Characteristics of neonatal neuroblastoma: analysis of 14 cases / 中华围产医学杂志

ABSTRACT

Objective:To analyze the diagnosis and treatment of neonatal neuroblastoma (NB) by summarizing its clinical characteristics.Methods:This study retrospectively recruited 14 neonates with NB in Beijing Children's Hospital (National Center for Children's Health) from February 1, 2015, to February 1, 2020. Medical records and follow-up data as of February 29, 2020, were collected, and clinical staging based on International Neuroblastoma Staging System, risk grouping based on American Childhood Oncology Group risk grouping system, diagnosis, treatment, and prognosis were analyzed. According to whether surgical treatment was performed in the neonatal period or not, these subjects were divided into surgical and non-surgical groups. A descriptive statistical analysis was used for data analysis.Results:(1) Neonates with NB accounted for 0.063% (14/22 006) of the total number of newborns admitted to the hospital during the same period. The 14 cases were all full-term aged 15 d (8 h-23 d) at admission. Tumors were found in seven cases in prenatal examinations, while others presented with shortness of breath (three cases), abdominal distension (two cases), fever (one case), and dysuria and difficult defecation after birth (one case). (2) The primary tumor sites included the adrenal gland (eight cases), posterior mediastinum (three cases), retroperitoneum (two cases), and sacrococcygeal (one case). Three cases had extensive diffuse liver metastasis at admission. (3) Except for two cases who refused to examine, the serum neuron-specific enolase of 12 cases was 57.2 ng/ml (35.9-158.3 ng/ml) during hospitalization, and the urine vanillyl-mandelic acid creatinine of four cases was 2 304.940 (685.748-9 595.314). (4) Primary tumor sites were found in 14 cases by imaging examination. Bone scanning was performed in three cases, including one with a concentrated shadow of the right sacroiliac joint and two with no abnormalities. Ten cases underwent bone marrow aspiration and all with normal results. (5) Of the ten neonates received surgery (the surgical group), nine had the primary tumor wholly removed, without chemotherapy after the operation, and the tumor-free survival period was 19 months (1-45 months). One case (case 5) had a substantial primary tumor that could not be completely resected. The patient underwent a second surgery five months after the first operation due to disease progression and received postoperative chemotherapy. The child had stopped chemotherapy for 24 months and survived without a tumor. (6) In the non-surgical group (cases 11 to 14), the tumor in case 11 who refused chemotherapy shrank spontaneously after discharge, and the patient survived for 20 months with the tumor. The parents of the case 12 withdrew treatment during hospitalization, while the primary tumor and metastases disappeared after discharge, and the specific tumor markers gradually decreased to normal levels. The patient has been tumor-free survived for 25 months. Case 13 received mediastinal tumor resection and chemotherapy during infancy. At the end of the follow-up, chemotherapy had been stopped for 12 months, and the patient survived without a tumor. Case 14 withdrew treatment and died. (7) Among the ten cases in the surgical group, one patient's pathological result indicated a composite tumor, while the others were low differentiated neuroblastoma. There was no MYCN gene amplification, 1p36 deletion, or 11q23 deletion in the ten cases. (8) Among the ten children in the surgical group, nine were in stage 1, and one was in stage 4S (case 5). Nine cases were classified into extremely low-risk groups, while the other was in the low-risk group. The four cases in the non-surgical group could not be grouped by risk. Conclusions:Clinical manifestations of neonatal NB are often atypical. NB in stage 4S might resolve spontaneously, and expectant observation may be considered. The overall prognosis of neonatal NB is generally good, but further researches are needed.