Clinical diagnosis and treatment analysis of olfactory neuroblastoma with paraneoplastic syndromes

SUN Yan; HUANG Qian; CUI Shunjiu; WANG Mingjie; ZHANG Shurong; YANG Bentao; QIU E; QU Jing; ZHOU Bing

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Medical Journal of the Chinese People Armed Police Forces ›› 2021, Vol. 32 ›› Issue (6) : 504-509.

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

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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.

Key words

olfactory neuroblastoma / paraneoplastic syndromes / endoscopic / syndrome of inappropriate antidiuretic hormone / ectopic ACTH secretion

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SUN Yan, HUANG Qian, CUI Shunjiu, WANG Mingjie, ZHANG Shurong, YANG Bentao, QIU E, QU Jing, ZHOU Bing. Clinical diagnosis and treatment analysis of olfactory neuroblastoma with paraneoplastic syndromes[J]. Medical Journal of the Chinese People Armed Police Forces. 2021, 32(6): 504-509

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