[Correlation study between tumor location and pathological subtypes of neuroblastic tumors associated with opsoclonus myoclonus ataxia syndrome].
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Objective: To analyze the correlation between the location and pathological subtypes of of neuroblastic tumors (NBT) associated with opsoclonus myoclonus ataxia syndrome (OMAS). Methods: A case-control study. Clinical data of 34 pediatric patients diagnosed with NBT complicated by OMAS (NBT-OMAS group), who were hospitalized in the Department of Pediatrics, Peking University First Hospital from January 2007 and December 2022 were collected retrospectively. The control group consisted of 34 age and sex-matched patients with NBT only (NBT-only group) admitted during the same period. Chi-square test or Fisher exact test was used to compare the distribution of tumor locations and pathological subtypes between the two groups. Cramer's V coefficient was applied to analyze the correlation between them. The outcomes of the patients were followed up. Results: The age of patients in the NBT-OMAS group was 18 (12, 28) months, while that of the NBT-only group was 20 (13, 31) months. In the NBT-OMAS group, 28 cases (82%) had tumors located in the paraspinal sympathetic chain and 6 cases (18%) in the adrenal glands. In the NBT-only group, 6 cases (18%) had tumors located in the adrenal glands and 28 cases (82%) in the paraspinal sympathetic chain, with a statistically significant difference between the two groups (χ2=10.69, P<0.01). The distribution of pathological subtypes of tumors in the NBT-OMAS group was as follows: 20 cases (59%) of ganglioneuroblastoma (GNB), 10 cases (29%) of neuroblastoma (NB), 4 cases (12%) of ganglioneuroma (GN). In the NBT-only group, NB was predominant with 21 cases (62%), GNB in 13 cases (38%), and no GN subtype was observed. There was a statistically significant difference between the two groups (χ2=8.59, P<0.01). In the NBT-OMAS group, the pathological subtype of tumors located in the adrenal gland was mainly NB, with 5 cases (5/6). For tumors located paravertebral to the thoracic and lumbar spine, the predominant subtype was GNB, with 8 cases (8/11) and 11 cases (11/13) respectively. As for tumors paravertebral to the sacral spine, the major subtype was NB, with 3 cases (3/4). In the NBT-only group, NB was the pathological subtype in 18 cases of adrenal gland tumors (18/19). For tumors paravertebral to the cervical spine, NB and GNB each accounted for 2 cases respectively (2/4). Tumors located paravertebral to the thoracic spine and paravertebral to the lumbar spine were all GNB for 5 cases (5/5), while those paravertebral to the sacral spine were all NB for 1 case (1/1). The Cramer's V coefficients of the tumor location and pathological subtype in the two groups were 0.65 and 0.81, respectively. The follow-up ended on December 31, 2023. In the NBT-OMAS group, 1 case had tumor recurrence and 1 case died due to tumor progression; both the recurrent and fatal tumors were located in the adrenal glands with the pathological subtype of NB. In the NBT-only group, 11 cases died of tumor progression, among which 8 cases had primary tumor located in the adrenal gland and with the pathological subtype of NB. Conclusions: In children with NBT-OMAS, tumors located in the paravertebral sympathetic chains are significantly more common than those in the adrenal gland, the pathological subtype of tumors was predominantly GNB. The tumor location was correlated with its pathological subtype.