Primary paratesticular neuroblastoma: An important differential diagnosis

Abstract Primary neuroblastoma as a paratesticular tumor is extremely rare with only 8 described cases worldwide. In this article, we present the case of a 5-month-old boy with this rare tumor location and give an update on the current literature. As in our case, typically these tumors present as Stage 1 disease and simple tumorectomy alone leads to excellent outcome and long-term survival. However, unawareness of this differential diagnosis and misinterpretation of intraoperative frozen sections have shown to result in delay of diagnosis and worse, overtreatment including orchiectomy and unnecessary chemotherapy. Therefore, primary paratesticular neuroblastoma must be considered as a differential diagnosis when evaluating and operating children with paratesticular tumors.