成人无骨质破坏鼻腔胚胎性横纹肌肉瘤1例

Embryonal rhabdomyosarcoma of nasal cavity without bone destruction in an adult: a case report

摘要:

Abstract:

Objective To analyze the clinicopathological features, IRS-G staging pitfalls, and treatment strategies of adult sinonasal embryonal rhabdomyosarcoma (ERMS), so as to improve early recognition and reduce misdiagnosis. Methods The clinical, imaging, surgical and pathological data of one adult ERMS patient were retrospectively analyzed, and relevant literatures were reviewed. Results A 51-year-old female presented with 6-month progressive left nasal obstruction and poor response to conservative therapy for rhinosinusitis and nasal polyp. Sinus CT showed soft-tissue density in left nasal cavity and anterior ethmoid sinus with intact sinus wall and no osteolytic bone destruction. Complete endoscopic tumor resection was achieved with R0 negative margin. Embryonal rhabdomyosarcoma was confirmed by pathology with Ki-67 index about 60% in hot-spot area. The tumor was confined to nasal soft tissue without sinus invasion, lymph node or distant metastasis and classified as IRS-G stage Ⅰa. Adjuvant chemoradiotherapy was performed after multidisciplinary team (MDT) evaluation and no recurrence was observed during follow-up. Conclusion Adult sinonasal ERMS may present without bone destruction and mimic inflammatory nasal polyp. IRS-G grouping should be determined by intraoperative findings and pathological examination rather than imaging alone. Aggressive adjuvant therapy is warranted for adult parameningeal tumors with high proliferative activity even at IRS-G stage Ⅰa.