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Treatment of nasolabial fold rhabdomyosarcoma in children: A single-institution experience.
Zhang, Ge; Sun, Nian; Ni, Xin; Su, Yan; He, Lejian; Liu, Zhikai; Zhang, Jie; Li, Yanzhen; Zhang, Xuexi; Liu, Qiaoyin; Liu, Zhiyong; Li, Xiaodan; Mei, Lin; Liu, Yuwei; Ji, Tingting; Wang, Shengcai.
Afiliación
  • Zhang G; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Sun N; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Ni X; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Su Y; Department of Beijing Key Laboratory for Pediatric Diseases of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • He L; Department of Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Liu Z; Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Zhang J; Department of Radiotherapy, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
  • Li Y; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Zhang X; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Liu Q; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Liu Z; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Li X; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Mei L; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Liu Y; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Ji T; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
  • Wang S; Department of Otolaryngology - Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Head Neck ; 46(4): 905-914, 2024 Apr.
Article en En | MEDLINE | ID: mdl-38214480
ABSTRACT

OBJECTIVES:

To summarize the clinical characteristics and prognosis of children with nasolabial fold rhabdomyosarcoma (RMS).

METHODS:

Retrospective review of children treated for nasolabial fold RMS from January 2014 to September 2019.

RESULTS:

Of 21 patients with nasolabial fold RMS, 90.48% were alveolar subtype, in which PAX3/7-FOXO1 fusion positive accounted for 87.5%. Ten patients (47.62%) had nodals invasion. Almost all patients received comprehensive treatment (chemotherapy [100%], radiation therapy [100%], and surgery [95.24%]). The median follow-up time was 34.3 months. The 3-year overall survival (OS) and event-free survival (EFS) was 67.7% ± 14.1% and 42.1% ± 13.5%, respectively. Four patients had regional lymph node relapse (NR), all in the ipsilateral submandibular lymph node region.

CONCLUSION:

Majority of the patients with RMS in the nasolabial fold area were alveolar subtype and had positive PAX3/7-FOXO1 gene fusion. In addition, the nasolabial fold RMS had a high probability of regional lymph node metastasis in the submandibular area. To maintain the facial aesthetics and functions, the surgical area for nasolabial fold RMS is often very conservative and restricted. This could be one of the contributors for the poor prognosis of nasolabial fold RMS beside its worse pathological subtype and gene fusion.
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Texto completo: 1 Base de datos: MEDLINE Asunto principal: Rabdomiosarcoma / Surco Nasolabial Tipo de estudio: Prognostic_studies Idioma: En Revista: Head Neck Asunto de la revista: NEOPLASIAS Año: 2024 Tipo del documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Base de datos: MEDLINE Asunto principal: Rabdomiosarcoma / Surco Nasolabial Tipo de estudio: Prognostic_studies Idioma: En Revista: Head Neck Asunto de la revista: NEOPLASIAS Año: 2024 Tipo del documento: Article