[An update on the pathology of peripheral neuroblastic tumors]

Peripheral neuroblastic tumors correspond to a generic term for a heterogeneous spectrum of tumors: neuroblastomas, ganglioneuroblastomas (mixed and nodular), and ganglioneuromas. In current practice, the term neuroblastoma is often used because it represents the most frequent and most aggressive tumor component. It is a common tumor in children with heterogeneous morphological, biological and clinical presentations, but with an often unfavorable prognosis. The histoprognostic classification defined by the International Neuroblastoma Pathology Committee is still the reference classification for establishing a prognosis. It must be performed on representative samples containing at least 5 000 cells at the time of diagnosis, before any treatment. It is essentially based on the patient's age, the differentiation of tumor cells, the abundance of schwannian stroma and the mitotic and karyorrhexic index. Immunohistochemical studies may help to make the diagnosis. Very specific tumor markers have been described and may be associated with stromal markers. Immunohistochemical prognostic markers are currently being evaluated. Molecular analysis, which is essential for establishing the prognosis, should be performed on fresh samples. These tumors, which are common in fetuses and rare in adults, display different morphology and prognosis depending on the age at diagnosis. In adults, composite adrenal tumor variants are more common and must be kept in mind. The histopathological examination of neuroblastomas is a key step for optimal management of these pediatric tumors.