Archer K F, Hurwitz J J, Balogh J M, Fernandes B J
Department of Ophthalmology, University of Texas Health Science Center, San Antonio.
Ophthalmology. 1989 Nov;96(11):1659-66. doi: 10.1016/s0161-6420(89)32681-9.
Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. Diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.
非嗜铬性副神经节瘤（NCP），也称为球旁体瘤或化学感受器瘤，在眼眶中很少见。眼眶非嗜铬性副神经节瘤的行为可能比其他头颈部部位的更具侵袭性。诊断取决于膜结合神经分泌颗粒的电镜显示。组织病理学研究结果显示，病变边界清晰，无真正的包膜，在网状纤维框架内有薄壁血管，上皮样细胞呈肺泡状或类器官样排列。细胞呈多边形，核圆形或椭圆形，有罕见的有丝分裂象，细胞质淡染。鉴别诊断包括肺泡软组织肉瘤、肺泡横纹肌肉瘤、神经母细胞瘤、类癌和颗粒细胞瘤。在之前报道的29例眼眶NCP病例中，16例已重新分类为肺泡软组织肉瘤。作者报告了1例经电镜确诊的眼眶NCP患者，其对侧颈静脉球瘤14年前曾接受过放疗。
