Neuroblastoma arising from the organ of Zuckerkandl: an unusual site with a favorable biologic outcome

Background Prognosis in neuroblastoma has been shown to correlate with age and stage at diagnosis and site of origin. Extra-abdominal tumors (chest, neck, pelvis) do better in terms of survival than tumors arising from the upper abdomen. Objective. We evaluated a subgroup of abdominal neuroblastomas arising near to the aortic bifurcation (commonly called organ of Zucker-kandl, O.Z.) to assess their biologic outcome and problems in diagnosis and therapy. Materials and Methods. Sixteen O.Z. primary tumors were seen at three children's hospitals. Their clinical records and imaging studies were reviewed, including the sonographic, CT, and MRI findings. When available, MYCN amplification was noted (MYCN is the current term previously called N-MYC). Results. Despite more than half of the tumors being very large, survival was the rule, with only one fatality (following multiple local recurrences). Only one patient (who survived) had bone metastases. The larger masses were usually palpated in otherwise well children, while the smaller ones were found in the course of evaluation for unrelated problems such as urinary tract infection. Intraspinal extension was common, though usually asymptomatic. MYCN amplification was absent in the four patients studied. Conclusions. Lower abdominal (O.Z,) neuroblastomas present technical problems of surgical removal, but form a group with a favorable outcome similar to cervical and thoracic primary sites. MRI was useful in delineating intraspinal extension.