Abstract
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.
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Change history
06 September 2021
A Correction to this paper has been published: https://doi.org/10.1007/s00234-021-02797-6
Abbreviations
- ADC:
-
Apparent diffusion coefficient
- aRMS:
-
Alveolar rhabdomyosarcoma
- DWI:
-
Diffusion-weighted imaging
- eRMS:
-
Embryonal rhabdomyosarcoma
- H&N:
-
Head and neck
- RMS:
-
Rhabdomyosarcoma
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This work was supported by the National Institute for Health Research, Great Ormond Street Hospital Biomedical Research Centre.
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Talenti, G., Picariello, S., Robson, C. et al. Magnetic resonance features and cranial nerve involvement in pediatric head and neck rhabdomyosarcomas. Neuroradiology 63, 1925–1934 (2021). https://doi.org/10.1007/s00234-021-02765-0
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DOI: https://doi.org/10.1007/s00234-021-02765-0