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INTRACRANIAL NEUROBLASTOMA METASTASIS WITHOUTH MYCN AMPLIFICATION
Case Presentation, Discussion, and Final Comments.
In March 2019, a 20 months old boy was admitted to the hospital with a history of a growing painless nodule on the left parieto-occipital region and abdominal distension. Head MRI showed an extra-axial, contrast enhancing lesion on the fronto-parietal region, measuring 54x53x43mm, while an abdominal MRI showed a left sided retroperitoneal mass measuring 46x34x32mm arising from the ipsilateral adrenal gland in association with disseminated abdominal lymphadenopathy.
Histopathology and immunohistochemistry of the intracranial lesion showed a highly cellular, poorly differentiated malignant tumor consisting of small cells compatible with the diagnosis of Neuroblastoma. Fluorescence in situ hybridization revealed absence of NMYC amplification. Bone scintigraphy demonstrated many areas of increased tracer activity. Bilateral bone marrow biopsy revealed neoplastic infiltrative cells. The patient was classified as a INSS stage 4 and received five cycles of chemotherapy, with an alternating combination of cyclophosphamide, topotecan, doxorubicin, carboplatin and etoposide. During the interval between the phases of each cycle the patient received filgrastim.
After receiving the fourth chemotherapy cycle the patient was reassessed for treatment response. Despite the partial response to treatment, ten months past the initial diagnosis, the patient evolved with febrile neutropenia during the fifth cycle of chemotherapy and died from infectious complications before completing the protocol for autologous stem cell transplantation.
Neuroblastoma intracranial metastases are a rare and serious complication mostly seen in patients with advanced disease with MYCN amplification and it relates to a significantly worse prognosis, with some studies suggesting a one-year survival rate of less than 50%.
Despite the advances in the comprehension of cytogenetic and molecular alterations involved in the pathogenesis of Neuroblastoma as well as the new treatment protocols based on risk stratification, the management of patients with this condition is still challenging, especially in patients with stage 3 and 4 diseases, in which the treatment itself imposes great risk of toxicity and comes associated with great morbidity and mortality. In addition, the concurrent presence of intracranial metastasis suggests an even more disheartening prognosis.
Neuroblastoma, Intracranial Neuroblastoma, Intracranial Neuroblastoma Metastasis,
EDER DA SILVA ROCHA, AIESKA KELLEN DANTAS DOS SANTOS