Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children.

Autor: Dombrowski ND; Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, U.S.A., Wolter NE; Otolaryngology - Head and Neck Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.; Otolaryngology - Head and Neck Surgery, University of Toronto, Toronto, Ontario, Canada., Robson CD; Department of Radiology, Boston Children's Hospital, Boston, Massachusetts, U.S.A.; Department of Radiology, Harvard Medical School, Boston, Massachusetts, U.S.A., Kawai K; Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, U.S.A.; Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts, U.S.A., Irace AL; Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, U.S.A., Vargas SO; Department of Pathology, Boston Children's Hospital, Boston, Massachusetts, U.S.A.; Department of Pathology, Harvard Medical School, Boston, Massachusetts, U.S.A., Marcus KJ; Division of Radiation Oncology, Boston Children's Hospital, Boston, Massachusetts, U.S.A.; Department of Radiation Oncology, Harvard Medical School, Boston, Massachusetts, U.S.A., Mack JW; Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts, U.S.A.; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, U.S.A., Collins NB; Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts, U.S.A.; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, U.S.A., Rahbar R; Department of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, Massachusetts, U.S.A.; Department of Otolaryngology, Harvard Medical School, Boston, Massachusetts, U.S.A.
Jazyk: angličtina
Zdroj: The Laryngoscope [Laryngoscope] 2021 Mar; Vol. 131 (3), pp. E984-E992. Date of Electronic Publication: 2020 Jul 20.
DOI: 10.1002/lary.28785
Abstrakt: Objectives: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. The goal of this research is to analyze the role of surgery in the management of pediatric parameningeal (PM) and non-PM head and neck RMS (HNRMS).
Study Design: Retrospective review.
Methods: Retrospective chart review of patients <20 years of age treated for HNRMS between 1970 and 2015. Clinical presentation, tumor characteristics, treatment, recurrence, follow-up, and outcome data were collected.
Results: Of 97 patients with HNRMS, 56% were male. Overall median (IQR: interquartile range) age at diagnosis was 5.8 (3.3-9.8) years. Sixty-five patients (67%) had PM tumors. Of 75 patients with histologic subtype identified, 51 (53%) had embryonal and 20 (21%) alveolar RMS. Almost all patients received chemotherapy (99%) and radiotherapy (95%). Forty-four patients (45%) underwent surgery. Surgery was more likely to be conducted in patients with lesions of a non-PM site. Median follow-up time was 3.4 years (IQR: 1.1-10.8). In 5 years of follow-up, 20% (17 of 85) died and 29% (20 of 70) had recurrence. The estimated 5-year survival rate was 72% (95% CI, 57.8, 81.5%). Surgery was associated with a reduced risk of mortality after accounting for TNM stage 4 and tumor site (adjusted HR 0.24; 95% CI, 0.07, 0.79; P = .02). The association between surgery and risk of mortality was similar in PM and non-PM tumors.
Conclusion: A multimodal protocol for treatment including chemotherapy, surgery, and radiotherapy is the mainstay for management of children with HNRMS. While surgery is more commonly used to treat non-PM HNRMS, patients who are able to undergo surgery have significantly higher 5-year survival.
Level of Evidence: 4 Laryngoscope, 131:E984-E992, 2021.
(© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)
Databáze: MEDLINE
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