Rabdomiosarcoma nasal: estrategias terapéuticas y factores pronósticos. Una revisión sistemática.

Abstract

Introduction and objective: Nasal rhabdomyosarcoma (RMS) is a rare malignant neoplasm in adults, accounting for less than 1% of sarcomas in this population. This tumour, derived from striated muscle precursor cells, poses significant diagnostic and therapeutic challenges due to the complex anatomy of the nasal cavity and its proximity to vital structures. This review analyses the management of nasal rhabdomyosarcoma in adults, with special attention to the most common histological subtypes, clinical characteristics, available therapeutic modalities, key prognostic factors, and future lines of research aimed at optimising treatment.

Method: A systematic review was conducted in PubMed, MEDLINE, Cochrane Library, and Scopus, including all studies published in English or Spanish between 2016 and 2024, according to PRISMA criteria.

Results: Fourteen studies with a total of 3,590 patients were included. The pleomorphic subtype was the most common in adults (43%), followed by the embryonic (34%) and alveolar subtypes. Multimodal treatment (surgery, chemotherapy, and radiotherapy) was the predominant approach. Proton radiotherapy showed better local control rates, while immunotherapy showed partial responses in selected cases. The most relevant prognostic factors were histology, local invasion, surgical margins, and the presence of PAX-FOXO1 fusion.

Discussion: Nasal RMS in adults responds less well to conventional treatments than in the paediatric population. New therapeutic modalities, such as proton therapy and immunotherapy, are emerging as promising alternatives.

Conclusions: The management of nasal RMS requires a personalised, multidisciplinary approach. Multicentre studies are needed to optimise therapeutic protocols and better characterise the molecular markers involved.