Zhu Binbin, Yan Jianhua
State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, China.
J Craniofac Surg. 2019 Sep;30(6):e503-e506. doi: 10.1097/SCS.0000000000005408.
Paragangliomas are groups of neuroendocrine neoplasms originating from neural crest cells throughout the body, but are rarely observed within the orbit.
Two patients, a 4-year-old male and 27-year-old female, presented with a slow-growing proptosis. Well-defined masses were located in the superomedial (male) and temporal (female) portion of the right orbit and involved the entire bodies of either the superior (male) or lateral (female) rectus muscles. B-mode ultrasound scan revealed a homogeneous, well-defined, hypoechoic mass, with rich blood flow signals inside the mass on CDI. CT scans indicated a well-defined, homogeneous mass with moderate enhancement, while MRI showed a well-defined mass with a salt and pepper appearance due to the prominence of blood vessels in the form of flow-void areas. Anterior orbitotomy and total tumor excision were performed on both patients. Subsequent histological and immunohistochemistry assays confirmed the diagnosis of orbital paraganglioma. The male patient is currently at 14 years of follow-up without recurrence or metastasis. The female patient developed recurrence at 3 months post-surgery. After a second surgery and local radiotherapy, there were no signs of the recurrence as assessed at 13 years of follow-up in this female patient.
Orbital paraganglioma is an extremely rare benign tumor. The salt-and-pepper appearance as observed with MRI scan represents a relatively salient characteristic of this condition. Total excision of the lesion by orbitotomy is the treatment of choice and the possibility of tumor recurrence warrants diligent consideration. Radiotherapy is useful in patients with tumor recurrence or subtotal surgical excision.
