Purpose

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.

Conclusion

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.

中文翻译：

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小儿头颈部横纹肌肉瘤的磁共振特征和颅神经受累

目的

横纹肌肉瘤（RMS）是一种常见于儿童的恶性肿瘤。几乎没有报道小儿头颈部 (H&N) RMS 中颅神经受累的频率和特征。本研究的目的是回顾一大群儿科头颈部 RMS，重点是颅神经受累。

方法

我们回顾性审查了 10 年间来自 3 家三级医院的 H&N RMS 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。扫描由两名儿科神经放射科医生审查，对临床数据视而不见。

结果

共有 52 名患者符合纳入标准。组织学上，39/52 为胚胎 RMS，而 13/52 为肺泡 RMS。19.2% 存在区域淋巴结转移。颅神经受累占 36.5%。神经主要作为肿块通过颅底孔直接延伸或侵入海绵窦、Meckel's 洞穴、眶尖或茎乳孔后受累。

结论

颅神经受累在儿科头颈部 RMS 中很常见，并且由于直接延伸通过颅底孔或海绵窦而继发于“地理”侵犯。这些肿瘤从未出现如成人头颈癌病例中所见的远处神经周围转移性疾病。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。