoli, Luca
AU - Pericoli, Roberta
AU - Basso, Eleonora
AU - Zanetti, Ilaria
AU - Scagnellato, Angela
AU - Bisogno, Gianni
N1 - © 2017 Wiley Periodicals, Inc.
PY - 2018/3
Y1 - 2018/3
N2 - BACKGROUND: Rhabdomyosarcoma (RMS) occurring at nonparameningeal head and neck (NPM-HN) sites carries a better prognosis than parameningeal RMS and some other sites. We analyzed the treatments administered and results obtained in patients with localized NPM-HN RMS, included in the protocols coordinated by the Italian Soft Tissue Sarcoma Committee (STSC), in an effort to identify prognostic factors that could facilitate the tailoring of treatment.METHODS: Sixty-six patients up to 18 years of age with previously untreated, localized NPM-HN RMS were prospectively registered in three consecutive protocols: RMS79, RMS88, and RMS96. Primary surgery was recommended when complete tumor resection was deemed feasible without mutilations. In other cases, only a biopsy was performed, followed by chemotherapy and delayed surgery and/or radiotherapy (RT).RESULTS: NPM-HN RMS showed favorable characteristics: 72.7% were <5 cm, 72.7% were T1, and 80.3% were N0. With a median follow-up of 16 years (range 7-27), the 10-year progression-free survival and overall survival for the whole group were 65.1% (confidence interval [CI]: 52.3-75.3) and 74.2% (CI: 61.8-83.1). Progressive improvement has been seen in the successive protocols. Age and RT emerged as independent prognostic factors. The group of young children (age CONCLUSION: The experience of the Italian STSC confirms that NPM-HN RMS has a favorable prognosis, which has improved over the years due to a better, multimodal treatment approach. RT has an important role, but different modalities such as proton therapy and brachytherapy should be explored in systematic ways in very young children.
AB - BACKGROUND: Rhabdomyosarcoma (RMS) occurring at nonparameningeal head and neck (NPM-HN) sites carries a better prognosis than parameningeal RMS and some other sites. We analyzed the treatments administered and results obtained in patients with localized NPM-HN RMS, included in the protocols coordinated by the Italian Soft Tissue Sarcoma Committee (STSC), in an effort to identify prognostic factors that could facilitate the tailoring of treatment.METHODS: Sixty-six patients up to 18 years of age with previously untreated, localized NPM-HN RMS were prospectively registered in three consecutive protocols: RMS79, RMS88, and RMS96. Primary surgery was recommended when complete tumor resection was deemed feasible without mutilations. In other cases, only a biopsy was performed, followed by chemotherapy and delayed surgery and/or radiotherapy (RT).RESULTS: NPM-HN RMS showed favorable characteristics: 72.7% were <5 cm, 72.7% were T1, and 80.3% were N0. With a median follow-up of 16 years (range 7-27), the 10-year progression-free survival and overall survival for the whole group were 65.1% (confidence interval [CI]: 52.3-75.3) and 74.2% (CI: 61.8-83.1). Progressive improvement has been seen in the successive protocols. Age and RT emerged as independent prognostic factors. The group of young children (age CONCLUSION: The experience of the Italian STSC confirms that NPM-HN RMS has a favorable prognosis, which has improved over the years due to a better, multimodal treatment approach. RT has an important role, but different modalities such as proton therapy and brachytherapy should be explored in systematic ways in very young children.
KW - Journal Article
U2 - 10.1002/pbc.26876
DO - 10.1002/pbc.26876
M3 - Article
VL - 65
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 3
ER -