Thompson L D, Gyure K A
Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 2000 May;24(5):640-50. doi: 10.1097/00000478-200005000-00002.
Extracranial meningiomas of the sinonasal tract are rare tumors. These tumors are frequently misclassified, resulting in inappropriate clinical management. To date, there has been no comprehensive study to evaluate the clinicopathologic aspects of meningioma in these anatomic sites. Thirty cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 were retrieved from the files of the Otorhinolaryngic Registry of the AFIP. Histologic features were reviewed, immunohistochemical studies were performed, patient follow up was obtained, and the results were statistically analyzed. The patients included 15 females and 15 males, aged 13 to 88 years (mean, 47.6 yrs). Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement. Symptoms were present for an average of 31.1 months. The tumors affected the nasal cavity (n = 14), nasopharynx (n = 3), frontal sinus (n = 2), sphenoid sinus (n = 2). or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). The tumors ranged in size from 1.0 to 8.0 cm in greatest dimension (mean, 3.5 cm). Radiographic studies demonstrated a central nervous system connection in six cases. The tumors often eroded the bones of the sinuses (n = 18) and involved the surrounding soft tissues, the orbit, and occasionally the base of the skull. Histologically, the tumors demonstrated features similar to intracranial meningiomas. The majority were of the meningothelial type (n = 23), although there were three atypical meningiomas. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (EMA) and vimentin (all tested). The differential diagnosis includes paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma. Surgical excision was used in all patients. Three patients died with recurrent disease (mean, 1.2 yrs), one was alive with recurrent disease (25.6 years), and the remaining 24 patients were alive or had died of unrelated causes (mean, 13.9 yrs) at the time of last follow up (two patients were lost to follow up). Extracranial sinonasal tract meningiomas are rare tumors which need to be considered in the differential diagnosis of sinonasal tumors. A whorled growth pattern and psammoma bodies, combined with positive EMA and vimentin immunohistochemical reactions, can confirm the diagnosis of meningioma. The overall prognosis is good, without a difference in outcome between benign and atypical meningiomas.
鼻窦道的颅外脑膜瘤是罕见肿瘤。这些肿瘤常被错误分类，导致临床处理不当。迄今为止，尚无全面研究评估这些解剖部位脑膜瘤的临床病理特征。从武装部队病理研究所耳鼻咽喉登记处档案中检索出1970年至1992年间诊断的30例鼻窦道脑膜瘤病例。回顾组织学特征，进行免疫组化研究，获取患者随访情况，并对结果进行统计学分析。患者包括15名女性和15名男性，年龄13至88岁（平均47.6岁）。患者临床症状取决于受累解剖部位，表现为肿块、鼻出血、鼻窦炎、疼痛、视力改变或鼻塞。症状平均出现31.1个月。肿瘤累及鼻腔（n = 14）、鼻咽（n = 3）、额窦（n = 2）、蝶窦（n = 2），或鼻腔与筛窦、额窦、蝶窦和/或上颌窦联合受累（n = 9）。肿瘤最大径1.0至8.0 cm（平均3.5 cm）。影像学检查显示6例有中枢神经系统连接。肿瘤常侵蚀鼻窦骨（n = 18），累及周围软组织、眼眶，偶尔累及颅底。组织学上，肿瘤表现出与颅内脑膜瘤相似的特征。大多数为脑膜上皮型（n = 23），不过有3例非典型脑膜瘤。免疫组化研究通过上皮膜抗原（EMA）和波形蛋白（全部检测）阳性反应确诊为脑膜瘤。鉴别诊断包括副神经节瘤、癌、黑色素瘤、砂粒体性骨化纤维瘤和血管纤维瘤。所有患者均采用手术切除。3例患者死于复发疾病（平均1.2年），1例复发患者存活（25.6年），其余24例患者在最后随访时存活或死于无关原因（平均13.9年）（2例患者失访）。鼻窦道颅外脑膜瘤是罕见肿瘤，在鼻窦肿瘤鉴别诊断中需予以考虑。漩涡状生长模式和砂粒体，结合EMA和波形蛋白免疫组化阳性反应，可确诊脑膜瘤。总体预后良好，良性和非典型脑膜瘤的预后无差异。
