Clinical characteristics and prognosis of 40 patients with periorbital metastasis of neuroblastoma

Abstract Background This study was performed to summarize the clinical characteristics of patients with periorbital metastasis of neuroblastoma (NB) and analyze the short-term prognosis. Methods This study included 40 patients with periorbital metastasis of NB from March 2007 to March 2018. The patients’ clinical characteristics and short-term prognosis were retrospectively analyzed. Results The 40 patients comprised 25 boys and 15 girls with a median age at onset of 31 months (range, 8–132 months). The patients’ symptoms included a periorbital mass and eye bruising (n = 37, 92.5%), limb pain (n = 14, 35.0%), a head and neck mass (n = 8, 20.0%), fever (n = 8, 20.0%), an abdominal mass (n = 4, 10.0%), weakness (n = 3, 7.5%), abdominal pain (n = 2, 5.0%), anemia (n = 2, 5.0%), and others (n = 3, 7.5%). All 40 patients had stage IV, high-risk NB. Various auxiliary structures of the eye were involved: ocular muscles (n = 11, 27.5%), lacrimal glands (n = 4, 10.0%), optic nerve (n = 2, 5.0%), and eyelid (n = 1 2.5%). Fifteen patients (37.5%) had tumor invasion in a periorbital sinus cavity. All patients had bone marrow and bone metastasis. Of 38 patients with a known MYCN gene status, 17 patients (44.7%) had MYCN amplification. The median follow-up duration was 19 months (range, 4–144 months). The 5-year event-free survival rate was 6%, and the 5-year overall survival rate was 16%. Conclusions Patients with periorbital metastasis of NB often have multi-organ metastases, extremely high rates of MYCN amplification and bone marrow metastasis, and a poor short-term prognosis.