Good Evolution of Stage IV Neuroblastoma in the Absence of Stem Cell Autograft
Journal Title: Progressing Aspects in Pediatrics and Neonatology - Year 2018, Vol 1, Issue 4
Abstract
Neuroblastoma is a relatively common pediatric pathology of the sympathetic nervous system. The particularity of neuroblastoma lies in its development from cells whose embryological maturation into adult sympathetic nerve cells or chromaffin cells is incomplete. In the literature, many cases of spontaneous regression of neuroblastoma have been reported, but the mechanisms for which they are responsible remain ambiguous. The specificity of our case is the benign evolution of an osteomedullary stage 4 neuroblastoma and its maturation into a ganglioneuroma 6 years after the end of chemotherapy. This transformation depends on various factors related to the tumor (such as the over expression or not of N-myc, the presence or absence of Treks and their receptors), the host (the intervention of the immune system) and to other external factors. Being able to induce this differentiation by not leaving it to chance would be a therapeutic challage and could categorically change the prognosis of neuroblastomas. This merits a lot of research and studies of various potential future treatments (such as treks inhibitors and retinoid). Neuroblastoma (NB) is a relatively common pediatric pathology of the sympathetic nervous system [1]. It is a malignant tumor of the neuroblast. The originality of neuroblastoma lies in its development from cells whose embryological maturation into adult sympathetic nerve cells or chromaffin cells is incomplete. In addition, the evolution of neuroblastoma is unlikely: the tumor can be aggressive and evolves rapidly into a metastatic disease, as it can be “nice” and undergoes regression or maturation (spontaneous or probably induced) into a benign tumor: the ganglioneuroma. Spontaneous regression of neuroblastoma is rare, occurring in 1 to 2% of cases. However, this tumor has the highest rate of spontaneous regression than any other cancer [2]. The incidence of spontaneous regression in neuroblastoma is estimated to be between 10 and 100 times greater than that of any other human cancer [3]. In the literature, many cases of spontaneous regression of neuroblastoma have been reported, but the mechanisms for which they are responsible remain ambiguous. Several theories have been advanced in order to solve this enigma, and to try to find some therapeutic approaches. We report the case of a female patient aged 21 months at the time of diagnosis, who was followed and treated for stage 4-neuroblastoma. The evolution, after six years of stopping treatment, was marked by the maturation of its tumor into a ganglioneuroma. From this case, we want to identify the various mechanisms that could explain this phenomenon which would encourage pushing the studies in this direction and try to induce the maturation or the regression of the tumor really and not to wait for its spontaneous occurrence desperately during the evolution of the disease.
Authors and Affiliations
Wala Ben Kridis, Ines Werda, Rim Kallel, Nabil Toumi, Tahia Boudawara, Afef Khanfir, Mounir Frikha
Keywords
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- EP ID EP580456
- DOI 10.32474/PAPN.2018.01.000119
- Views 57
- Downloads 0
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