Clinical diagnosis and treatment analysis of olfactory neuroblastoma with paraneoplastic syndromes
SUN Yan1, HUANG Qian1, CUI Shunjiu1, WANG Mingjie1, ZHANG Shurong2, YANG Bentao3, QIU E4, QU Jing1, ZHOU Bing1
1. Department of Otolaryngology-Head and Neck Surgery,2. Department of Oncology,3. Department of Radiology,4. Department of Neurosurgery, Beijing Tongren Hospital of Capital Medical University, Beijing 100730, China
Abstract：Objective To summarize the clinical diagnosis and treatment features of olfactory neuroblastoma(ONB) with paraneoplastic syndromes (PNS).Methods Four ONB patients with PNS in our department from August 2010 to August 2020 were reviewed. Their clinical data were collected. There were 2 males and 2 females, with an average age of 39.5 years. According to the modified Kadish stage, all the patients were Kadish stage C; while according to Dulguerov stage, there was 1 case of T3 and 3 cases of T4.Results All patients underwent endoscopic resection with negative margins. Diabetes insipidus, electrolyte disturbance and hormone changes occurred in 3 patients during the perioperative period. Postoperative complications such as cerebrospinal fluid leakage and meningitis were not found, but 1 patient had intracranial hematocele. Diabetic insipidus, ion disorders, and hormone changes generally returned to normal about 2 weeks after surgery. The follow-up time was 6-125 months, and no tumor recurrence or metastasis was observed. Hormone replacement therapy was not performed. Immunohistochemical staining showed that most of the tumor cells were positive for synaptophysin (Syn) and pheochromochrome A(ChgA), and Ki-67 staining showed cell proliferation index ranging from 1% to 50%.Conclusions ONB patients with PNS usually have insidious symptoms and need to be vigilant. These patients are prone to electrolyte disturbance and hormone level disturbance during the perioperative period. Electrolyte levels can be monitored regularly to prevent tumor recurrence.
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2021, Vol. 32 
