Purpose or Case Report: Neuroblastoma (NB) is the most common malignancy in neonate and infancy, and, furthermore, growing use of prenatal US has led to increased detection of congenital NB. This study aims to evaluate imaging assessment and clinical features of congenital NB with a special focus on cystic NB.
Methods & Materials: A total of 53 cases (M:F 24:29, mean age 1.27 months) with surgically confirmed congenital NB in a single tertiary center was enrolled. We reviewed imaging features including location, cystic vs non-cystic and presence of metastasis. We also evaluated clinical features including outcome.
Results: Out of 53, 28 tumors (53 %) were detected prenatally and, in the remaining, the most common presentation was abdominal distension. The location was as following; adrenal gland (n=41, 77%), mediastinum (n=6, 11.3%), extraadrenal retroperitoneum (n=5, 9.4%), chest wall (n=1, 1.8% ). Out of 41 adrenal NB, 41% (n=17) appeared cystic as a complex cyst (n=16) or a purely cystic type (n=1) based on US findings and all of cystic tumor were detected prenatally. All of tumors in the other sites were solid. In terms of imaging modality for initial diagnosis, US (n=53) followed by CT ( n= 47 ) or MR (n= 10 ) was performed. In cystic NB (n=17), there was no additional diagnostic information by CT/MR compared with US. All of metastasis (IVS n=8 , IV n=7) were noted in non-cystic NB including liver (n=12), lymph node (n= 3), and bone marrow (n=1). Out of 53, 9 were n-myc amplified tumors (16.8 %) and among them, 3 tumors were cystic NB (17.6 %). Most of the cases (n=49, 92.4 %) showed good outcome without relapse for mean 59.6 month follow-up.
Conclusions: Congenital NB often appears as a prenatally detected adrenal cystic lesion with overall good prognosis. US seems to be the only imaging tool needed in the cystic type.