Bilateral congenital cystic adrenal neuroblastoma (NB) with cystic liver metastasis is a very rare condition and only few cases have been reported in the literature. Herein we report a case of a congenital bilateral cystic adrenal NB with cystic liver metastasis and briefly discuss characteristic imaging features of cystic NB. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
CASE REPORTA 3-month-old baby presented to clinic with marked abdominal distention. His medical history was insignificant. Laboratory findings were within normal limits except for mildly elevated serum transaminases (AST: 54 IU/L, ALT: 45 IU/L) and neuron-specific enolase (NSE: 21.9 ng/ml). An abdominal ultrasound (US) was performed. US revealed 10x10x12cm heterogeneous cystic mass in the right suprarenal region, which was displacing the right kidney downward. US scan also showed a 2x2x2cm anechoic cystic mass in the left adrenal gland. Borders between the right lobe of the liver and lesion were indistinguishable. Multiple anechoic cystic lesions up to 10mm were detected in the right and the left lobes of the liver. Intravenous contrast enhanced magnetic resonance imaging (MRI) was performed for further evaluation of the lesions to avoid the ionizing radiation of computed tomography (CT) scan. MRI revealed that both lesions were cystic in nature and thick walls of the lesion were enhanced with contrast. No solid components were detected in lesions. Calcifications were noted in the inferior-posterior part of the right-sided suprarenal mass. Lesions in the liver also showed no contrast enhancement on MRI (Fig.1). Cystic nature of the lesion, mildly increased NSE and presence of multiple cystic lesions in the liver, were supporting the diagnosis of congenital bilateral adrenal NB with cystic liver metastasis. Bone marrow aspiration was negative for tumor cells and a probable diagnosis of neuroblastoma was made. In the following days, the patient started to develop a significant shortness of breath due to rapidly growing abdominal masses (NSE also increased) and underwent operation. During the operation, the right-sided lesion was dissected carefully from the surface of the liver and the right kidney. Then both lesions in the adrenal gland were completely excised. Aspiration was performed from one of the cystic lesions in the liver for histopathologic examination. The pathology specimen revealed stroma poor, poorly differentiated cystic NB with 10 fold N-myc amplification, which is favoring bad prognosis according to