Surgical Management of Rhabdomyosarcoma of the Nasal Cavity and Paranasal Sinuses: Analysis of Operative Indications, Settings, and Outcomes

Abstract Objectives  The role of surgery in management of sinonasal rhabdomyosarcoma (SNRMS) has traditionally been limited, owing to anatomic and technological challenges and the established role of systemic therapy. Herein, we report our institutional experience with surgical management of SNRMS,...

Full description

Saved in:
Bibliographic Details
Published in:Journal of neurological surgery. Part B, Skull base Skull base, 2022-08, Vol.83 (4), p.350-358
Main Authors: Kana, Lulia A., Smith, Joshua D., Bellile, Emily L., Chugh, Rashmi, McKean, Erin L.
Format: Article
Language:English
Subjects:
Original
Original Article
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Objectives  The role of surgery in management of sinonasal rhabdomyosarcoma (SNRMS) has traditionally been limited, owing to anatomic and technological challenges and the established role of systemic therapy. Herein, we report our institutional experience with surgical management of SNRMS, with a particular focus on operative approaches, extent and outcomes. Design  This study is a retrospective cohort study. Setting  This study was conducted at a single-institution, academic center. Participants  Patients of any age with histologically confirmed RMS of the nasal cavity, maxillary, ethmoid, frontal, or sphenoid sinus, nasolacrimal duct, or nasopharynx presenting between 1994 and 2020 were included in this study. Main Outcome Measures  Demographics, tumor characteristics, operative settings, complications and recurrence, and survival outcomes were the primary outcomes of this study. Results  Our study cohort comprised of 29 patients (mean [range] age: 27.0 [3.1–65.7], n  = 12 [41%] female). Tumors of the nasal cavity ( n  = 10, 35%) and ethmoid sinuses ( n  = 10, 35%) and those with alveolar histology ( n  = 21, 72%) predominated. Patients who had surgery as part of their treatment ( n  = 13, 45%) had improved distant metastasis-free survival (DMFS) overall (hazard ratio [HR]: 0.32, 95% CI: 0.11, 0.98, p  = 0 .05 ) as compared with those who did not have surgery. Surgical approaches included open ( n  = 7), endoscopic ( n  = 4), and combined ( n  = 2). Eight of these 13 patients (62%) had an R0 resection. Additionally, surgical salvage of recurrent disease was employed in five patients (17%). Conclusion  SNRMS is an aggressive malignancy with a high rate of recurrence and spread requiring a multidisciplinary approach for optimal outcomes. Our data supports an expanding role for surgery for SNRMS given its feasibility, tolerability, and potential to improve outcomes.
ISSN:2193-6331
2193-634X
DOI:10.1055/s-0041-1736590