| Popis: |
Rhabdomyosarcoma (RMS) of head and neck region in adult is rare. Orbital and parameningeal subtypes constitute a treatment challenge due its relationship to the skull base and orbit. We report a 21-year-old male patient who presented with right ocular proptosis, bilateral nasal obstruction, frontal headache, right nasal discharge, epiphora and decrease of visual acuity in the right eye for the past 6 months. Computed Tomography (CT) and Resonance Magnetic Images (RMI) scans documented a large mass of the right nasal cavity, occupying totally the ethmoid and maxilar sinuses with markedly extension into the right orbit and anterior cranial base erosion with post contrast homogeneous enhancement. A previous biopsy of the neoplasm revealed an esthesioneuroblastoma. An endonasal endoscopic approach extended to the anterior cranial base and right orbit was performed with orbital preservation. Minimally invasive pericranial flap was used for the reconstruction. There were not transoperative or postoperative complications. 24 hours CT and three months postoperative RMI scans show a gross total resection. Definitive biopsy informs an embryonal RMS. He was immediately referred to chemoradiotherapy. Orbital parameningial RMS is a rare entity in adults but should be included in differential diagnosis. Endonasal endoscopic resection offers a faster recovery with minimal morbidity, providing a better life quality and immediately adjuvant treatment. Minimally invasive pericranial flap constitute a good alternative in cases with non-viable mucosal vascularized nasoseptal flap. Multimodality treatment, including chemoradiation and surgery, play an important role in the management. Highlights Rhabdomyosarcoma is a rare soft tissue malignancy derived from myogenic cells. There is very little literature that provides substantial evidence regarding the outcomes of surgical treatment of parameningeal subtype. Endonasal endoscopic resection offers a faster recovery with minimal morbidity, providing a better life quality and immediately adjuvant treatment. Minimally invasive pericranial flap constitute a good alternative in cases with non-viable mucosal vascularized nasoseptal flap. Multimodal treatment ae essential. |
