目的:横纹肌肉瘤(RMS)是儿童常见的恶性肿瘤。很少报道小儿头颈部(H&N)RMS的颅神经受累的频率和特征。这项研究的目的是回顾大量儿科头颈部RMS,重点是颅神经受累。
方法:我们回顾性分析了3家三级医院10年的H&NRMS病例。颅神经受累定义为放射学上明显的肿瘤沿神经延伸和/或存在继发性体征。两名儿科神经放射科医生对扫描进行了审查,对临床数据不知情。
结果:共有52例患者符合纳入标准。组织学上,39/52是胚胎RMS,而13/52为肺泡RMS。区域淋巴结转移占19.2%。颅神经受累占36.5%。神经主要通过颅底孔或海绵窦侵入后直接延伸,梅克尔的洞穴,眶尖,或茎乳突孔。
结论:颅神经受累在小儿头颈部RMS中很常见,并且由于直接通过颅底孔或海绵窦延伸而继发于“地理”侵袭。这些肿瘤从未表现出远处的神经周围转移性疾病,如成人头颈部癌所示。这意味着与成人H&N肿瘤相比,神经和这些肿瘤之间存在不同的生物学相互作用。
OBJECTIVE: Rhabdomyosarcoma (RMS) is a malignant tumor frequent in children. The frequency and characteristics of cranial nerve involvement in pediatric head and neck (H&N) RMS have been scarcely reported. The aim of this study is to review a large cohort of pediatric head and neck RMS with an emphasis on cranial nerve involvement.
METHODS: We retrospectively reviewed H&N RMS cases from 3 tertiary hospitals over a 10-year period. Cranial nerve involvement was defined as radiologically apparent tumor extension along a nerve and/or the presence of secondary signs. Scans were reviewed by two pediatric neuroradiologists, blinded to clinical data.
RESULTS: A total of 52 patients met the inclusion criteria. Histologically, 39/52 were embryonal RMS, while 13/52 were alveolar RMS. Regional lymph nodes metastases were present in 19.2%. Cranial nerve involvement was present in 36.5%. Nerves were mainly involved as a direct extension of the mass through skull base foramina or after invasion of cavernous sinus, Meckel\'s cave, orbital apex, or stylomastoid foramen.
CONCLUSIONS: Cranial nerve involvement is frequent in pediatric head and neck RMS and occurs secondary to \"geographic\" invasion due to direct extension through skull base foramina or cavernous sinus. These tumors never showed distant perineural metastatic disease as is seen in cases of adult head and neck carcinomas. This implies a different biological interaction between the nerves and these tumors in comparison to adult H&N tumors.